Diffuse Ground-glass Opacities Research Articles

An atypical cause of dyspnea

A 53-year old woman was admitted to hospital with a six-month history of cough and dyspnea, weight loss and left abdominal and lumbar pain. Inspiratory crackles were diffusely heard during physical examination. The chest radiography revealed bilateral symmetric opacities. Chest CT-scan showed bilateral crazy paving patterns with diffuse ground-glass opacities, inter and intralobular septal thickening and alveolar infiltrates with peripheral sparing. Bronchoscopy with bronchoalveolar (BAL) returned abundant opaque and milky lavage fluid (Fig.

Granulomatous and lymphocytic interstitial lung disease

A middle-aged woman with common variable immunodeficiency (CVID) presented with progressive dyspnoea and fevers. She had a temperature of 38.9°C, oxygen saturation of 91% on room air and decreased breath...

SAT-507 Pneumocystis Carinii Pneumonia: A Rare Cause of Granulomatous Hypercalcemia

Pneumocystis Carinii pneumonia (PCP) is a well-known complication of immunosuppression. Scattered case reports have linked PCP and its ability to induce a granulomatous response to hypercalcemia. PCP related hypercalcemia appears to be resistant to standard therapy. We report a case of hypercalcemia that preceded PCP and continued to worsen during the course of infection. A 63y man with renal transplant for polycystic kidney disease one year prior, presented with a three week history of fatigue, cough and chills. Patient was hypoxic and CT of the thorax revealed diffuse ground glass opacities. He was started on empiric therapy for PCP with intravenous methylprednisolone, clindamycin, and primaquine. Laboratory studies revealed a serum calcium of 12 mg/dl (baseline 9.2mg/dl, reference range 8.6-10.4 mg/dl) and creatinine of 3.23 mg/dl, which rose from a baseline value of 1.6 mg /dl. The patient’s bronchoalveolar lavage confirmed PCP. Endocrinology was consulted for evaluation of hypercalcemia. Further investigations revealed a suppressed PTH of 15 pg/ml from a baseline of 97 pg/ml (reference range 15-65pg/ml) post-transplant, 25-hydroxyvitamin D level of 30 ng/ml (reference range >20 ng/ml ), and 1,25-dihydroxyvitamin D(1,25D) level was elevated (>156 pg/ml; reference range 20-79 pg/ml). A diagnosis of 1,25D mediated hypercalcemia was made, intravenous fluids started and high dose steroids continued. Serum calcium levels improved transiently but subsequently rose to a peak level of 13.5 mg/dl. Ketoconazole 200 mg every 8hrs was started to reduce 1,25D production. Serum calcium remained high despite a reduction in 1,25D level (33 pg/ml). Bisphosphonates therapy was considered unsafe because of decreased GFR. Therefore, denosumab 30mg was administered, which resulted in decrease in serum calcium level to 10.3 mg/dl by day 19. Improvement of hypercalcemia correlated with improvement of PCP and renal function. Patient was discharged home after completing the 21 day course of treatment for PCP. Five weeks later, serum calcium stayed normal with an elevated PTH of 153 pg/ml and 1,25D level of 20 pg/ml. Hypercalcemia heralding PCP infection has been reported in the literature. Elevated calcium of 10.6 mg/dl was present one month prior to our patient’s hospitalization around the time of onset of his symptoms. Of the 19 cases of hypercalcemia due to PCP infection, 5 had hypercalcemia that preceded PCP infection by few weeks. The gold standard for diagnosis of PCP involves identification of the organism in induced sputum or bronchoalveolar lavage specimen. Measurement of serum 1,3-β-d-Glucan, which has high sensitivity, may be used as a screening tool in the right clinical setting such as our patient with immunosuppression and hypercalcemia to diagnose PCP at an earlier stage. We believe that hypercalcemia in a patient with immunosuppression should alert the possibility of PCP infection.

PULMONARY INVOLVEMENT IN INTRAVASCULAR LARGE B-CELL LYMPHOMA

PURPOSE: To describe the clinical and radiologic correlates of pulmonary involvement with intravascular large B Cell Lymphoma (IVLBCL). METHODS: We retrospectively identified 26 patients with IVLBCL encountered between 1998-2017 at Mayo Clinic, Rochester, Minnesota. We analyzed five of these patients (19%) who had pulmonary involvement confirmed histopathologically (3 surgical lung biopsy, 2 autopsy). RESULTS: Median age was 68 years (range 44-73) and included 1 woman. On presentation, 4 (80%) patients had respiratory complaints including dyspnea, hypoxia, cough, and tachypnea. Chest radiography failed to demonstrate parenchymal infiltrates in all patients. Chest computed-tomography (CT) was obtained in 4 patients revealing intrathoracic adenopathy in all 4 patients and diffuse ground glass opacities in 1 (25%) patient. Two patients underwent positron emission tomography (PET) scanning before treatment and fluorodeoxyglucose (FDG) avidity was noted in intrathoracic adenopathy in one patient; neither manifested parenchymal FDG avidity. Pulmonary function test obtained in 3 patients demonstrated reduced diffusing capacity of the lungs for carbon monoxide (DLCO) in all. Two patients were diagnosed to have IVLBLC only at autopsy. Remaining 3 patients were initiated on chemotherapy; two died within a few months after initiating treatment, one remains in remission at four year follow-up. CONCLUSIONS: IVLBCL is a rare form of non-Hodgkin lymphoma that is difficult to diagnose and pulmonary involvement may be radiologically occult including on CT and PET scanning. CLINICAL IMPLICATIONS: Absence of radiologic abnormalities on CT or PET scanning does not exclude the diagnosis of IVLBCL in patients presenting with respiratory symptoms, decreased diffusing capacity, and intrathoracic adenopathy.

Combined-modality therapy for pulmonary alveolar proteinosis in a remote setting: a case report

BackgroundPulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of phospholipoproteinaceous material in the alveoli. The presentation is nonspecific but typically includes dyspnea; the spectrum of disease includes rapidly progressive hypoxic respiratory failure. Whole lung lavage (WLL) is the treatment of choice in symptomatic PAP, but transient worsening of oxygenation sometimes requires salvage modalities of support such as extracorporeal membrane oxygenation (ECMO). Granulocyte macrophage colony-stimulating factor (GM-CSF) plays a role in the pathophysiology of PAP. We highlight a case of severe PAP treated with exogenous GM-CSF and sequential lobar lavage due to the unavailability of salvage methods of oxygenation.Case presentationA 36 year old female was admitted with fevers, chills, and progressive dyspnea. On presentation she was tachypneic, tachycardic, and hypoxemic; labs revealed leukocytosis and lactic acidosis. Chest CT identified diffuse ground glass opacities in a ‘crazy-paving’ pattern. Following intubation due to impending respiratory failure, bronchoscopy with bronchoalveolar lavage was performed. The lavage return stained positive with Periodic Acid Schiff, confirming the diagnosis of PAP. Continued deterioration necessitated treatment; however, at this geographically remote center without ECMO services WLL was judged to carry significant risk. Nebulized GM-CSF was administered without significant improvement. Subcutaneous GM-CSF was administered and isolated subsegmental lavages of the bilateral upper lobes were performed, with rapid improvement in oxygenation. Additional sequential lobar lavage and continued GM-CSF therapy as an outpatient resulted in complete resolution of oxygen requirement and return to normal pulmonary physiology.ConclusionsThe autoimmune form of PAP is the most common, indicating that therapy with GM-CSF may play an important role for many patients. Treatment with WLL may be impractical in some clinical settings due to the expertise and salvage modalities required. Sequential lobar lavage requires less specialized expertise and may incur less risk of refractory hypoxemia. We posit that this combined-modality therapy is ideally suited to geographically-remote centers such as our own.

Hyaluronic acid-induced diffuse alveolar hemorrhage: unknown complication induced by a well-known injectable agent

Hyaluronic acid (HA) is widely used in medicine, especially for contouring or volumizing soft tissue and intra-articular injection. However, it is not well known that HA injection can cause life-threatening pulmonary complications. We recently experienced a case of a woman who was diagnosed with diffuse alveolar hemorrhage (DAH) after receiving an illegal vaginal HA filler injection. A 54-year-old female visited our clinic complaining of hemoptysis and dyspnea. Chest computed tomographic (CT) scan showed diffuse ground glass opacities and consolidation in both lower lobes. Bronchoalveolar lavage (BAL) disclosed hemorrhagic feature in sequential samples compatible with DAH. There were no relevant drugs or past medical histories, and no positive result in autoantibodies tests. During detailed history taking, she recalled that she got an illegal HA filler injection in her vaginal wall performed by nonmedical personnel the very day before the onset of symptoms. Although the procedure itself or an additive of HA preparation could be a cause, HA that might have been introduced into circulation was thought to be the most possible cause for the development of DAH. The clinical symptoms and lung lesions were dramatically improved after the treatment with systemic steroid. Pulmonary complications after HA injections were scarcely reported. Only one case of HA-related DAH has been previously described so far. Our present case emphasizes that physician and other health care providers must be aware of possible pulmonary complications of this most widely-using injectable agent.

Intravascular NK/T-cell lymphoma, Epstein\u2013Barr virus positive with multiorgan involvement: a clinical dilemma

BackgroundIntravascular lymphoma is a rare type of non-Hodgkin lymphoma mostly of B-cell lineage. A few cases of intravascular lymphoma have been found to be of NK/T-cell origin, mainly affecting the skin and central nervous system.Case presentationA 54-year-old Caucasian man sought care because of a 2 weeks history of jaundice and intermittent fever, not responsive to antibiotics and antipyretics. Laboratory tests showed low blood oxygen concentration and pancytopenia. Serum microbiological tests were negative. Computerized tomography (CT) scan revealed hepatosplenomegaly and diffuse ground-glass opacities in both lungs without interlobular septal thickening. Despite oxygen therapy, the clinical conditions rapidly deteriorated leading to death 3 days after admission. Autopsy revealed a multiorgan involvement by an Epstein-Barr virus positive NK/T-cell lymphoma, strikingly growing within the blood vessel lumina, in absence of skin lesions.ConclusionsThe current case highlights the pathological features of this rare entity, the protean clinical presentation of which is often misleading, resulting in delayed diagnosis and treatment.

INTRAVENOUS, BUT NOT ORAL, STEROID REQUIRED TO CONTROL EOSINOPHILIA IN HYPEREOSINOPHILIC SYNDROME

Introduction Hypereosinophilic syndrome (HES) is a condition resulting in peripheral eosinophilia (>1500 cells/μL) with end organ damage. Flares of this disease can be controlled with corticosteroids, although alternate agents are typically used for baseline control. Case Description A 59-year-old woman with HES with pulmonary involvement, presented to the emergency department with hemoptysis, dyspnea, and fever. The patient had been tapering her prednisone dose of 60mg daily and was also taking hydroxyurea 500mg BID. Upon presentation, her eosinophil count was 1670 cells/μL. CT revealed diffuse groundglass opacities throughout the lungs. Bronchoalveolar lavage (BAL) revealed 55% eosinophils. Methylprednisolone 80mg IV every 8 hours was administered for 4 days. The patient clinically improved and her eosinophil count dropped to 370 cells/μL. Alternate steroids were attempted with the goal of switching to an oral route for taper and discharge. Intramuscular triamcinolone, intravenous dexamethasone and oral prednisone were attempted. The patient failed all therapies with rising eosinophilia to peak of 6320 cells/μL. She was administered mepolizumab 300mg subcutaneously for long-term control of eosinophilia and to facilitate tapering of methylprednisolone. She was discharged with a peripherally inserted central catheter for a 4 week taper. Her eosinophil count has since remained zero while on mepolizumab. Discussion This case illustrates a scenario where outpatient therapy with intravenous methylprednisolone was needed to treat eosinophilic lung involvement in HES. Use of intravenous methylprednisolone therapy may be considered as bridge to steroid sparing therapies. Treatment with mepolizumab is very effective for treatment of HES and can spare patients of long-term corticosteroid therapy and associated side effects. Hypereosinophilic syndrome (HES) is a condition resulting in peripheral eosinophilia (>1500 cells/μL) with end organ damage. Flares of this disease can be controlled with corticosteroids, although alternate agents are typically used for baseline control. A 59-year-old woman with HES with pulmonary involvement, presented to the emergency department with hemoptysis, dyspnea, and fever. The patient had been tapering her prednisone dose of 60mg daily and was also taking hydroxyurea 500mg BID. Upon presentation, her eosinophil count was 1670 cells/μL. CT revealed diffuse groundglass opacities throughout the lungs. Bronchoalveolar lavage (BAL) revealed 55% eosinophils. Methylprednisolone 80mg IV every 8 hours was administered for 4 days. The patient clinically improved and her eosinophil count dropped to 370 cells/μL. Alternate steroids were attempted with the goal of switching to an oral route for taper and discharge. Intramuscular triamcinolone, intravenous dexamethasone and oral prednisone were attempted. The patient failed all therapies with rising eosinophilia to peak of 6320 cells/μL. She was administered mepolizumab 300mg subcutaneously for long-term control of eosinophilia and to facilitate tapering of methylprednisolone. She was discharged with a peripherally inserted central catheter for a 4 week taper. Her eosinophil count has since remained zero while on mepolizumab. This case illustrates a scenario where outpatient therapy with intravenous methylprednisolone was needed to treat eosinophilic lung involvement in HES. Use of intravenous methylprednisolone therapy may be considered as bridge to steroid sparing therapies. Treatment with mepolizumab is very effective for treatment of HES and can spare patients of long-term corticosteroid therapy and associated side effects.

Pneumocystis jirovecii pneumonia in AIDS and non-AIDS immunocompromised patients - an update.

Pneumocystis jirovecii (PJ) pneumonia (PJP) is an important opportunistic infection affecting various types of immunocompromised patients and is associated with an increased risk of mortality. PJ is a unique fungal pathogen which is increasingly common and maybe associated with a higher mortality rate in patients without AIDS. We present the characteristics of PJP, diagnosis, and treatment outcomes between AIDS and non-AIDS patients. We conducted a review of studies of AIDS and non-AIDS patients with PJP using PubMed to search for studies until December 2017. The annual incidence of AIDS-PJP decreased from 13.4 to 3.3 per 1000 person-years in industrialized countries, while the incidence of non-AIDS-PJP varied widely. Both groups had similar clinical manifestations and radiological features, but the non-AIDS-PJP group potentially had a more fulminant course, more diffuse ground glass opacities, and fewer cystic lesions. The mortality rate decreased in the AIDS-PJP group after the advent of antiretroviral therapy; however, the mortality rate remained high in both groups. A laboratory diagnosis was usually nonspecific; CD4+ T-cell < 200 cells/mL or < 14% favored AIDS-PJP. Serum 1,3-β-D-glucan (BDG) had a high diagnostic odds ratio. Combining BDG and lactic dehydrogenase improved the diagnosis of AIDS-PJP. Histopathological staining and polymerase chain reactions could not discriminate infection from colonization when the result was positive. The use of antibiotics, prophylaxis, and adjunctive corticosteroids was controversial. Early diagnosis and treatment can be achieved through vigilance, thereby improving the survival rate for PJP in immunocompromised patients.

Acute exacerbation of idiopathic pulmonary fibrosis

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is defined as an acute clinically significant respiratory deterioration characterized by evidence of new, widespread alveolar abnormalities, such as diffuse bilateral ground-glass opacification and/or consolidation, and the absence of other obvious clinical causes like fluid overload, left heart failure, or pulmonary embolism, etc. AE-IPF is subcategorized as “triggered” (where specific causes are identified, for example, infections, surgery procedures, drug toxicity, etc.) or “idiopathic” (where no specific causes are identified). In randomized trials, the annual incidence of AE-IPF is about 8%, in retrospective studies it reaches 19%. Severe forms of IPF are an important risk factor for the development of AE-IPF. In-hospital mortality from AE-IPF is more than 50%, and the average survival of patients with AE-IPF is 1–4 months. Currently, there remain no proven, effective therapies for AE-IPF. In real clinical practice patients with AE-IPF still receive high doses of systemic corticosteroids and antibiotics. Antifibrotic therapy can reduce the risk of exacerbations; it has been shown that therapy with nintedanib leads to a reduction in the number of confirmed/suspected AE-IPF by 68%. It is necessary to further study the potential methods of prevention and therapy of AE-IPF in future clinical trials.

Assessment and agreement of the CT appearance pattern and its severity grading of radiation-induced lung injury after stereotactic body radiotherapy for lung cancer.

PurposeRadiographic severity of radiation-induced lung injury (RILI) has not been well-studied. The goal of this study was to assess the CT appearance pattern and severity of RILI without consideration of the clinical presentation.Material and methodsA total of 49 patients, 41 with primary lung cancer and 8 with metastatic lung cancer, were treated by 4-fraction stereotactic body radiotherapy (SBRT). RILI after SBRT was separately assessed by two observers. The early and late CT appearance patterns and CT-based severity grading were explored.ResultsThe median follow-up period was 39.0 months. In the early CT findings of observers 1 and 2, there was diffuse consolidation in 15 and 8, diffuse ground glass opacity (GGO) in 0 and 0, patchy consolidation and GGO in 17 and 20, patchy GGO in 3 and 3, and no changes in 10 and 14, respectively (kappa = 0.61). In late CT findings of observer 1 and 2, there were modified conventional pattern in 28 and 24, mass-like pattern in 8 and 11, scar-like pattern in 12 and 12, and no changes in 1 and 2, respectively (kappa = 0.63). In the results of the CT-based grading by observers 1 and 2, there were grade 0 in 1 and 2, grade 1 in 10 and 14, grade 2 in 31 and 29, grade 3 in 7 and 4, and none of grade 4 or more, respectively (kappa = 0.66). According to multivariate analyses (MVA), the significant predicting factors of grade 2 or more CT-based RILI were age (p = 0.01), oxygen dependence (p = 0.03) and interstitial shadow (p = 0.03).ConclusionsThe agreement of the CT appearance and CT-based grading between two observers was good. These indicators may be able to provide us with more objective information and a better understanding of RILI.

HIGH DOSE CORTICOSTEROIDS IN ACUTE MANAGEMENT OF ACUTE INTERSTITIAL PNEUMONIA

SESSION TITLE: Critical Care 3 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Little advancements have been made in the management of acute interstitial pneumonia (AIP) since its discovery. This case report describes the use of high dose corticosteroids in the resolution of presumed AIP. CASE PRESENTATION: The patient is a middle aged, African American, nonsmoking female with a past medical history of morbid obesity and obstructive sleep apnea complicated by noncompliance with nightly CPAP who presented with a combination of fevers, chills and emesis. She was initially treated with vancomycin and pipercillin-tazobactam. Her hospital course was rapidly complicated by fluid refractory hypotension, which required ICU transfer for vasopressor therapy. At the time of transfer, the patient was found to be profoundly hypoxemic requiring the initiation of high flow nasal cannula at a rate of 55 liters per minute and 100% FiO2. Plain chest film at that time was notable for hypoinflation without other abnormalities. Chest CT completed shortly thereafter revealed a few small ground glass opacities in the bilateral apices, small pleural effusions and dependent compressive atelectasis of the lower lobes. Within 24 hours, the patient developed diffuse airspace disease on plain film chest imaging. When she failed to respond to aggressive diuresis, concern for a potential noninfectious, inflammatory process was considered. This diagnosis was furthered when repeat chest CT imaging demonstrated multilobar, diffuse ground glass opacities with septal thickening and consolidations in the setting of a sedimentation rate greater than 100. She was then started on high dose steroids (methylprednisolone 500mg IV every 6 hours) for presumed acute interstitial pneumonia. Repeat CT chest completed four days into the course of corticosteroid therapy revealed near complete resolution of the radiographic abnormalities. The patient was also weaned off supplemental oxygen over the same time period and was ultimately discharged on a prolonged prednisone taper. DISCUSSION: AIP is often defined as a rapidly progressive respiratory failure in the setting of bilateral radiographic infiltrates and an absence of a known, underlying inciting cause. While the diagnosis of AIP requires a surgical lung biopsy demonstrating organizing diffuse alveolar damage, it too often is a diagnosis of exclusion given the severity of most patients' illness. Corticosteroids have been shown to inhibit proinflammatory mediators and limit the fibrotic response seen in the pathogenesis of AIP. Patients are believed to require deliberate steroid tapers to prevent reoccurrence. CONCLUSIONS: This case report reaffirms the notion that high dose corticosteroid therapy can be useful in the acute management of AIP. There are currently limited treatment options for AIP, and given the high mortality associated with AIP more advanced therapies need to be investigated. Reference #1: Avnon LS, Pikovsky O, Sion-vardy N, Almog Y. Acute interstitial pneumonia-Hamman-Rich syndrome: clinical characteristics and diagnostic and therapeutic considerations. Anesth Analg. 2009;108(1):232-7. Reference #2: Suh GY, Kang EH, Chung MP, et al. Early intervention can improve clinical outcome of acute interstitial pneumonia. Chest. 2006;129(3):753-61. Reference #3: Vourlekis JS. Acute interstitial pneumonia. Clin Chest Med. 2004;25(4):739-47, vii. DISCLOSURES: No relevant relationships by Seth Brant, source=Web Response No relevant relationships by Jared Dyer, source=Web Response

PNEUMONITIS SECONDARY TO EBV-SPECIFIC CYTOTOXIC T-LYMPHOCYTES

SESSION TITLE: Diffuse Lung Disease 1 SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/07/2018 03:30 pm - 04:30 pm INTRODUCTION: Novel immune based therapies are now being used for a wide range of malignancies including post-transplantation lymphoproliferative disorders (PTLD), however, the short and long term risk of these therapies remains unclear. Here, we present a case of a patient who developed hypoxemia one day after receiving an infusion of EBV-specific cytotoxic T-lymphocytes. CASE PRESENTATION: A 66-year-old male presented to the hospital with 4 days of progressive dyspnea, cough, and hypoxemia. He had a history of end stage renal disease and EBV-positive cadaveric renal transplantation which was complicated by EBV viremia and PTLD. Two months prior, the patient began therapy with HLA-matched EBV-specific T-lymphocytes after failing several immunosuppressive and chemotherapeutic regimens. His most recent infusion was 1 day prior to symptom onset. On admission, oxygen saturation was 91% on room air and 96% on 2 liters of oxygen by nasal cannula. Physical exam at that time was notable for faint crackles throughout all lung fields. Chest radiograph was negative for infiltrates or effusions although computed tomography imaging of the chest revealed diffuse bilateral ground glass opacities and patchy airspace disease with an upper lobe predominance (see Figure 1). Bronchoscopy with transbronchial biopsy revealed interstitial inflammation and fibrosis, hyperplasia of type II pneumocytes, and scattered foci of organizing pneumonia consistent with active pneumonitis. Bronchoalveolar lavage demonstrated a lymphocyte predominant cell distribution with 56% lymphocytes, 41% macrophages, and 3% neutrophils. BAL and biopsy were negative for viral, fungal, and bacterial elements. With supportive care alone, the patient improved over the course of 5 days and was discharged to a short term rehabilitation facility. DISCUSSION: PTLD is a serious complication for patients undergoing organ transplantation. EBV-specific cytotoxic T-cells have shown promising results in the treatment of some of these patients, however, emerging evidence suggests that pulmonary complications are a consequence of this therapy. Indeed, in a series of 39 patients there was 1 reported death due to pneumonitis. Moreover, outcomes in another study of 114 patients found that 1 patient developed severe pulmonary infiltrates within one week of receiving EBV-specific T-cells. This patient did not suffer long-term sequelae from this complication. Our case suggests that pneumonitis from EBV-specific T-lymphocytes is characterized by a histopathological pattern of organizing pneumonia and a lymphocyte predominant cellular distribution. CONCLUSIONS: EBV-specific cytotoxic T-cell therapy causes acute pneumonitis in some patients. Additional studies will be important to understand the true incidence of pneumonitis after EBV-specific therapies, and for determining the factors that predispose or prevent the development of this therapeutic complication. Reference #1: Heslop, H. E., Slobod, K. S., Pule, M. A., et al. Long-term outcome of EBV-specific T-cell infusions to prevent or treat EBV-related lymphoproliferative disease in transplant recipients. Blood. 2010; 115(5):925-935. Reference #2: Rooney, C. M., Smith, C. A., Ng, C. Y., et al. Infusion of Cytotoxic T Cells for the Prevention and Treatment of Epstein-Barr Virus–Induced Lymphoma in Allogeneic Transplant Recipients. Blood. 1998; 92(5):1549-1555. Reference #3: Dierickx, D., and Habermann, T.M. Post-Transplantation Lymphoproliferative Disorders in Adults. N Engl J Med. 2018; 378:549-562. DISCLOSURES: No relevant relationships by Robert Hilton, source=Web Response No relevant relationships by Carl Nieweld, source=Web Response No relevant relationships by ross summer, source=Web Response

AN EVANESCENT RIGHT ATRIAL MASS FROM CATHETER-RELATED THROMBOSIS

SESSION TITLE: Cardiovascular Disease 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: A diagnosis of catheter-related upper extremity deep vein thrombosis (CRUE-DVT) should be considered in the differential for right atrial mass in a patient with history of malignancy and presence of peripherally inserted central catheter (PICC) because the pulmonary embolism (PE) can be fatal even with anticoagulation.[1] CASE PRESENTATION: A 34 year old male with a history of diabetes mellitus, chronic kidney disease, testicular cancer status post orchiectomy with mediastinal metastatic seminoma resection, and intravenous drug use, presented with acute encephalopathy. He was admitted for diabetic ketoacidosis secondary to sepsis from osteomyelitis of his right foot and found to have MRSA bacteremia and candida glabrata fungemia. The patient continued to improve clinically until he developed acute hypoxic respiratory failure requiring ventilator support several days after admission. Physical exam was notable for tense right upper extremity at site of PICC without erythema or tenderness. Laboratory studies were notable for WBC of 21.5 with neutrophilia, and MRSA from right foot abscess culture and blood cultures. CT of thorax revealed extensive diffuse bilateral groundglass opacities with intralobular thickening and areas of consolidation, concerning for aggressive acute interstitial pneumonia. Bronchoalveolar lavage showed mildly elevated eosinophilia suggestive of eosinophilic pneumonia, for which the patient was treated with corticosteroids. A critical care transesophageal echocardiogram (TEE) was performed to evaluate for endocarditis and revealed a small gelatinous appearing mass contiguous with right atrial wall at the entrance of superior vena cava and interatrial shunt with suspected patent foramen ovale (Figure 1-3). Right upper extremity venous duplex ultrasonography showed completely thrombosed right basilic, brachial, axillary, and subclavian veins likely catheter-related with thrombus extension to right atrium.PICC was removed for concern of right atrial thrombus infection and anticoagulation was started. The repeat TEE performed 10 days later did not reveal a right atrial mass. The patient left the hospital against medical advice, with a therapeutic international normalized ratio but returned within 24 hours following a cardiac arrest. CT thorax angiography revealed PE with right ventricular strain, and the patient expired 48 hours later, with anoxic brain injury noted following resuscitation. DISCUSSION: CRUE-DVT should be considered with an evanescent right atrial mass, and a duplex ultrasonography should be considered because incidence of PE can be as high as 36 percent in patients with malignancy.[2,3] Optimal therapy is unknown although anticoagulation and infection-driven catheter removal appear to be the first line therapy. CONCLUSIONS: CRUE-DVT patients with ASD can develop PE and paradoxical arterial embolism, which can prove fatal, even with anticoagulation. Reference #1: Monreal M et al. Pulmonary embolism in patients with upper extremity DVT associated to venous central lines--a prospective study. Thromb Haemost. 1994;72(4):548. Reference #2: Muñoz FJ et al. Clinical outcome of patients with upper-extremity deep vein thrombosis: results from the RIETE Registry. Chest. 2008;133(1):143. Reference #3: Prandoni P et al. Upper-extremity deep vein thrombosis. Risk factors, diagnosis, and complications. Arch Intern Med. 1997;157(1):57. DISCLOSURES: No relevant relationships by Michel Boivin, source=Web Response No relevant relationships by Chih-Wei Chang, source=Web Response No relevant relationships by Nicholas Villalobos, source=Web Response

SHOE SHINING: HAZARDOUS TO THE HEALTH

SESSION TITLE: Occupational and Environmental Lung Diseases SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Hydrocarbons are ubiquitous in society and are found in many common household products, including gasoline, cleaning agents, mineral oil, and industrial solvents. In 2016, the American Association of Poison Control Centers reported 29,796 cases of hydrocarbon exposure, with approximately 20 deaths attributed to this exposure. There are three main types of hydrocarbon exposure: children with unintentional ingestion of household hydrocarbons, workers with dermal or inhalational occupational exposures, and adolescents and young adults with intentional inhalational abuse. CASE PRESENTATION: Our patient is a 40-year-old male sailor who presented to the emergency department with productive cough and dyspnea. Approximately two hours prior to arrival, the patient was waterproofing his boots in an enclosed space. On initial evaluation, his heart rate was in the 100s, respiratory rate in the 30s, and oxygen saturation in the 70s%. His exam was notable for diffusely diminished breath sounds. He was placed on 10 liters via nonrebreather. The Poison Control Center was contacted, and the inhaled toxin was identified as a petroleum-based hydrocarbon. A chest x-ray showed diffuse bilateral hazy opacities. He was admitted to the intensive care unit and placed on high-flow nasal cannula with flow and FiO2 titrated to maintain oxygen saturation greater than 92%. A high resolution CT scan of the chest showed diffuse bilateral upper and central lobe predominant groundglass opacities with subpleural sparing consistent with diffuse alveolar damage. Treatment with high dose steroids and bronchodilators was initiated, and over the next 48 hours the patient had significant improvement. He was discharged on a tapering dose of prednisone and supplemental oxygen at 4 liters per minute for exertion. After discharge, his spirometry showed a mild restrictive pattern with a mild reduction in DLCO; he no longer required supplemental oxygen. Follow up chest imaging is currently pending. DISCUSSION: The mechanisms by which hydrocarbons cause pulmonary toxicity are dose-dependent, and include alveolar and interstitial edema, desquamation of pneumocytes, and disruption of surfactant. Hypoxia results from a combination of ventilation-perfusion mismatch, shunt formation, and bronchospasm. Initial imaging may be normal, with delay of radiographic changes for several hours after the development of pneumonitis. Radiographic findings typically demonstrate bilateral interstitial infiltrates, though may also reveal pneumatocele, pneumothorax, pleural effusions, and lung necrosis; additionally, intra-alveolar oils can coalesce into nodules surrounded by fibrosis called parafinomas. While symptoms usually resolve within a few days, radiographic abnormalities may last months. Most cases of hydrocarbon lung toxicity resolve with supportive care of oxygen and ventilatory support, but there have been case reports of benefit from corticosteroids, whole or segmental lung lavage, and intratracheal surfactant in severe cases, though there is no firm evidence for these treatments. CONCLUSIONS: Hydrocarbons are found in several household products, making exposure to these items not uncommon. While the acute effects of hydrocarbon toxicity are well recognized, the long-term effects of acute and chronic exposure require further research. Reference #1: Connellan SJ. Lung diseases associated with hydrocarbon exposure. Resp Med 2017; 126:46-51. Reference #2: Gummin DD, Mowry JB, Spyker DA, Brooks DE, Fraser MO, Banner W. 2016 Annual Report of the American Association of Poison Control Centers’ National Poison Data System (NPDS): 34th Annual Report, Clin Toxicol 2017;55:(10)1072-1254. Reference #3: Tormoehlen LM, Tekulve KJ, Nanagas KA. Hydrocarbon toxicity: A review. Clin Toxicol 2014; 52:479-489. DISCLOSURES: No relevant relationships by Jeannette Collins, source=Web Response No relevant relationships by Angeline Lazarus, source=Web Response

UTILIZATION OF A PROMISING NOVEL TREATMENT PROTOCOL FOR AN ACUTE EXACERBATION OF IDIOPATHIC PULMONARY FIBROSIS ULTIMATELY INEFFECTIVE

SESSION TITLE: Diffuse Lung Disease 2 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 pm - 02:15 pm INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a fibrotic lower respiratory tract disease of spontaneous origin characterized by progressive dyspnea on exertion and a nonproductive cough that presents insidiously over months to years. Exacerbations of IPF reflect an acute deterioration in lung function and are often fatal despite mechanical ventilation, glucocorticoids, and antibiotics. Here, we present a 71-year-old woman diagnosed with IPF managed with a novel therapeutic strategy involving IVIG, plasma exchange, and rituximab that was ultimately ineffective. CASE PRESENTATION: A 71-year-old woman with squamous cell lung carcinoma (SCLC) and biopsy-proven IPF requiring 8L of supplemental oxygen at baseline initially presented with shortness of breath and was admitted for management of acute hypoxic respiratory failure suspected to be secondary to acute IPF exacerbation. The patient had a history of SCLC treated with right upper lobe resection and radiation one year before presentation. The diagnosis of IPF was made by a wedge resection of the right middle lobe. She was initially treated with nintedanib, which was discontinued due to gastrointestinal intolerance.Two days prior to admission, the patient developed progressive shortness of breath and hypoxia. On admission, she was severely hypoxic with O2 saturation of 85% on 50 L of O2 and 100% FiO2. Laboratory results showed leukocytosis of 13x109/L, hematocrit of 34%, platelets of 187 109/L, and procalcitonin < 0.05 ng/mL. A chest X ray was unrevealing. A CT chest with contrast showed diffuse ground glass opacities, chronic fibrotic changes, and multiple nodules; no evidence of pulmonary embolism. Respiratory samples were negative for any growth. She was managed with intravenous steroids, broad spectrum antibiotics, and diuretics. She showed initial improvement, but subsequently declined. Given the lack of evidence identifying a specific etiology of the presentation, a diagnosis of IPF exacerbation was made.After weighing the options for treatment considering her highly debilitated baseline state, a newly described protocol consisting of rituximab, IVIG, and plasmapheresis was selected. She completed nine sessions of plasma exchange with no improvement and given her continuing dyspnea and hypoxia, the patient and her family decided to pursue a comfort care approach. DISCUSSION: IPF exacerbations are severe, challenging to treat, and often prove fatal. In a novel eleven-patient pilot trial utilizing rituximab, plasma exchange, and IVIG, nine patients showed improvement in pulmonary gas exchange. Four patients were notably younger, and three patients did not require supplemental oxygen for up to a week prior to admission. CONCLUSIONS: This may indicate that advanced age and severity of baseline hypoxia, such as in our patient, serve as limiting factors to the above described approach. Reference #1: Donahoe M, Valentine VG, Chien N, et al. Autoantibody-targeted treatments for acute exacerbations of idiopathic pulmonary fibrosis. PLoS One. 2015;10(6). https://doi.org/10.1371/journal.pone.0127771. DISCLOSURES: No relevant relationships by Vinay Goswamy, source=Web Response no disclosure on file for Laurie Lerner; No relevant relationships by Ignacio Portales Castillo, source=Web Response No relevant relationships by Sumon Roy, source=Web Response No relevant relationships by harinder singh, source=Web Response

THE CURIOUS COUGH: A CASE REPORT OF CYSTIC LUNG DISEASE CAUSED BY BORDETELLA BRONCHISEPTICA IN A PATIENT WITH AIDS

SESSION TITLE: Chest Infections 3 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Bordetella bronchiseptica species are documented as commensals and a respiratory pathogen in a number of mammals,pertinently causing tracheobronchitis in dogs(i.e canine cough), atrophic rhinitis in pigs,and snuffles in rabbits.Since 1911,cases of B. bronchiseptica have been reported to cause respiratory infection in humans with underlying immunosuppression. Here we report a case of B.bronchiseptica pneumonia successfully treated with piperacillin-tazobactam & azithromycin. CASE PRESENTATION: A 24-year-old male was admitted to the ICU with a week history of fever,dry cough,post-tussive emesis, and hypotension with concern for sepsis.The patient had a history of perinatal HIV,CD4+ count on admission was <10 and the viral load was 109,295 cop/ml.He had been admitted with similar complaints twice in the past two months, primarily for chronic cough and fever.His vitals on admission were a fever of 38°C, BP 84/62 mmHg and HR 123.He had coarse breath sounds bilaterally, with crackles in the left lower chest.Chest imaging showed a cystic/cavitary lesion was noted measuring 5 x 2 cm in axial dimensions within the left lower lobe with an air-fluid level,and multiple small cystic lesions with a background of mild diffuse ground-glass opacity observed.Sputum culture and bronchoalveolar lavage fluid culture grew gram-negative coccobacillus that was identified as B.bronchiseptica. DISCUSSION: B. bronchiseptica naturally infects non-human mammals, unlike B.pertussis which is an obligate human pathogen.Reports of human B. bronchiseptica infections are limited to case reports or case report series; infection in humans usually requires immunosuppression or malignancy.The infection presents as a lobar pneumonia but often includes chronic cough > 1 month. The incubation period ranges from 1 - 8 days, and the infection can be transmitted from infected animals or humans through respiratory droplets.It has the potential to cause recurrent infection due to its ability to survive intracellularly and evade killing by macrophages due to acid tolerance.It expresses protein adhesions called filamentous hemagglutinin and pretactin that allows it to invade the respiratory epithelium where it can survive and resist antibody and compliment mediated phagocytosis,it does not produce the pertussis toxin,but causes local damage by adenylate cyclase hemolysin and dermonecrotic toxins. These factors allow it to persist for long durations and cause cavitary pneumonias. CONCLUSIONS: B. bronchiseptica is an animal pathogen and there are no guidelines on the management of human infection.Pulse-field get electrophoresis analysis by Celia et al. showed that it shows resistance for aminopenicillins, cephalosporins and aztreonam; but generally susceptible to macrolides and quinolones with total duration of antibiotic therapy 2 - 4 weeks and consideration for longer duration therapy in severely immunocompromised and neutropenic patients. Reference #1: García-de-la-Fuente C, Guzmán L, Cano ME, et al. Microbiological and clinical aspects of respiratory infections associated with Bordetella bronchiseptica. Diagnostic Microbiology and Infectious Disease. 2015;82(1):20-25. https://doi.org/10.1016/j.diagmicrobio.2015.01.011. Reference #2: Gueirard P, Weber C, Le Coustumier A, Guiso N. Human Bordetella bronchiseptica infection related to contact with infected animals: persistence of bacteria in host. J Clin Microbiol. 1995;33(8):2002-2006. Reference #3: Schneider B, Gross R, Haas A. Phagosome Acidification Has Opposite Effects on Intracellular Survival of Bordetella pertussis andB. bronchiseptica. Infect Immun. 2000;68(12):7039-7048. https://doi.org/10.1128/IAI.68.12.7039-7048.2000. DISCLOSURES: No relevant relationships by Janaki Deepak, source=Web Response No relevant relationships by Muhammad Sameed, source=Web Response No relevant relationships by Scott Sullivan, source=Web Response

Successful Steroid Therapy for Lipoid Pneumonia Developing After Allogeneic Hematopoietic Stem Cell Transplant: A Case Report

Lipoid pneumonia is an uncommon noninfectious inflammatory lung disease characterized by lipid deposition in the alveoli, and its etiology and treatment have not been elucidated. We report the case of a 32-year-old woman who developed lipoid pneumonia 9 months after allogeneic hematopoietic stem cell transplant for chronic myelogenous leukemia in lymphoid blast crisis. She complained of progressive cough and dyspnea shortly after discontinuation of immunosuppressive therapy given for graft-vs-host disease. Computed tomography demonstrated diffuse ground-glass opacities in the lungs, and pulmonary function test revealed restrictive impairment. Bronchoalveolar lavage fluid showed milky appearance, and transbronchial lung biopsy specimen revealed foamy macrophages infiltrating the alveoli. Based on these findings, she was diagnosed as having lipoid pneumonia. Prednisolone (1 mg/kg/d) promptly improved the symptoms, pulmonary shadows, and pulmonary function. The findings and clinical course of this case suggest that lipoid pneumonia should be recognized as one of the pulmonary complications of allogeneic hematopoietic stem cell transplantation.

Medically Clear

immunotherapy, cancer: immunotherapy, cancerFigureFigureA 69-year-old man with a history of cancer presented to our ED on day four of taking Ceftin for pneumonia with a fever of 102.5°F and shortness of breath. He had a history of non-small cell lung cancer after a wedge resection and hepatocellular carcinoma after a hepatectomy. His chest film was read as “patchy airspace disease over mid- and lower lung without improvement from prior,” and he was worked up, treated, and admitted for severe sepsis. Sounds like a fairly bread-and-butter emergency department case, right? Of course, there was a twist, which emerged after a CT of the chest and bronchoscopy demonstrated diffuse ground glass opacity and tracheobronchitis, findings that resolved with high-dose prednisone. The diagnosis was not pneumonia but pneumonitis, a complication of the patient's cancer treatment—pembrolizumab (Keytruda), last administered several months before presentation. Pembrolizumab is one of the new-generation cancer treatments that deploys the patient's immune system against cancer cells by removing the malignant cells' ability to disguise themselves rather than killing them directly. These checkpoint inhibitors are revolutionizing oncologic treatment, but are not a risk-free panacea. We all know where these patients are likely to be seen and evaluated when severe side effects emerge. Be on the Lookout Immunotherapy complications have been receiving some attention from scientific journals as well as the lay press this year. Actual data on adverse effect risk, especially for those with a delayed presentation like our patient, remain limited. An Annals of Emergency Medicine study gives us our best sense of the prevalence and nature of side effects in practice, albeit with data from a single and specialized ED setting. (Ann Emerg Med 2018 Jun 4; doi: 10.1016/j.annemergmed.2018.04.019.) The authors retrospectively reviewed records of patients over age 17 on immune checkpoint therapy who visited the University of Texas MD Anderson Cancer Center ED between March 2011 and March 2016. The study's primary goal was to characterize the nature and mortality risks of any adverse reactions to one or more of three immunotherapy medications (pembrolizumab, nivolumab [Opdivo], ipilimumab [Vervoy]). The study identified 1,026 visits among 628 patients, and 257 visits (25%) were related to one or more adverse effects. The list of these starts with diarrhea (12.7%) and continues in descending order of frequency: colitis, pneumonitis, dermatitis, hypophysitis, hepatitis, thyroiditis, pancreatitis, adrenalitis, myocarditis, and vasculitis. Pneumonitis posed the greatest survival risk (hazard ratio 1.72 [95% CI 1.03-2.87] in multivariable Cox regression modeling), and multiple drug therapy made certain specific reactions more likely (e.g., thyroiditis and hypophysitis) than single-drug therapy. We EPs should presume that any patient with active cancer could be on these medications, and we should be on the lookout for their adverse reactions. Fortunately, recent articles in Academic Emergency Medicine and Cureus addressed the physiology, grading, and treatment of checkpoint inhibitor therapies. (Acad Emerg Med 2018 May 5; doi: 10.1111/acem.13443; Cureus 2017;9[10]:e1774; http://bit.ly/2L5zgOk.) The American Society of Clinical Oncology and the National Comprehensive Cancer Network also published guidelines on these. (J Clin Oncol 2018;36[17]:1714; http://bit.ly/2L1RgZV.) Most important are the grading recommendations (1-4) with sensible management ramifications. The skinny is that minor toxicities (Grade 1) can usually be closely monitored without stopping treatment, but Grade 2 and above require immunosuppression (corticosteroid first-line, infliximab second-line) and holding or suspending immunotherapy altogether. The test with this practice-changer will be in the awareness and recall. We must recognize the at-risk patient and remember that delayed symptoms can be common. We penned a limerick to help us remember to add checkpoint inhibitors to the differential diagnosis checklist. (See sidebar.) Share this article on Twitter and Facebook. Access the links in EMN by reading this on our website or in our free iPad app, both available at www.EM-News.com. Comments? Write to us at [email protected].

Clinical Features of Pneumocystis Jiroveci Pneumonia in Patients with Inflammatory Bowel Disease

Objective To investigate the clinical features of patients with inflammatory bowel disease (IBD) complicated with Pneumocystis Jiroveci Pneumonia (PJP). Methods We retrospectively analyzed the clinical data of 5 patients who were hospitalized in Peking Union Medical College Hospital from January 2012 to July 2017 for treatment of IBD complicated with PJP. Demographic characteristics,clinical manifestations,treatments,and outcomes were descriptively analyzed. Results Of these five patients,four had ulcerative colitis (UC) and one had Crohn's disease (CD). All patients were males,with an average age of (61.8±1.9) years. All patients were in active disease status and had symptoms including cough and suffocation. Three patients had hypoxemia,among whom two developed type 1 respiratory failure. Three patients were treated with immunosuppressive medications (corticosteroids and/or immunosuppressant drugs) before the diagnosis of PJP. Lymphocyte counts in three patients were less than 0.6×109/L. CD4+T cells in two patients were less than 200×106/L. Four patients had elevated serum cytomegalovirus DNA. The level of β-D-glucan was elevated in four patients. Chest CT showed bilateral diffuse ground glass opacification. PJP-DNA was positive in sputum or bronchoalveolar lavage fluid in all patients. Two patients with type 1 respiratory failure required invasive mechanical ventilation. All patients received trimethoprim-sulfamethoxazole and methylprednisolone treatment. Four patients recovered completely and one died. Conclusion Elderly (aged>55 years) IBD patients who are receiving immune-suppressive therapy or with decreased peripheral blood lymphocyte count are at higher risk of PJP.