Respir Med Research Articles

A LATE PRESENTATION OF MOUNIER-KUHN SYNDROME IN A 71-YEAR-OLD MAN

TOPIC: Imaging TYPE: Fellow Case Reports INTRODUCTION: Mounier-Kuhn Syndrome (MKS) is a rare congenital entity characterized by abnormal enlargement of the trachea and main bronchi. MKS was first described at autopsy in 1897 by Czyhlarz. Mounier-Kuhn described the association of endoscopic and radiographic appearance of the enlarged airways in 1932. CASE PRESENTATION: A 71-year-old male, former smoker, with a past medical history of an intellectual disability, severe malnutrition, weight loss, chronic decubitus ulcers, on 2 L. home oxygen therapy and multiple prior episodes of pneumonia, presented to the emergency department by Emergency Medical Service (EMS) after experiencing cardiac arrest at home. Earlier in the day, the patient was reported to be intermittently hypoxic with desaturations to the 70s. After lunch, he suddenly became unresponsive. He had bystander cardiopulmonary resuscitation (CPR) for 10 minutes until EMS arrived. Return of spontaneous circulation (ROSC) was achieved, but he arrested again after intubation, with additional 7 minutes of CPR prior to achieving ROSC. At the hospital, the patient was hypotensive and required intravenous fluids and multiple vasopressor support. A chest x-ray reported a right-sided pneumonia. He was treated with antibiotics. CTA of the chest showed enlargement of the trachea and bilateral bronchi with bronchiectasis, consistent with MKS. Also showed a pneumomediastinum and a small pneumothorax on the right. Pulmonary embolism (PE) was ruled out. Bronchoalveolar lavage showed Methicillin resistance Staphylococcus aureus (MRSA) and antibiotics were de-escalated. He eventually required a tracheostomy and discharged to LTAC. DISCUSSION: MKS is an entity more common in males with 8:1 predominance. The prevalence in the population has not been determined yet but a review found 360 cases published until 2016. The mean age of diagnosis is 53.9 years.The diagnosis of MKS is made when the transverse and sagittal diameters of the trachea exceed 25 mm and 27 mm in men and 21 mm and 23 mm in women, respectively; or when the transverse diameters of the right and left main bronchi exceed 21.1 mm and 18.4 mm in men and 19.8mm and 17.4 mm in women, respectively. There has been no correlation between increasing age and increasing tracheal diameter.Bronchiectasis, tracheal diverticulosis and tracheobronchial dyskinesia have been associated frequently. The most common complains are cough, dyspnea and recurrent respiratory infections.This case was unfortunately missed by many providers and incidentally found in post cardiac arrest admission. CONCLUSIONS: Providers should recognize the enlarge airways on chest imaging and the diagnosis is easily made once obtaining measurements. REFERENCE #1: Krustins E, Kravale Z, Buls A. Mounier-Kuhn syndrome or congenital tracheobronchomegaly: a literature review. Respir Med. 2013;107(12):1822-1828. doi:10.1016/j.rmed.2013.08.042. REFERENCE #2: Menon B, Aggarwal B, Iqbal A. Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications. South Med J. 2008;101(1):83-87. doi:10.1097/SMJ.0b013e31815d4259 REFERENCE #3: Krustins E. Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years. Clin Respir J. 2016;10(1):3-10. doi:10.1111/crj.12192 DISCLOSURES: no disclosure on file for Frank Biscardi; No relevant relationships by Moises Cossio, source=Web Response No relevant relationships by Fernando Fuentes, source=Web Response

LATENT MYCOBACTERIUM TB REACTIVATION IN TWO PATIENTS WITH COVID-19 PNEUMONIA

TOPIC: Chest Infections TYPE: Medical Student/Resident Case Reports INTRODUCTION: Since December 2019, the world has been suffering from the pandemic of the new coronavirus (COVID-19) with a significant mortality rate. It mainly affects the respiratory system with a wide severity range from being asymptomatic to causing severe acute respiratory distress syndrome (ARDS). Few case reports have reported pulmonary tuberculosis co-infection or reactivation in COVID-19 pneumonia patients(1-2). We present two cases of COVID-19 pneumonia who presented, after the resolution of the acute symptoms, with shortness of breath and were found to have pulmonary cavities, suspicious for tuberculosis (TB). CASE PRESENTATION: Case 1: 76-year-old Brazilian male with multiple comorbidities including COPD on home oxygen, who received the monoclonal antibody infusion (Bamlanivimab) a month ago for mild COVID-19 pneumonia, presented with generalized weakness associated with fever and cough. CT chest showed extensive peribronchiolar and centrilobular patchy and nodular opacities with internal cavities (Figure 1 Right). A previous chest x-ray showed left basal lung scarring (Figure 1 left). Case 2: 71-year-old Columbian male with a history of diabetes mellitus and COVID-19 pneumonia treated with dexamethasone and remdesivir for 10 days, presented 3 months later with dyspnea, fever, and productive cough. X-ray and CT chest showed diffuse nodular opacities with dense consolidation in both upper lobes associated with areas of cavitations (Figure 2 Right). A previous chest x-ray did not show any evidence of prior lung pathologies (Figure 2 Left). Bronchial washing PCR was positive for mycobacterium tuberculosis in both cases. DISCUSSION: Cavitary lung lesions are usually caused by mycobacterial, fungal, neoplastic, or autoimmune pathologies. Viral pneumonia, including SARS-CoV and MERS-CoV, is not a common cause of lung cavities even in severe viral infections (3). Although few case reports have reported cavitary lesions as sequelae of COVID-19 pneumonia, common causes should be excluded first. The two most common CT chest findings in COVID-19 patients are ground-glass opacities and bilateral patchy shadowing (4). The presence of lung cavities in such patients should raise the suspicion of TB, especially in high-risk patients. Whether or not the immune system reaction to the COVID-19; including leukopenia, lymphopenia, and an inflammatory cytokine storm can lead to latent TB reactivation is not fully understood yet. However, treatment of COVID-19 with immunosuppressant drugs such as corticosteroids could be a risk factor for TB reactivation. Both of our patients had new lung cavitary lesions with confirmed mycobacterial infection. CONCLUSIONS: Pulmonary cavities in COVID-19 pneumonia patients should raise the suspicion of other pathologies such as tuberculosis, especially in high-risk patients. REFERENCE #1: 1.Tham, S.M., et al., Four Patients with COVID-19 and Tuberculosis, Singapore, April-May 2020.Emerg Infect Dis, 2020. 26(11): p. 2764-2766.10.1056/NEJMoa2002032. REFERENCE #2: 2. Torre, A., et al., Preliminary observations on IGRA testing for TB infection in patients with severe COVID-19 eligible for immunosuppressive therapy. Respir Med, 2020. 175: p. 106204. REFERENCE #3: 3. Zoumot Z, et al. Pulmonary cavitation: an under-recognized late complication of severe COVID-19 lung disease. BMC Pulm Med 21, 24 (2021). https://doi.org/10.1186/s12890-020-01379-14. Guan WJ, et al. Clinical Characteristics of Coronavirus Disease 2019 in China. N Engl J Med 2020; 382:1708-1720. DOI: 10.1056/NEJMoa2002032. DISCLOSURES: No relevant relationships by Sherif Elkattawy, source=Web Response No relevant relationships by Muhammad Atif Noori, source=Web Response No relevant relationships by onyeka Nwachukwu, source=Web Response No relevant relationships by John Viechweg, source=Web Response No relevant relationships by Islam Younes, source=Web Response

EXTENSIVE CYSTIC LUNG DISEASE IN EXTRANODAL MARGINAL ZONE B CELL LYMPHOMA (MALT LYMPHOMA) OF THE LUNG WITH LIGHT CHAIN DEPOSITION DISEASE

TOPIC: Diffuse Lung Disease TYPE: Fellow Case Reports INTRODUCTION: Extranodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT Lymphoma) is a rare disorder, but is the most common cause of primary pulmonary lymphoma involvement. Cystic changes along with nodularity has been previously reported in MALT lymphomas along with light chain deposition disease (LCDD). CASE PRESENTATION: A 44 y/o previously smoking female patient presented with a 3-year history of progressive dyspnea in the setting of extensive cystic lung disease. Her extensive lung cysts had previously raised concern for lymphangioleiomyomatosis (LAM) and she had undergone an open lung biopsy (OLB) about 2 years prior. She also had a 10-year history of Rheumatoid Arthritis (RA) for which she had previously received methotrexate, adalimumab and leflunomide. The OLB was negative for LAM, but no alternative diagnosis was made based on the biopsy results. Due to progressive dyspnea, she presented to our facility where a fresh evaluation of her case was undertaken. A repeat CT Chest again confirmed extensive cystic lung changes that were not present 12 years ago and had minimally progressed from the time of OLB two years ago (figure 1). Pulmonary function testing (PFTs) showed a severe obstructive pattern with a positive bronchodilator response. Laboratory testing confirmed positive results for rheumatoid factor, CCP, ANA as well as SSA & SSB antibodies. A review of the OLB confirmed the presence of an atypical lymphoplasmacytic infiltrate associated with nodular kappa light chain deposition disease (LCDD). The overall pattern was consistent with extranodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT lymphoma). The biopsy confirmed the presence of cystic changes within the nodular light chain deposits. PET scan was negative for extrathoracic lymphoma and the patient was started on Rituximab. DISCUSSION: We present a case of pulmonary MALT Lymphoma with LCDD presenting with extensive cystic lung disease and severe airflow obstruction. The case was initially mistaken for LAM and appropriate therapy was delayed for 2 years. MALT lymphomas with LCDD are rare causes of pulmonary cystic disease and generally have a good prognosis with an overall 5-year survival rate exceeding 80% and a median survival over 10 years. Given the positive Sjogren's antibodies and the presence of lung cysts; lymphocytic interstitial pneumonia (LIP) was also considered in the differential diagnosis. The presence of severe airflow obstruction on lung function was somewhat atypical in this case and a component of bronchiolitis obliterans from RA could not be entirely excluded. CONCLUSIONS: Extranodal marginal zone B cell lymphoma of the lung can present with extensive cystic lung disease that can mimic other common causes of cystic lung disease such as LAM, LIP and Birt Hogg Dube disease and PLCH. Prognosis is generally good and Rituximab is the most commonly used initial treatment approach. REFERENCE #1: pulmonary and nodular cystic light chain deposition disease: a retrospective review of 10 cases. Baqir M et al. Respir Med. 2020 Apr; 164:105896. REFERENCE #2: Nodular pulmonary light chain deposition disease: an entity associated with Sjögren syndrome or marginal zone lymphoma. Arrossi AV et al. J Clin Pathol. 2016 Jun; 69(6): 490-6. REFERENCE #3: Pulmonary mucosa-associated lymphoidtissue lymphoma revisited. Borie R et al. Eur Respir J 2016; 47: 1244-1260. DISCLOSURES: No relevant relationships by Vaibhav Ahluwalia, source=Web Response No relevant relationships by Hasan Ahmad Hasan Albitar, source=Web Response No relevant relationships by Vivek Iyer, source=Web Response

PULMONARY SARCOMATOID CARCINOMA: A CASE REPORT AND REVIEW OF LITERATURE

TOPIC: Lung Cancer TYPE: Medical Student/Resident Case Reports INTRODUCTION: Pulmonary sarcomatoid carcinomas are a rare subtype of poorly differentiated non-small cell lung cancer that accounts for less than 1 percent of primary lung malignancies. This case highlights the clinical presentation & diagnostic evaluation undertaken to identify this rare lung cancer. CASE PRESENTATION: A 77-year-old male with a medical history of tobacco abuse, hypertension & mutism was evaluated for pleuritic chest pain, cough and subjective fevers. Initial vitals were stable and pulmonary examination revealed focal expiratory wheezes auscultated on the right anterior chest with a prolonged expiratory phase throughout. CXR demonstrated a mass-like opacity in the inferior right hilum and the patient was treated with antibiotics for presumed community-acquired pneumonia. Chest CT was obtained due to persistent symptoms which showed a right hilar mass encasing the right pulmonary artery with calcific mediastinal lymphadenopathy. Bronchoscopy with endobronchial ultrasound was performed and samples for cytology were taken from the culprit endobronchial lesion. Lymph node surveillance revealed enlarged left paratracheal, right paratracheal & right hilar lymph nodes from which FNA samples were taken, respectively. Rapid onsite pathological evaluation demonstrated the presence of atypical cells and ultimately revealed sarcomatoid carcinoma. Oncology, radiation oncology, and palliative care were consulted. After a lengthy discussion concerning the treatment options, the patient & family elected to transition to comfort care. The patient was discharged home with hospice. DISCUSSION: Pulmonary sarcomatoid carcinoma is a rare non-small cell carcinoma accounting for approximately 0.1-0.4% of all primary pulmonary malignancies. The disease has a predilection for older males with a prolonged smoking history. Morphologically, sarcomatoid carcinoma is a heterogeneous group of neoplasms that originate from epithelial & mesenchymal tissue containing intermingled elements of carcinoma & sarcoma. There are five recognized subtypes: pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma & pulmonary blastoma. Given the immense histological variability, a pathologic diagnosis frequently requires a generous tissue sample and can often pose a diagnostic challenge. The clinical course is aggressive with early metastasis rendering the overall prognosis is poor despite therapy. Surgical resection offers the best therapeutic option in early disease and remains the standard of care. Targeted therapies & immunotherapy remain a promising option, but additional research & clinical experience is needed in this patient population. CONCLUSIONS: This case highlights the clinical presentation & diagnostic evaluation undertaken to identify sarcomatoid carcinoma, a rare and aggressive lung cancer with heterogeneous pathology & limited response to traditional therapy. REFERENCE #1: Rajdev K, Siddiqui AH, Ibrahim U, Agarwal S, Ding J, Chalhoub M. Sarcomatoid Carcinoma of the Lung Presenting as Localized Bronchiectasis: A Case Report and Review of Literature. Respir Med Case Rep. 2018;24:143-146. Published 2018 May 17. doi:10.1016/j.rmcr.2018.05.016 REFERENCE #2: Shen XY, Lin ZF, Lin Q, et al. Pulmonary sarcomatoid carcinoma: a case report. Contemp Oncol (Pozn). 2013;17(2):210-213. doi:10.5114/wo.2013.34375 DISCLOSURES: No relevant relationships by Kari McCoy, source=Web Response No relevant relationships by Caroline Mears, source=Web Response No relevant relationships by Xian Qiao, source=Web Response

PNEUMATOCELE FORMATION DUE TO SARS-COV2: A CASE REPORT

PNEUMATOCELE FORMATION DUE TO SARS-COV2: A CASE REPORT

NOT EVERY PET POSITIVE MASS IS CANCER

TOPIC: Lung Pathology TYPE: Fellow Case Reports INTRODUCTION: Tuberculosis (TB) is well known as a diagnostic chameleon.1 Both symptomatic and asymptomatic cases of TB can mimic lung cancer with cough, hemoptysis, and weight loss common for both diseases. Many radiological findings on CT imaging and PET avidity can occur with both.2 We present a patient who presented with abnormal imaging who was ultimately diagnosed with pulmonary TB. CASE PRESENTATION: A 55-year old man presented to pulmonary clinic with an enlarging right upper lobe (RUL) mass. The mass was first noted a year prior while being evaluated for anemia and lymphadenopathy thought to be autoimmune. Despite steroids and antibiotics, the mass increased in size. He also had diabetes, tobacco use, and treated latent TB (positive QuantiFERON gold). To evaluate the RUL mass, he had a diagnostic bronchoscopy with transbronchial biopsy which showed scant non-necrotizing granulomas but negative fungal/AFB stains and cultures, and no evidence of malignancy. Given lack of certainty, he underwent navigational bronchoscopy with repeat bronchial wash, and a biopsy of the right upper lobe mass was obtained. The MTB PCR was positive and pathology showed necrotizing granulomas. Despite negative fungal/AFB stains and cultures, the positive PCR and caseating granulomas were consistent with pulmonary TB and he was promptly started on rifampin, isoniazid, pyrazinamide, and ethambutol with pyridoxine. With treatment of TB, both his symptoms and anemia resolved. DISCUSSION: While a growing, PET-avid lung mass in a patient with tobacco exposure is most likely malignant, a broad differential, including granulomatous diseases, should be considered. Of note, some malignancies may co-exist with granulomatous infections, including intra-pulmonary lymphoma or mucinous adenocarcinoma, requiring careful attention to follow-up imaging and a low threshold for repeat biopsies. Repeat navigational bronchoscopy with bronchial wash confirmed the presence of necrotizing granulomas and a positive PCR test, sparing the patient from a surgical biopsy. CONCLUSIONS: Differentiating pulmonary TB from malignancy based on clinical and radiological evaluation can be challenging. Both diseases can present with similar symptoms and parenchymal infiltrates with high metabolic activity on the 18-FDG-PET. The presence of necrotizing granulomas on transbronchial biopsy along with positive PCR testing confirms the diagnosis, but careful attention to follow-up imaging with treatment is warranted. REFERENCE #1: Lang S, Sun J, Wang X, et al. Asymptomatic pulmonary tuberculosis mimicking lung cancer on imaging: A retrospective study. Exp Ther Med. 2017;14(3):2180-2188. doi:10.3892/etm.2017.4737 REFERENCE #2: Hammen I. Tuberculosis mimicking lung cancer. Respir Med Case Reports. 2015;16:45-47. doi:10.1016/j.rmcr.2015.06.007 DISCLOSURES: no disclosure on file for George Cheng; No relevant relationships by Brinda Desai, source=Web Response No relevant relationships by Russell Miller, source=Web Response No relevant relationships by Matthew Nobari, source=Web Response No relevant relationships by Rebecca Sell, source=Web Response

LEGIONNAIRES' DISEASE: A FATAL FORM OF COMMUNITY-ACQUIRED PNEUMONIA

TOPIC: Chest Infections TYPE: Fellow Case Reports INTRODUCTION: Legionella pneumophila is gram-negative bacilli known to cause community-acquired pneumonia (CAP), also termed Legionnaires' disease. While pneumonia is the most common manifestation, extrapulmonary infections include renal injury and rhabdomyolysis. Age, immunosuppression and multi-system infection increase mortality, even with early initiation of therapy. CASE PRESENTATION: A 30-year-old male with history of bipolar disease, alcohol and tobacco abuse presented with diarrhea, fever and confusion for three days. He was found to be febrile, tachycardic, encephalopathic and hypoxic requiring high-flow nasal cannula. Initial evaluation revealed leukocytosis, acute liver injury, acute renal injury, respiratory alkalosis, lactic acidosis, and elevated troponin and creatine kinase levels. Chest x-ray (CXR) showed a dense left upper lobe consolidation. Sepsis was worked up and he was given intravenous fluids and antibiotics (Vancomycin, Cefepime, Metronidazole, Azithromycin). Over several hours, he became oliguric, tachypnic and hypotensive requiring vasopressors, continuous renal replacement therapy (CRRT) and mechanical ventilation. Urinary legionella antigen returned positive so Levofloxacin was added. Despite antibiotics and CRRT, his shock worsened and he ultimately had pulseless electrical activity arrest with demise. DISCUSSION: The presence of acute renal injury in Legionnaires' disease increases mortality up to 51% so early diagnosis is crucial to guide management. The urinary antigen test for L. pneumophila only identifies serogroup 1, which is the most common type. When prevalence is high, specificity of this test is >99%. Severe cases of CAP should be tested for Legionella as half of patients with Legionnaires' disease will require intensive care. Rhabdomyolysis occurs due to direct toxin invasion and renal injury occurs subsequently or due to sepsis. Levofloxacin or Azithromycin are the antibiotics of choice as they are bactericidal and reach high intracellular concentrations. There are twenty-two known cases of Legionnaires' disease with rhabdomyolysis and acute renal injury. Nearly all patients with this triad survived and of those who did, all received antibiotics and about half required hemodialysis (HD). To our knowledge, there is only one other case where the outcome was death despite several days of HD. In comparison, presentation to death occurred in less than twenty-four hours in our case. Cause of death was refractory hyperkalemia due to rhabdomyolysis-induced renal injury despite CRRT. CONCLUSIONS: Even with timely diagnosis and treatment of Legionnaires' disease, renal involvement portends a high mortality rate. At the earliest evidence of renal injury in Legionnaires' disease complicated by rhabdomyolysis, immediate placement of central venous catheters and early initiation of CRRT is mandatory and may be life saving. REFERENCE #1: Soni AJ, Peter A. Established association of legionella with rhabdomyolysis and renal failure: A review of the literature. Respir Med Case Rep. 2019 Oct 28;28:100962. REFERENCE #2: Mercante, J. W., & Winchell, J. M. (2015). Current and emerging Legionella diagnostics for laboratory and outbreak investigations. Clinical microbiology reviews, 28(1), 95–133. REFERENCE #3: Straus W.L., Plouffe J.F., File T.M. Risk factors for domestic acquisition of Legionnaires' disease. Ohio Legionnaires Disease group. Arch. Intern. Med. 1996;156(15):1685–1692. DISCLOSURES: No relevant relationships by Allen Roberts, source=Web Response No relevant relationships by Jigna Solanki, source=Web Response

ACUTE LUNG INJURY IN A WORKER AFTER INHALATION OF ETHYLENE PHOSPHORODIFLUORIDITE

TOPIC: Disaster Medicine TYPE: Global Case Reports INTRODUCTION: Ethylene phosphorodifluoridite (1,2-bis-difluorophosphanyloxy-ethane) [CAS# 3965-00-2](C2H4F4O2P2) is a colorless, corrosive fuming liquid that is water-soluble (3.9 g/100 ml water), and adopted as an electrolyte stabilizer in rechargeable batteries. There are no previous reports of the toxic effects of ethylene phosphorodifluoridite inhalation. In this report, we describe a patient who developed acute lung injury after accidental inhalation of ethylene phosphorodifluoridite. CASE PRESENTATION: We describe a 28-year-old male who complained of respiratory distress after accidental inhalation of ethylene phosphorus difluoride for 30 min. The patient developed acute lung injury with non-cardiogenic pulmonary edema and was treated with supportive management. The patient fully recovered and was discharged after 7 days without any significant sequelae. DISCUSSION: This case report has several toxicological implications. First, many new industrial chemicals with unknown toxicity, such as ethylene phosphorodifluoridite, are produced and used at workplaces due to the rapid development of the materials industry. Caution should be exercised to prevent acute lung injury due to inhalation of unknown chemical compounds that include phosphorus and fluorine. Second, it is important to consider that inhaled toxic chemicals, such as ethylene phosphorodifluoridite, may lead to significant pulmonary disease even if the initial symptoms are mild. Therefore, patients should be monitored closely regardless of initial symptoms. A limitation of this case report is that we were unable to estimate the amount of ethylene phosphorodifluoridite that the patient inhaled. CONCLUSIONS: This case highlights the importance of safety when using ethylene phosphorodifluoridite, which was recently introduced for use in rechargeable batteries due to the rapid development of the materials industry. Inhaled ethylene phosphorodifluoridite may cause acute lung injury with non-cardiogenic pulmonary edema. Supportive management with oxygen led to complete clinical recovery of the patient described here. However, further studies should be performed to confirm the relationship between inhalation of ethylene phosphorodifluoridite and non-cardiogenic pulmonary edema. REFERENCE #1: McKay CA, Jr. Toxin-induced respiratory distress. Emerg Med Clin North Am. 2014 Feb;32(1):127-47. REFERENCE #2: Jegal Y, Kim Y. Industrial chemicals and acute lung injury with a focus on exposure scenarios. Curr Respir Med Rev. 2016;12(1):44-55. REFERENCE #3: Akira M, Suganuma N. Acute and subacute chemical-induced lung injuries: HRCT findings. Eur J Radiol. 2014 Aug;83(8):1461-9. DISCLOSURES: No relevant relationships by Yangho Kim, source=Web Response No relevant relationships by Woon Jung Kwon, source=Admin input No relevant relationships by jimi oh, source=Web Response No relevant relationships by Seung Won Ra, source=Web Response

MASSIVE SPONTANEOUS PLEURAL EFFUSION IN END-STAGE RENAL DISEASE PATIENT WITH VANCOMYCIN RESISTANT ENTEROCOCCUS EMPYEMA AND COVID-19 INFECTION

TOPIC: Disorders of the Pleura TYPE: Medical Student/Resident Case Reports INTRODUCTION: Pleural effusion is one of the most common complications seen in end-stage renal disease (ESRD) patients [1]. Here we report a case of massive spontaneous pleural effusion in an ESRD patient, who tested positive for coronavirus disease 2019 (COVID-19) on hospital admission, and later grew vancomycin resistant enterococcus (VRE) from the pleural space. CASE PRESENTATION: 79-year-old male with past medical history of ESRD on hemodialysis, hypertension, diabetes mellitus and history of prostate cancer presented with abdominal discomfort and progressive nausea and vomiting. COVID-19 test was positive on screening, although vital signs and review of systems were negative except as above. Labs were remarkable for leukocytosis, elevated blood urea nitrogen and creatinine. Chest X-ray showed nearly complete opacification of the left hemithorax associated with dense consolidation of the left lower lobe and the lingula. Computerized tomography scan showed a large complex fluid attenuating lesion involving the left hemithorax, which may represent a large loculated left-sided effusion versus a large left-sided empyema, resulting in mediastinal shift to the right and inferior displacement of the left hemidiaphragm. Pleural fluid was drained via pigtail catheter, and on analysis was found to be exudative with few leukocytes and no microorganisms. Due to septations of the hemithorax and development of hydropneumothorax, chest tube was placed and eventually video-assisted thoracoscopic surgery was performed for further drainage and decortication. Biopsy obtained during surgery showed no malignant cells; tissue culture and subsequent pleural fluid cultures were positive for VRE. DISCUSSION: Pleural effusion is a pathological finding that is often seen in ESRD patients with heart failure, pneumonia, pulmonary embolism, cancer and uremia [1]. Interestingly, our patient received regular hemodialysis, and presented with no signs of heart failure or fluid overload, making ESRD less likely to be a major or direct cause of the massive pleural effusion. Studies show VRE is prevalent in dialysis centers and frequently colonizes ESRD patients [2]. Rarely, VRE can cause empyema, as illustrated by Cotton in an asplenic patient with multiple co-morbidities who presented with pneumonia [3]. Our patient might have VRE colonized in his airway, so pleural cultures were positive after decortication. Additionally, COVID-19 infection has been associated with pleural effusion [4]. Hussein reported an acute accumulation of moderate exudative pleural effusion in a patient with no past medical history except for concurrent COVID-19 infection. As a result, the positive COVID-19 test in our patient might also contribute to his massive effusion. CONCLUSIONS: Factors like ESRD on hemodialysis, positive VRE in pleural tissue culture, as well as COVID-19 infection, may all contribute to the rare massive pleural effusion in our case. REFERENCE #1: Uzan, G. and H. Ikitimur, Pleural Effusion in End Stage Renal Failure Patients. Sisli Etfal Hastan Tip Bul, 2019. 53(1): p. 54-57. REFERENCE #2: Zacharioudakis, I.M., et al., Vancomycin-resistant enterococci colonization among dialysis patients: a meta-analysis of prevalence, risk factors, and significance. Am J Kidney Dis, 2015. 65(1): p. 88-97.Cotton, M.J. and C.D. Packer, Vancomycin-resistant Enterococcus faecium Empyema in an Asplenic Patient. Cureus, 2018. 10(8): p. e3227. REFERENCE #3: Hussein, M., et al., Pleural effusion as an isolated finding in COVID-19 infection. Respir Med Case Rep, 2020. 31: p. 101269. DISCLOSURES: No relevant relationships by Aram Hambardzumyan, source=Web Response No relevant relationships by Stephen Jesmajian, source=Web Response No relevant relationships by Liyun Liu, source=Web Response Speaker/Speaker's Bureau relationship with GSK Please note: approx 2016 to now Added 05/02/2021 by Michael Mandel, source=Web Response, value=Consulting fee No relevant relationships by Corina Nava, source=Web Response No relevant relationships by ABHISHEK NIMKAR, source=Web Response No relevant relationships by Carlos Romano, source=Web Response

THE PARADOXICAL PRESENTATION OF PRE-ECLAMPSIA WITH DIFFUSE ALVEOLAR HEMORRHAGE AND VENOUS THROMBOSIS AS CLINICAL MANIFESTATIONS OF ANTIPHOSPHOLIPID SYNDROME

TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Antiphospholipid Syndrome (APS) is an autoimmune disorder clinically defined by the presence of antiphospholipid antibodies (aPL), history of arterial or deep venous thrombosis (DVT), and/or repeat episodes of early pregnancy loss. Due to the increased risk for thrombosis, often overlooked is a rare and seemingly paradoxical association between APS and diffuse alveolar hemorrhage (DAH) - a disorder characterized by non-localized alveolar bleeding. Rare manifestation of APS presenting with preeclampsia, DAH, and DVT offers a variety of multi-disciplinary challenges. CASE PRESENTATION: A 34-year-old G6P3 woman at 28 weeks gestation with a remote history of pulmonary embolism (PE), presented to the Emergency Department with a two-day history of dyspnea and hemoptysis. Exam was remarkable for sinus tachycardia, grade 3 systolic murmur at the apex, and increased work of breathing with bilateral pulmonary rhonchi. Computed tomography (CT) scan demonstrated centrally predominant opacities without signs of PE. Bronchoscopy was significant for diffusely hemorrhagic airways with progressively hemorrhagic serial lavage aliquots. Emergent delivery was performed in the setting of Preeclampsia while high-dose steroids were administered for DAH. The patient returned to baseline over the next 3 days without returning signs of hemoptysis. Lupus anticoagulant was positive but PT and PTT were both normal. Steroids were discontinued and therapeutic enoxaparin was started for a possible novel diagnosis of APS.Six days later, she reexhibited hemoptysis. Repeat CT revealed bilateral alveolar infiltrates consistent with the recurrence of DAH. Disproportionate left lower extremity edema and a femoral vein DVT were also discovered. Anticoagulation was again withheld, high-dose steroids were re-started, and an inferior vena cava filter was placed. Repeat lupus anticoagulant testing was positive. In addition, the dilute russell viper venom time (DRVV) was prolonged in the setting of normal PT/PTT, confirming APS diagnosis. She recovered within five days and was prescribed warfarin for chronic anticoagulation, glucocorticoids were tapered, while rituximab infusions were initiated as glucocorticoid-sparing therapy. DISCUSSION: This case highlights challenges associated with the diagnosis and management of rare simultaneous manifestations of APS. In particular, the management of DAH in this scenario is unique because unlike the glucocorticoid-responsive antibodies of ANCA-associated vasculitides, aPL are not known to cause alveolar capillaritis; therefore, the role of steroids is unclear. CONCLUSIONS: Chronic anticoagulation is an absolute requirement for long-term management of APS. Yet as this case exemplifies, in the case of concomitant DAH, administration of glucocorticoids, and subsequently glucocorticoid-sparing immunosuppression is recommended as the safest approach to avoid life-threatening hemorrhage. REFERENCE #1: Cartin-Ceba R, Peikert T, Ashrani A, Keogh K, Wylam ME, Ytterberg S, Specks U. Primary antiphospholipid syndrome-associated diffuse alveolar hemorrhage. Arthritis Care Res (Hoboken). 2014 Feb;66(2):301-10. doi: 10.1002/acr.22109. PMID: 23983016. REFERENCE #2: Stoots SA, Lief L, Erkan D. Clinical Insights into Diffuse Alveolar Hemorrhage in Antiphospholipid Syndrome. Curr Rheumatol Rep. 2019 Sep 6;21(10):56. doi: 10.1007/s11926-019-0852-7. PMID: 31493005; PMCID: PMC7102334. REFERENCE #3: Janowiak P, Sieminska A, Porzezinska M, Smolenska Z, Suchanek H, Jassem E. Difficulties in the treatment of recurring diffuse alveolar hemorrhage accompanying primary antiphospholipid syndrome: a case report and literature review. Adv Respir Med. 2018;86(3). doi: 10.5603/ARM.2018.0020. PMID: 29960279. DISCLOSURES: No relevant relationships by Eric Knott, source=Web Response no disclosure on file for Eric Lang; No relevant relationships by Claudia Saborit, source=Web Response

A RARE CASE OF EXTRA-PULMONARY ANTHRACOSIS

TOPIC: Occupational and Environmental Lung Diseases TYPE: Medical Student/Resident Case Reports INTRODUCTION: We present a unique case of anthracosis, common in coal workers, which caused FDG-PET/CT positive mediastinal lymphadenopathy. The majority of cases are associated with malignancy, infectious processes, granulomatous disease, and benign conditions [1]. The exact prevalence of this disease is difficult to estimate because many people with anthracosis are asymptomatic, hence, many cases of anthracosis are discovered incidentally [2]. Endobronchial ultrasonography (EBUS) with transbronchial needle aspiration (TBNA), has been an important method frequently used both in the diagnosis and staging of mediastinal lesions with suspected malignancy and in the initial investigation of the diagnosis of benign lesions [3]. CASE PRESENTATION: 80-year-old male who was referred to the pulmonary clinic after an incidental finding of a lung nodule. The patient reported no symptoms at the time of visit. Past medical history was insignificant. Social history was pertinent for a 55 pack-year smoking history with last use one year before the presentation. The patient was a retired plumbing excavator. There was no known history of tuberculosis. Physical examination was unremarkable. Quantiferon testing was indeterminate, angiotensin-converting enzyme (ACE) test and fungal serum markers were negative. A recent CT chest without contrast identified a left lung nodule and mediastinal lymph nodes. A Follow-up FDG-PET scan demonstrated small and mildly enlarged hypermetabolic mediastinal lymph nodes. The right lower paratracheal lymph node measured 1.6 x 1.1 cm and had an SUV ranging from 7.8-10.7. EBUS guided lymph node biopsy showed found carbon-containing histiocytic aggregates and no malignant cells on cytopathology of the paratracheal lymph nodes, 4L, 4R. DISCUSSION: Extra-pulmonary anthracosis is a rare finding that requires accurate lymph-node sampling to establish a diagnosis. It's commonly due to the inhalation of coal dust, smoke, or pollution [4]. Our case is unique because not all elevated SUV is associated with malignancy and the bronchoscopy didn't show any anthracotic pigmentation or any narrowing of the airways [5]. CONCLUSIONS: We highlight the importance of complete workup for incidental nodules found on routine imaging. Based on the absence of malignant, granulomatous, and infectious processes in the presence of carbon-containing histiocytic aggregates, we confidently diagnosed extra-pulmonary anthracosis. REFERENCE #1: Hewitt RJ, Wright C, Adeboyeku D, Ornadel D, Berry M, Wickremasinghe M, Wright A, Sykes A, Kon OM. Primary nodal anthracosis identified by EBUS-TBNA as a cause of FDG PET/CT positive mediastinal lymphadenopathy. Respir Med Case Rep. 2013 Sep 27;10:48-52. Doi: 10.1016/j.rmcr.2013.09.005. PMID: 26029513; PMCID: PMC3920429. DISCLOSURES: No relevant relationships by Rabia Anees, source=Web Response No relevant relationships by Rosa Arancibia, source=Web Response No relevant relationships by Andrew Doodnauth, source=Web Response No relevant relationships by Khawaja Omar, source=Web Response

A CASE OF SHRINKING LUNG SYNDROME ASSOCIATED WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND SJÖGREN SYNDROME

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Shrinking lung syndrome (SLS) is a rare disease that presents with progressive dyspnea, pleuritic chest pain, and restrictive lung disease. Most commonly seen with systemic lupus erythematosus (SLE), but also seen in other rheumatologic conditions such as Sjogren's syndrome, scleroderma, and rheumatoid arthritis. We present a case of a 28-year-old female with shrinking lung syndrome in the setting of SLE and Sjogren's Syndrome. CASE PRESENTATION: A 28-year-old female with an 18-year history of SLE with +ANA > 1:2560, +Smith, + SS-A/SS-B, and +dsDNA >379, presented with six months of dyspnea on exertion. Her other medical history included moderate mitral valve regurgitation, Sjogren's syndrome, depression, and hypothyroidism. Her SLE was managed with prednisone 7.5 mg daily, hydroxychloroquine, and mycophenolate, with a SELENA-SLEDAI score of 18, indicating high disease activity. Evaluation of her dyspnea included a high-resolution chest CT showing pleural thickening with normal lung parenchyma and an echocardiogram with evidence of moderate mitral valve regurgitation with normal ejection fraction. Pulmonary function testing revealed severe restrictive disease with TLC 1.6 L (39% of predicted), DLCO 6.23 ml/min/mmHg (24%), FEV1 0.91 L (32%) and FVC 1.13 L (34%). Right heart catheterization (RHC) was notable for PCWP 19, mPAP 28, PVR 160.75 dynes, CO 5.65 L/min concerning for pulmonary hypertension. DISCUSSION: Shrinking lung syndrome is a rare condition with a reported incidence in patients with SLE of 0.26% to 1.1%, with a female predominance up to 94% in some case series. First described by Hoffbrand and Beck in 1965, SLS commonly presents with pleuritic chest pain, dyspnea, diaphragmatic elevation, and restrictive lung disease. The diagnosis of SLS requires evidence of reduced lung volumes and DLCO in the setting of normal lung parenchyma. The pathogenesis and pathophysiology are still unknown and may be a result of phrenic nerve paralysis, alveolar microatelectasis, and hyaline membrane formation, or pleural thickening and adhesions. Most cases respond to high-dose steroids and/or immunosuppression with stabilization or improvement in lung function in the majority of patients. One case series showed a 95% clinical improvement in patients treated with steroids and/or immunosuppressants; however partial chronic restriction remained. CONCLUSIONS: This patient demonstrated severe restrictive disease with normal lung parenchyma. Although the reduced DLCO seen in our patient's pulmonary function testing could be seen with pulmonary hypertension, the severe restriction with normal parenchyma is consistent with a diagnosis of shrinking lung syndrome. She is currently undergoing treatment for her lupus with prednisone and immunosuppressants. REFERENCE #1: Carmier D, Diot E, Diot P. Shrinking lung syndrome: recognition, pathophysiology and therapeutic strategy. Expert Rev Respir Med 2011;5(1):33-9. REFERENCE #2: Duron L, Cohen-Aubart F, Diot, E, et al. Shrinking lung syndrome associated with systemic lupus erythematosus: a multicenter collaborative study of 15 new cases and a review of the 155 cases in the literature focusing on treatment response and long-term outcomes. Autoimmun Rev 2016;15(10):994-1000. REFERENCE #3: Deeb M, Tselios K, Gladman DD, et al. Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience. Lupus 2018;27(3):365-371. DISCLOSURES: Consultant relationship with Boehringer Ingelheim Please note: 12 months Added 04/20/2021 by Ryan Boente, source=Web Response, value=Consulting fee No relevant relationships by Matthew Glick, source=Web Response

A PRIMARY EMPYEMA OF UNKNOWN DURATION DUE TO ANAEROBIC GRAM-NEGATIVE BACILLUS HAFNIA ALVEI

TOPIC: Chest Infections TYPE: Medical Student/Resident Case Reports INTRODUCTION: Empyema is the presence of pus or bacteria in the pleural space. Incidence is increasing gradually with higher rates in the elderly with a greater prevalence in males [1]. Empyema is a fatal infection with a mortality rate of 20% [2]. Anaerobic Enterobacteriaceae accounting for less than 6% of empyema cases, are an infrequent cause [1]. CASE PRESENTATION: An 80-year-old male with a history of coronary disease, hypertension, hyperlipidemia, peripheral vascular disease, moderate aortic stenosis, a former smoker was seen at the pulmonary clinic for intermittent dyspnea with right-sided abdominal fullness of unknown duration and no symptoms of infection. He had significant asbestos exposure from his shipyard work. Around six months back, he underwent superior mesenteric artery (SMA) stenting for chronic abdominal angina. He was taking a super enzyme supplement to enhance his digestion. Chest x-ray review revealed right-sided effusion, which was also present before SMA stenting (Figure A1-2). A diagnostic thoracentesis showed brownish-green fluid with analysis indicative of empyema (Figure B). His blood work was normal. He was admitted, and a pigtail catheter was inserted into the right pleural space with purulent fluid drainage. He also underwent fibrinolysis. The pleural fluid culture grew Hafnia alvei sensitive to quinolones, aminoglycoside, and carbapenems (Amp C resistance pattern). At discharge, computed tomography and x-ray of the chest revealed empyema resolution, and the chest tube was removed (Figure C1-2). The initial antibiotic ertapenem was transitioned to levofloxacin for one month at discharge. At four weeks after discharge, his symptoms had entirely resolved DISCUSSION: H. alvei is a gram-negative facultative motile anaerobe of the Enterobacteriaceae family, a commensal in the gastrointestinal (GI) tract of humans and animals. It colonizes the oropharynx in humans [3]. It produces a cytolytic toxin, a virulent factor, causing acute gastroenteritis. It is a rare cause of nosocomial and community-acquired infection with a preference for biliary tree infection and has lower mortality [4-5]. In patients with multiple comorbidities and immunosuppressed, it causes extraintestinal diseases such as endophthalmitis, bronchopneumonia, gastroenteritis, and meningitis [5]. The infection source is the GI tract via gut translocation or oropharynx venous drainage draining into the heart and forced into the visceral pleura or alveolar capillary bed [5-6]. In our patient, another source could be the contaminated supplement (it contains Ox bile) CONCLUSIONS: In primary empyema of remote duration and an infrequent cause, a possible source could be the GI tract or the oropharynx. Clinicians should be aware of supplement contents that could be a source of infection REFERENCE #1: Godfrey MS, Bramley KT, Detterbeck F. Medical and Surgical Management of Empyema. Semin Respir Crit Care Med. 2019;40(3):361-74 REFERENCE #2: Corcoran JP, Wrightson JM, Belcher E, DeCamp MM, Feller-Kopman D, Rahman NM. Pleural infection: past, present, and future directions. Lancet Respir Med. 2015;3(7):563-77 REFERENCE #3: Fazal BA, Justman JE, Turett GS, Telzak EE. Community-acquired Hafnia alvei infection. Clin Infect Dis. 1997;24(3):527-8 DISCLOSURES: No relevant relationships by Phillip Beck, source=Web Response no disclosure on file for Wilson Grace; No relevant relationships by Ethan Karle, source=Web Response No relevant relationships by AARON MILLER, source=Web Response No relevant relationships by Shakuntala Patil, source=Web Response No relevant relationships by SACHIN PATIL, source=Web Response No relevant relationships by ANTONY RAWINDRARAJ, source=Web Response

PEDIATRIC PLASTIC BRONCHITIS IN THE SETTING OF SICKLE CELL DISEASE TREATED WITH INHALED TISSUE PLASMINOGEN ACTIVATOR

TOPIC: Pediatrics TYPE: Medical Student/Resident Case Reports INTRODUCTION: Plastic bronchitis is seen in congenital heart disease, cystic fibrosis, asthma, and less commonly sickle cell disease. It has high morbidity and can be difficult to treat. CASE PRESENTATION: We present an 11-year-old African American boy with past medical history of hemoglobin SS disease, acute chest syndrome, pain crises, and obstructive sleep apnea status post tonsillectomy and adenoidectomy who presented with vaso-occlusive pain crisis consisting of chest pain. CXR was clear without consolidation. He was placed on a pain regimen of morphine and ketorolac. Acute chest prophylaxis was initiated with albuterol plus PEP every 4 hours, incentive spirometry, and oxygen saturation maintenance ≥95%. Over the next two days he developed fevers, increased oxygen requirement, and crackles. CXR progressed to consolidation of left lung. He was transferred to the Pediatric ICU for BiPAP and given 2 units pRBCs, 7 days ceftriaxone, and 5 days azithromycin for acute chest treatment. Despite intense respiratory treatments while on BiPAP with albuterol every 4 hours, dornase alfa every 12 hours, CPT every 4 hours, and home mometasone/formoterol every 12 hours, his CXR demonstrated persistent opacification of left lung. Pulmonology was consulted for bronchoscopy which demonstrated large white mucoid material in the left mainstem consistent with plastic bronchitis. The cast was unable to be removed on initial bronchoscopy. He was initiated on inhaled tissue plasminogen activator 4 times daily in addition to above respiratory treatments. Bronchoscopy was repeated two days later and cast was successfully removed. BiPAP and oxygen were weaned as tolerated and he was discharged home on mometasone/formoterol twice daily. DISCUSSION: Plastic bronchitis describes endobronchial plugs of rubber-like casts in the bronchial trees. It is more commonly seen in patients with congenital heart disease after single-ventricle palliation. There have been various treatments trialed including bronchoscopy, chest physiotherapy, inhaled steroids, hypertonic saline, dornase alfa, nebulized heparin, lymphatic embolization, and inhaled tissue plasminogen activator. Studies performed on casts have demonstrated a substantial fibrin component supporting inhaled tissue plasminogen activator as a potentially effective treatment option. CONCLUSIONS: We present a case of pediatric plastic bronchitis in the setting of a patient with sickle cell disease. This case emphasizes that plastic bronchitis must be considered in patients diagnosed with acute chest. Additionally, inhaled tissue plasminogen activator may be considered as a treatment modality in pediatric patients with plastic bronchitis, particularly in patients who are unable to undergo bronchoscopy or when it has been unsuccessful for removal. REFERENCE #1: Feray S, Mora P, Decavele M, Pham T, Hafiani EM, Fartoukh M. Plastic bronchitis: An unusual complication of acute chest syndrome in adult. Respir Med Case Rep. 2017 Apr 7;21:93–5. REFERENCE #2: Li Y, Williams RJ, Dombrowski ND, Watters K, Daly KP, Irace AL, et al. Current evaluation and management of plastic bronchitis in the pediatric population. Int J Pediatr Otorhinolaryngol. 2020 Mar 1;130:109799. REFERENCE #3: Heath L, Ling S, Racz J, Mane G, Schmidt L, Myers JL, et al. Prospective, Longitudinal Study of Plastic Bronchitis Cast Pathology and Responsiveness to Tissue Plasminogen Activator (tPA). Pediatr Cardiol. 2011 Dec;32(8):1182–9. DISCLOSURES: No relevant relationships by Megan Lilley, source=Web Response No relevant relationships by Carla Roberts, source=Web Response No relevant relationships by Heather Staples, source=Web Response

GASTRIC ADENOCARCINOMA MASQUERADING AS CHRONIC COUGH

TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Chronic cough in adults is defined by cough lasting for at least eight weeks in duration. It remains one of the most common symptoms encountered in ambulatory care, largely attributed to Upper Airway Cough Syndrome (UACS), Asthma, Non-asthmatic Eosinophilic Bronchitis (NAEB), or Gastroesophageal Reflux Disease (GERD). Other less common etiologies shouldn't be overlooked, such as those disorders which stimulate cough receptors. These receptors are located throughout the body including the gastrointestinal tract;irritation of any of these can trigger the vagal-mediated afferent limb of the cough reflex arc. We present the case of a patient with gastric cancer with an initial complaint of three months of cough with associated dyspnea. CASE PRESENTATION: A 33-year-old male with a medical history of obstructive sleep apnea, prior tobacco use, and GERD presented with a three month history of dry cough, fatigue, and dyspnea. Previous primary care visits were conducted via telehealth amid the COVID-19 pandemic, where he was empirically treated with therapies for UACS, asthma, and reflux disease without improvement. His physical exam and chest radiograph were unremarkable. Pulmonary function tests were not obtained secondary to profound coughing with forced exhalation maneuvers. On further questioning, he endorsed early satiety, 10-pound weight loss, and a family history of stomach cancer. CT of the chest demonstrated questionable gastric thickening, retroperitoneal lymphadenopathy surrounding the mesenteric root, a nine millimeter lytic lucency along the right periphery of the 11th thoracic vertebral body, and subtle diffuse peripheral tree-in-bud pulmonary opacities. Bronchoscopy with lavage, brushings, and endobronchial biopsies were negative for infection, granulomas, or malignancy. Upper endoscopy revealed a laterally spreading lesion with central ulceration in the gastric cardia, with biopsy consistent with poorly differentiated gastric adenocarcinoma. Stage IV disease was confirmed by retroperitoneal lymph node sampling. DISCUSSION: Gastric cancer represents 1.4% of all new cancer cases annually in the U.S., most frequently in the sixth decade of life in those with multiple risk factors. Our patient presented nearly three decades earlier with a chronic cough which is exceedingly rare. Plausible risk factors for our patient include male sex, Hispanic ethnicity, obesity, prior tobacco use, and familial predisposition. Early symptoms are equivocal but usually include abdominal pain and weight loss. Respiratory symptoms generally portend late-stage metastasis to the lungs or pulmonary embolism, which our patient did not exhibit. CONCLUSIONS: While UACS, Asthma, NAEB, and GERD remain the most conceivable etiologies of chronic cough, fully investigating all red flag symptoms during patient encounters, with consideration of uncommon causes, endure as foundational tenets of chronic cough evaluation. REFERENCE #1: Irwin RS, French CL, Chang AB, Altman KW, CHEST Expert Cough Panel*. Classification of cough as a symptom in adults and management algorithms: chest guideline and expert panel report. Chest. 2018;153(1):196-209. REFERENCE #2: Kou A, Patel JK, Adetula I, Frunzi J. Gastric cancer presenting as persistent pneumonia: an unusual case report. Cureus. Published online March 21, 2021. REFERENCE #3: Polverino M, Polverino F, Fasolino M, Ando F, Alfieri A, De Blasio F. Anatomy and neuro-pathophysiology of the cough reflex arc. Multidiscip Respir Med. 2012;7(1):5. DISCLOSURES: No relevant relationships by Patrick Kicker, source=Web Response No relevant relationships by Ashley Soler Acevedo, source=Web Response

AN INCEDENTAL NODULE: ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS

TOPIC: Chest Infections TYPE: Medical Student/Resident Case Reports INTRODUCTION: Repeated inhalation of aspergillus spores can trigger a hypersensitivity immune reaction in susceptible hosts, producing allergic bronchopulmonary aspergilloses (ABPA) with a range of findings. Patients may be asymptomatic, or have symptoms including fever, dry or productive cough, dyspnea, chest pain, eosinophilia, radiographic infiltrates, as well as extensive lung disease and respiratory failure. Here we present a case of APBA discovered incidentally as a pulmonary nodule. CASE PRESENTATION: A 54-year-old female with asthma, hepatitis C and cirrhosis presented with melena. Variceal banding, transfusions, and supportive care stabilized her condition. Admission chest x-ray demonstrated a left pulmonary nodule. CT chest revealed a 2.9 cm left upper pulmonary nodule with tree-in-bud appearance and mucoid impaction. The patient had no respiratory signs or symptoms. The patient was discharged with nebulizers and pulmonary follow up. Outpatient bloodwork showed elevated absolute eosinophils. Pulmonary function tests demonstrated obstructive defect with reduced DLCO. Repeat CT chest confirmed diffuse left upper lobe tree-in-bud opacities and mucus plugging, with new bronchiectasis and wall thickening in the right middle, anterior right lower, and left lower lobes, suggestive of atypical infection. Although still asymptomatic, airway clearance and further workup were initiated. A bronchoscopy with lavage and endobronchial ultrasound with transbronchial needle aspiration demonstrated abundant eosinophils on biopsy with positive universal PCR for aspergillus. Gram stain, AFB and fungal cultures were negative. Preliminary diagnosis of ABPA was made. For the first time, the patient noted symptoms of cough, headache and back pain. Confirmatory testing showed aspergillus precipitans IgG and elevated total IgE. Negative aspergillus galactomannan antigen, and negative coccidioides, blastomyces dermatitidis, and histoplasma antibodies clarified the ABPA diagnosis. Prednisone and itraconazole were arranged alongside CT chest in 3-4 months. DISCUSSION: This patient with cirrhosis and asthma had decreased lung function, elevated serum IgE, aspergillus fumigatus precipitans, progressive CT infiltrates, bronchiectasis and mucous plugging. These findings strongly suggest ABPA. Although initially without respiratory symptoms, given irreversible airway dilation, therapy was initiated to reduce inflammation and prevent/slow progression into fibrotic or cavitary disease. CONCLUSIONS: In patients with asthma and recurrent infiltrates on chest imaging, one should consider the diagnosis of ABPA, even if asymptomatic. Early identification and treatment can reduce symptoms, slow progression or prevent permanent airway damage. Azoles reduce fungal burden, steroids suppress immune hyperreactivity, and radiologic/serum total IgE surveillance assist monitoring. REFERENCE #1: Shah A, Panjabi C. Allergic Bronchopulmonary Aspergillosis: A Perplexing Clinical Entity. Allergy Asthma Immunol Res. 2016 Jul;8(4):282-97. doi: 10.4168/aair.2016.8.4.282. PMID: 27126721; PMCID: PMC4853505. REFERENCE #2: Kosmidis C, Denning DW. The clinical spectrum of pulmonary aspergillosis. Thorax. 2015 Mar;70(3):270-7. doi: 10.1136/thoraxjnl-2014-206291. Epub 2014 Oct 29. PMID: 25354514. REFERENCE #3: Agarwal R, Sehgal IS, Dhooria S, Aggarwal AN. Developments in the diagnosis and treatment of allergic bronchopulmonary aspergillosis. Expert Rev Respir Med. 2016 Dec;10(12):1317-1334. doi: 10.1080/17476348.2016.1249853. Epub 2016 Nov 7. PMID: 27744712. DISCLOSURES: No relevant relationships by Sami Bashour, source=Web Response No relevant relationships by Philip Lavere, source=Web Response no disclosure on file for Dipaben Modi

PULMONARY PARENCHYMAL AND PLEURAL INVOLVEMENT BY MULTIPLE MYELOMA

TOPIC: Disorders of the Pleura TYPE: Fellow Case Reports INTRODUCTION: Extramedullary multiple myeloma (EMM) is an uncommon complication that presents in only 7% of patients with multiple myeloma. Common sites of involvement include the gastrointestinal and central nervous system. Pulmonary infiltration is very rarely seen, accounting for less than 1% of patients with multiple myeloma and is indicative of poor overall short-term survival. Here we present a case of pulmonary multiple myeloma with parenchymal as well as pleural involvement diagnosed via cytology and flow cytometry. CASE PRESENTATION: A 71-year-old female with multiple myeloma presented to oncology clinic noting progressive dyspnea, 15lb weight loss, and night sweats in the preceding month. She was admitted to our hospital where CT of her chest demonstrated diffuse ground glass opacities with central predominance and associated septal thickening, a moderate-sized right pleural effusion, and innumerable lytic lesions. She underwent diagnostic and therapeutic thoracentesis which was exudative with 67% lymphocytosis. Pleural fluid cytology was positive for malignant cells associated with IgG lambda-restricted multiple myeloma, and flow cytometry demonstrated a 48% population of plasmacytoid cells. Bronchoscopy ruled out opportunistic infection and alveolar lavage had only a few nucleated cells with a normal differential. Cytology showed rare malignant cells suspicious for involvement by multiple myeloma. Our patient expired approximately 1 month after this diagnosis despite rapid initiation of chemotherapy. DISCUSSION: Most commonly seen thoracic abnormalities with myeloma are osteolytic lesions, plasmacytomas, and pulmonary infiltrates as a result of infection. Infiltration of plasma cells within the parenchyma or pleural space is rarely seen and considered a poor prognostic factor. In one study of 958 patients with multiple myeloma seen within the thorax, parenchymal myelomatous infiltration and myelomatous effusions were noted in only 24 and 8 patients, respectively. Our patient with advanced disease presented with both parenchymal and pleural infiltration by plasma cells which was readily diagnosed via pleural fluid analysis and alveolar lavage using cytology and flow cytometry. In other instances, diagnosis may be more challenging and require either pleural biopsy or VATS if clinical suspicion remains despite non-diagnostic work-up. CONCLUSIONS: Diffuse pulmonary opacities and pleural effusions due to myelomatous infiltration can be diagnosed via analysis of pleural & alveolar lavage samples using cytology and flow cytometry. This is unfortunately associated with rapid progression and poor overall survival, even despite institution of therapy. REFERENCE #1: Lok R, Golovyan D, Smith J. Multiple myeloma causing interstitial pulmonary infiltrates and soft-tissue plasmacytoma. Respir Med Case Rep. 2018;24:155-157. REFERENCE #2: Kim YJ, Kim SJ, Min K, et al. Multiple myeloma with myelomatous pleural effusion: a case report and review of the literature. Acta Haematol. 2008;120(2):108-111. REFERENCE #3: Kintzer JS, Rosenow EC, Kyle RA. Thoracic and pulmonary abnormalities in multiple myeloma. A review of 958 cases. Arch Intern Med. 1978;138(5):727-730. DISCLOSURES: No relevant relationships by Brandon Jakubowski, source=Web Response Scientific Medical Advisor relationship with Boehringer Ingelheim Please note: May 2021 (one time) Added 04/20/2021 by Corey Kershaw, source=Web Response, value=Consulting fee No relevant relationships by Margaret Kypreos, source=Web Response

HEAD ABOVE WATER: A RARE CASE OF SCALP ANGIOSARCOMA YIELDING SPONTANEOUS HYDROPNEUMOTHORAX

TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Angiosarcomas are rare, highly aggressive neoplasms originating from vascular or lymphatic endothelium [1]. Cutaneous angiosarcoma of the scalp has a predilection to involve pleural/subpleural surfaces [2,3]. Able to induce pneumothorax, hemothorax, and pleural effusion, few reports link angiosarcoma of the scalp to cystic lung disease [3]. Herein, we present a case of cutaneous angiosarcoma of the scalp associated with cystic lung disease and hydropneumothorax. CASE PRESENTATION: An 80-year-old Asian man with a pertinent medical history of smoking, atrial fibrillation, hypertension, and coronary artery disease presented with 2 weeks of dyspnea and cough. Additionally, he endorsed the worsening of a large fungating scalp lesion for the past 3 months. Biopsy of the lesion was diagnostic of well-differentiated and atypical cell angiosarcoma of the scalp. This admission, CXR showed left-sided, moderate hydropneumothorax. CT thorax revealed new cystic/cavitary disease bilaterally, not present on prior imaging. The patient was administered 100% FiO2 via NRB with no resolution of pneumothorax. Subsequent thoracocentesis produced exudate. Upon worsening of symptoms, bronchoscopy with biopsy was performed, and chest tube placement drained voluminous serosanguinous fluid. BAL, quantiferon gold, AFBx2, fungal culture, and ova/parasite all returned negative. No evidence of metastatic disease resulted from biopsy. Upon patient stabilization and without significant reaccumulation of fluid/hydropneumothorax, he was discharged. Follow-up with pulmonology/CVT was recommended for small-bore catheter placement and possible pleurodesis. Eventually, pleural fluid analysis identified the presence of nontuberculous mycobacteria. DISCUSSION: Constituting only 2% of all soft tissue sarcomas, angiosarcoma has a propensity to metastasize to the lung [2]. Our patient presented with cystic lung disease and spontaneous hydropneumothorax with no evidence of metastases from the primary site. A series of Japanese autopsy data compared scalp vs. non-scalp angiosarcoma, in which pneumothorax was solely observed in patients with the scalp subtype [4]. Only 24 cases of angiosarcoma with spontaneous pneumothorax have been reported from 1978-2014, with fewer cases documented since [5]. Limited reports have linked angiosarcoma to spontaneous hydropneumothorax, as seen in our case. CONCLUSIONS: Angiosarcoma of the scalp has a predilection to yield pulmonary sequelae. Considering the scarcity of documented cases, such diagnosis should be considered in patients presenting with cystic lung disease or hydropneumothorax. The eventual identification of concurrent nontuberculous mycobacterium infection within our patient is remarkable. Mycobacteria actuating cystic lung disease is unorthodox [6]. However, the distinct etiology of our patient's presentation may have been multifactorial in nature, compounding the originality of this case. REFERENCE #1: Somasekharan Nair KK, Zabell AS, Vo KL, Shaikh MA. Pneumothorax: a classical presentation of metastatic scalp angiosarcoma. Ann Thorac Surg. 2012 Sep;94(3):e77-8. doi: 10.1016/j.athoracsur.2012.02.030. PMID: 22916786.2. Andersen KF, Albrecht-Beste E, Berthelsen AK, Loft A. Angiosarcoma of the Scalp: Metastatic Pulmonary Cystic Lesions Initially Misinterpreted as Benign Findings on 18F-FDG PET/CT. Diagnostics (Basel). 2015 Dec 22;6(1):1. doi: 10.3390/diagnostics6010001. PMID: 26838798; PMCID: PMC4808816. REFERENCE #2: Olsen D, Molloy C, Sriram PS. Rapidly progressive lung cysts and pleural effusion: a case report. Case Rep Pulmonol. 2011;2011:790274. doi: 10.1155/2011/790274. Epub 2011 Sep 8. PMID: 22937430; PMCID: PMC3420654.4. Kitagawa M, Tanaka I, Takemura T, Matsubara O, Kasuga T. Angiosarcoma of the scalp: report of two cases with fatal pulmonary complications and a review of Japanese autopsy registry data. Virchows Arch A Pathol Anat Histopathol. 1987;412(1):83-7. doi: 10.1007/BF00750735. PMID: 3120405. REFERENCE #3: Chang JH, Kim JH, Hong SH, Song ME, Ryu YJ, Lee JH, Shim SS, Cho MS, Sim YS. Angiosarcoma presenting with spontaneous hydropneumothorax: report of a case and review of the literature. Open Respir Med J. 2014 Dec 26;8:48-54. doi: 10.2174/1874306401408010048. PMID: 25614772; PMCID: PMC4296473.6. Baldi BG, Carvalho CRR, Dias OM, Marchiori E, Hochhegger B. Diffuse cystic lung diseases: differential diagnosis. J Bras Pneumol. 2017 Mar-Apr;43(2):140-149. doi: 10.1590/S1806-37562016000000341. PMID: 28538782; PMCID: PMC5474378. DISCLOSURES: No relevant relationships by Nicholas Barreras, source=Web Response No relevant relationships by Azim Hemani, source=Web Response No relevant relationships by Michael Markos, source=Web Response No relevant relationships by Travis Yamanaka, source=Web Response

SWEET SYNDROME WITH PULMONARY INVOLVEMENT

TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Sweet syndrome (acute neutrophilic dermatosis) typically presents with cutaneous lesions, fever, and arthralgia. Sweet syndrome with pulmonary involvement is rare and to our knowledge, only 40 cases have been reported in the literature. In this case, we will highlight the clinical and radiographic findings seen in Sweet syndrome with pulmonary involvement. CASE PRESENTATION: A 58-year-old male with past medical history of GERD presented to the ED with worsening SOB and a nonproductive cough. He endorsed recent completion of Levofloxacin therapy for presumed pneumonia. Vital signs were notable for fever of 39º C. Patient was tachypneic and required 3L NC for O2 saturations > 92%. PE revealed decreased LLL sounds as well as erythematous nodules noted on bilateral lower extremities. CBC showed elevated WBC of 18.2. CMP and urinalysis were within normal limits. CT Chest showed bilateral ground glass opacities and minimal pleural effusions. IV Vancomycin and Zosyn were initiated for community acquired pneumonia coverage, however patient continued to spike fevers. Blood and urine cultures remained negative. On day eight of admission, bronchoscopy was performed. BAL and transbronchial biopsy showed extensive neutrophilic infiltrate. Skin biopsy also showed a dense dermal infiltrate composed of neutrophils. Four days after the biopsy, patient developed acute hypoxic respiratory failure requiring ICU admission for intubation. CT Chest showed worsening bilateral pleural effusions and ground glass opacities. Intravenous methylprednisolone was started with rapid improvement in patient's respiratory and cutaneous symptoms. Patient was eventually transitioned to oral prednisone. DISCUSSION: In this case, we present a patient with histopathological and radiographic findings of Sweet syndrome with pulmonary involvement. Our patient fulfills two major and at least two minor criteria of Sweet syndrome including abrupt onset of tender erythematous nodules and a dense dermal neutrophilic infiltrate without leukocytoclastic vasculitis on histology. Minor criteria included fever > 38º C, elevated ESR, neutrophilia, and a dramatic response to treatment with steroids. Prompt transbronchial and skin biopsies in combination with an excellent response to corticosteroid therapy confirmed the diagnosis of Sweet syndrome with pulmonary involvement. Our patient continues to require daily steroid therapy to prevent recurrence of symptoms. CONCLUSIONS: Overall, the aim of this case is to recognize the clinical and radiographic findings of pulmonary Sweet syndrome and to confirm the diagnosis based on the clinical criteria presented. Early initiation of corticosteroid therapy can result in favorable patient outcomes as discussed in this case. REFERENCE #1: Robbins CM, Mason SE, Hughey LC. Sweet Syndrome With Pulmonary Involvement in a Healthy Young Woman. Arch Dermatol. 2009;145(3):344–346. doi:10.1001/archdermatol.2008.610 REFERENCE #2: Fernandez-Bussy S, Labarca G, Cabello F, Cabello H, Folch E, Majid A. Sweet's syndrome with pulmonary involvement: Case report and literature review. Respir Med Case Rep. 2012;6:16-19. Published 2012 Oct 17. doi:10.1016/j.rmcr.2012.08.004 REFERENCE #3: Nofal A, Abdelmaksoud A, Amer H, Nofal E, Yosef A, Gharib K, Albalat W, Eldesouky F, Ebrahim HM, Abdelshafy AS, Fayed H. Sweet's syndrome: diagnostic criteria revisited. J Dtsch Dermatol Ges. 2017 Nov;15(11):1081-1088. doi: 10.1111/ddg.13350. Epub 2017 Oct 5. PMID: 28981179. DISCLOSURES: No relevant relationships by Huma Khan, source=Web Response

CHRONIC CAVITARY PULMONARY ASPERGILLOSIS IN A PATIENT WITH IDIOPATHIC PULMONARY FIBROSIS

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Chronic cavitary pulmonary aspergillosis (CCPA) is a rare form of aspergillosis involving patients with structural lung diseases. Here we will describe a case of CCPA in a patient with idiopathic pulmonary fibrosis (IPF), which is rarely reported. CASE PRESENTATION: The patient was a 78-year-old male with a medical history significant for IPF diagnosed 13 years prior, on oral pirfenidone and long-term oxygen therapy, who presented to the hospital for worsening shortness of breath, productive cough, and fatigue of two months duration. The patient was initially hospitalized in another facility for the above symptoms where he received two courses of antibiotics without significant improvement. Chest examination showed bilateral fine inspiratory crackles, worse on the right side. The results of routine blood tests were normal. The nasal swab for the respiratory viral panel was negative. Chest X-ray showed fibrotic lung disease with a superimposed alveolar and interstitial process involving the lung bases and right upper lobe. Computed Tomography (CT) scan of the chest was suggestive of a thick-walled cavitary lesion in the right apex measuring 4.8 x 3.3 cm, enlarged mediastinal and right hilar lymph nodes along with other chronic changes. Three sets of sputum acid-fast bacilli (AFB) smears along with serum Quantiferon TB gold plus test were negative. The patient was empirically started on intravenous (IV) ceftriaxone and azithromycin. Later, blood and sputum cultures were reported negative for bacteriological growth. Interestingly, the sputum cultures were positive for Aspergillus species along with positive serum Aspergillus immunoglobulin (Ig)G level. The patient was diagnosed with CCPA based on the serology, culture results, and radiological findings. IV antibiotics were discontinued and the patient was started on oral posaconazole 300 mg daily resulting in clinical improvement. Currently, the patient is on a 6-month posaconazole regimen. DISCUSSION: Pulmonary aspergillosis is a common opportunistic infection that can present as aspergilloma, allergic bronchopulmonary aspergillosis, CCPA, and invasive pulmonary aspergillosis based on lung changes. Mycobacterium infection and obstructive lung diseases are the most frequent underlying pulmonary diseases that are associated with CCPA. However, IPF with CCPA is rare, and its clinical features and radiological findings lack specificity leading to delays in diagnosis and treatment. Once confirmed, the treatment is straightforward with long-term triazole antifungals. CONCLUSIONS: In a patient with IPF, presenting with rapidly progressing radiologic findings suggestive of pulmonary cavitation, CCPA should be considered in the differential diagnosis. REFERENCE #1: Liu C, Yang J, Lu Z. Idiopathic pulmonary fibrosis with chronic necrotizing pulmonary aspergillosis: a case report. Int J Clin Exp Pathol. 2019 Jul 1;12(7):2653-2656. PMID: 31934094; PMCID:PMC6949565 REFERENCE #2: Graham KG, Nasir A. Chronic Cavitary Pulmonary Aspergillosis: A Case Report and Review of the Literature. Am J Case Rep. 2019 Aug 18;20:1220-1224 doi: 10.12659/AJCR.915893. PMID: 31422416; PMCID: PMC6711266. REFERENCE #3: Sehgal IS, Dhooria S, Muthu V, Prasad KT, Agarwal R. An overview of the available treatments for chronic cavitary pulmonary aspergillosis. Expert Rev Respir Med. 2020 Jul;14(7):715-727. doi: 10.1080/17476348.2020.1750956. Epub 2020 Apr 20. PMID: 32249630. DISCLOSURES: No relevant relationships by Rabab Elmezayen, source=Web Response No relevant relationships by Syed yousaf Shah, source=Web Response