Eur Respir Research Articles

Bronchiectasis in patients with chronic obstructive pulmonary disease in a tertiary care center in North-East India

Bronchiectasis in patients with chronic obstructive pulmonary disease in a tertiary care center in North-East India

"Functional ageing in fibrotic interstitial lung disease: the impact of frailty on adverse health outcomes." Sabina A. Guler, Joanne M. Kwan, Janice M. Leung, Nasreen Khalil, Pearce G. Wilcox and Christopher J. Ryerson. Eur Respir J 2020; 55: 1900647.

Figures 1 and 2 in this article were published with the incorrect frailty index definitions for “frail” and “non-frail” subjects (in the key to the figures). Frail subjects were incorrectly defined as those with frailty index ≤0.21, and non-frail subjects with frailty index >0.21. The correct definitions of frail and non-frail subjects are those with frailty index >0.21 and ≤0.21, respectively, as presented in the amended figures below.

"Is bronchiectasis really a disease?" Michal Shteinberg, Patrick A. Flume and James D. Chalmers. Eur Respir Rev 2020; 29: 190051.

"Is bronchiectasis really a disease?" Michal Shteinberg, Patrick A. Flume and James D. Chalmers. Eur Respir Rev 2020; 29: 190051.

Are the Effects of High-Intensity Exercise Training Different in Patients with COPD Versus COPD+Asthma Overlap?

This study aimed to investigate whether patients with chronic obstructive pulmonary disease (COPD) presenting asthma overlap (ACO) benefit similarly in comparison to patients with only COPD after a 12-week high-intensity exercise training (ET) program. Subjects with a diagnosis of COPD alone or ACO were evaluated and compared before and after a high-intensity ET program composed of walking and cycling plus strengthening exercises of the upper and lower limbs (3days/week, 3months, 36 sessions). Assessments included spirometry, bioelectrical impedance, 6-min walk test (6MWT), London Chest Activity of Daily Living Scale (LCADL), Hospital anxiety and depression Scale, modified Medical Research Council Scale (mMRC), Saint George Respiratory Questionnaire (SGRQ), and respiratory and peripheral muscle strength [manovacuometry and 1-repetition maximum test (quadriceps femoris, biceps and triceps brachialis), respectively]. ACO was defined according to Sin et al. (Eur Respir J 48(3):664-673, 2016). The sample was composed of 74 subjects (57% male, age 67 ± 8years, BMI 26 (21-32) kg/m2, FEV1 47 ± 17%predicted), and 12 (16%) of them were classified as presenting ACO. Both groups improved pulmonary function, 6MWT, peripheral and inspiratory muscle strength, LCADL, and SGRQ after ET (p < 0.005 for all). There were no significant interactions between ACO and COPD on ET effects (p > 0.05 for all). Likewise, there was no difference in the proportion of patients achieving the minimum clinical important difference for 6MWT and mMRC. High-intensity exercise training generates similar benefits in patients with COPD regardless of whether presenting asthma overlap or not.

"Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis." Bridget F. Collins and Ganesh Raghu. Eur Respir Rev 2019; 28: 190022.

"Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis." Bridget F. Collins and Ganesh Raghu. Eur Respir Rev 2019; 28: 190022.

"Cellular mechanisms underlying steroid-resistant asthma." Ridhima Wadhwa, Kamal Dua, Ian M. Adcock, Jay C. Horvat, Richard Y. Kim and Philip M. Hansbro. Eur Respir Rev 2019; 28: 190021.

"Cellular mechanisms underlying steroid-resistant asthma." Ridhima Wadhwa, Kamal Dua, Ian M. Adcock, Jay C. Horvat, Richard Y. Kim and Philip M. Hansbro. Eur Respir Rev 2019; 28: 190021.

"Central sleep apnoea and periodic breathing in heart failure: prognostic significance and treatment options." Winfried Randerath, Oana Deleanu, Sophia Schiza and Jean-Louis Pepin. Eur Respir Rev 2019; 28: 190084.

"Central sleep apnoea and periodic breathing in heart failure: prognostic significance and treatment options." Winfried Randerath, Oana Deleanu, Sophia Schiza and Jean-Louis Pepin. Eur Respir Rev 2019; 28: 190084.

ES19.02 Best Endoscopic Tools for the Best Results

Undiagnosed pleural effusion is an increasingly common clinical problem and represents significant burden of disease both to patients and healthcare resources. With the increase in annual incidence of both primary and secondary pleural malignancies, better diagnostics and treatment options are very much needed. Medical thoracoscopy, also known as local anaesthetic thoracoscopy [2], is a procedure where a rigid or semi-rigid scope is inserted into the pleural cavity via a port for direct visualisation of the pleura and biopsy of abnormal areas, besides completion of talc poudrage, where appropriate. It is usually performed under conscious sedation and local anaesthesia. This procedure avoids risks of general anaesthesia and single lung ventilation, required for video-assisted thoracoscopic surgery (VATS) and therefore can be performed in patients who are unfit for anaesthesia/surgery. The procedure of thoracoscopy is performed in a controlled environment such as in an operating theatre setting, endoscopy suite or treatment room with adequate staffing. Diagnostic advantage A significant number of cases of pleural effusion are undiagnosed after a single diagnostic pleural aspiration and the diagnostic yield of pleural fluid cytology is only approximately 60% [3]. A second aspiration only modestly increases diagnostic yield by 15% and a third sample is non-contributory [3]. A blind pleural biopsy (also known as closed pleural biopsy) increases the diagnostic yield above pleural fluid cytology by only 7-27% [3]. In mesothelioma however, the diagnostic yield of pleural fluid cytology is even lower, at around 32% [4]. A blind pleural biopsy only increases sensitivity to around 50% [5]. Medical thoracoscopy is substantially superior in diagnostic power compared to pleural fluid cytology and blind pleural biopsy. As it allows direct visual assessment of the pleura and subsequent biopsy of the abnormal areas, it maximises diagnostic yield to >90% in malignant pleural diseases [5,6]. Rigid thoracoscopy generates similar diagnostic yield compared to semirigid thoracoscopy in exudative pleural effusions but larger biopsy samples can be obtained during rigid thoracoscopy [5,6]. The sensitivity of medical thoracoscopy in malignant mesothelioma appears to be equally good and the efficacy of rigid medical thoracoscopy in regards to diagnosis in pleural malignancy is as high as VATS [2]. With the increasing need to secure an accurate diagnosis and plan optimal treatment in possible pleural malignancy, medical thoracoscopy offers a high diagnostic yield earlier in the patient journey. Therefore, this is the preferred procedure where the option exists and helps to reduce the need for repeated diagnostic procedures and reduces the time taken to establish diagnosis and commence appropriate treatment. Medical thoracoscopy as a therapeutic procedure Another advantage of medical thoracoscopy is that it is a diagnostic and therapeutic procedure in the same setting. Complete drainage of pleural fluid can be achieved during the procedure and talc poudrage can also be performed during medical thoracoscopy. It is a highly effective method of pleurodesis with an efficacy of 84% at 1 month, which is at least equivalent to talc slurry via a seldinger chest drain, with possibly increased efficacy in the subgroup of patients who have breast or lung carcinoma and without trapped lung [2]. Medical thoracoscopy is also effective in the management of TB pleurisy and empyema. Septations and adhesions in complex infected effusions can be divided during thoracoscopy which can facilitate accurate chest tube placement and drainage. Advanced Thoracoscopy Techniques Narrow Band Imaging- using Semirigid Thoracoscope Autofluorescence Rigid Thoracoscopy Biopsy- with Insulated Tip Diathermy Knife Cryobiopsy- using Semirigid Thoracoscope Rigid Thoracoscope Semi-rigid Thoracoscope Diacon AH, Van de Wal BW, Wyser C, et al. Diagnostic tools in tuberculous pleurisy: a direct comparative study. Eur Respir J 2003;22:589e91. Rahman NM, Ali NJ, Brown G, Chapman SJ, O’Davies RJ, Downer NJ, Gleeson FV, Howes TQ, Treasure T, SinghS and Phillips GD Local anaesthetics thoracoscopy: British Thoracic Society pleural disease guideline 2010. Thorax 2010;65(Suppl 2):ii54-ii60 Mohan A, Chandra S, Agarwal D, Naik S and Munavvar M. Utility of semirigid thoracoscopy in the diagnosis of pleural effusions: a systematic review. Journal of Bronchology and Interventional Pulmonology 17 (3), 195-201 Munavvar M, Khan MAI, Edwards J, Waqaruddin Z and Mills J. The autoclavable semirigid thoracoscope: the way forward in pleural disease? Eur Respir J 2007; 29: 571-574 Dhooria S, Singh N, Agarwal AN, Gupta D and Agarwal R. A randomized trial comparing the diagnostic yield of rigid and semirigid thoracoscopy in undiagnosed pleural effusion. Respir Care 2014:59 (5), 756-764 Rozman A, Camlek L, Marc-Malovrh M, Triller N, Kern I. Rigid versus semi-rigid thoracoscopy for diagnosis of pleural disease: a randomized pilot study. Respirology, 2013 May;18(4):704-10. Loddenkemper R, Lee P, Noppen M, Mathur PN. Medical thoracoscopy/pleuroscopy: step by step. Breathe. 2011;8(2):156-67. Rigid Thoracoscopy, Semi-rigid Thoracoscopy, Advanced Techniques

FLARE OF RHEUMATOID ARTHRITIS-ASSOCIATED INTERSTITIAL LUNG DISEASE SECONDARY TO LUNG BIOPSY

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) often presents with dyspnea and a nonproductive cough. The progression and prognosis of the disease are usually determined by the ILD subtype. The most common subtype is usual interstitial pneumonia (UIP) followed by non-specific interstitial pneumonia (NSIP)[1]. For most, the disease remains sub-clinical without progression. Flares can occur, being precipitated by insult to the lungs including infection and even lung biopsy[2]. CASE PRESENTATION: A 44-year-old female with a history of non-erosive rheumatoid arthritis on methotrexate, IV drug abuse, and slowly progressive ILD presented to the hospital due to hypoxemia and worsening cough. Computed tomography (CT) of the chest demonstrated interlobular septal thickening, subpleural peripheral honeycombing and ground-glass opacities with a bibasilar and posterior predominance. Methotrexate was discontinued and the patient was started on broad spectrum antibiotics with initiation of an infectious workup. Due to her unclear etiology of lung disease and failure to improve on antibiotics, a video assisted right upper lung biopsy on day six of hospitalization was performed. The pathology showed fibrosis with honeycombing and dilatation of bronchi consistent with UIP. The patient was then started on pulse dose steroids and rituximab as her hypoxia worsened. Eleven days after biopsy the patient was emergently intubated and ultimately placed on veno-venous extracorporeal membrane oxygenation (VV-ECMO) for refractory hypoxemia. Her course was complicated by streptococcal bacteremia with septic shock, Pseudomonas ventilator associated pneumonia, superior vena cava thrombus, and severe oropharyngeal bleeding. Unfortunately, the patient was not a candidate for lung transplant due to her severity of illness and multiple infections. She was ultimately taken off of life support on day 68 of hospitalization. DISCUSSION: Acute respiratory failure of unclear etiology can be difficult to treat as management can vary drastically. The differential diagnosis in this case includes methotrexate toxicity, infection, and RA-ILD. Ultimately the patient was diagnosed with a flare of her ILD with surgical lung biopsy possibly further exacerbating the flare. Biopsy may have been avoided with her CT findings being consistent with UIP pattern. One could argue that pathology would likely show similar findings and not be contributory to changing the course of treatment. Regardless of the cause, RA-ILD flares can be difficult to manage as treatment with steroids may not be enough and rituximab can take up to 4-6 weeks before seeing clinical improvement, if at all. CONCLUSIONS: RA-ILD can atypically progress at a rapid rate. When considering open lung biopsy, it is judicious to weigh the risks versus the benefits involved. For patients with already progressive ILD, flares must be considered as one of those risks. Reference #1: Solomon JJ, Chung JH, Cosgrove GP, et al. Predictors of mortality in rheumatoid arthritis-associated interstitial lung diseases. Eur Respir J. 2016;47(2):588-96. Reference #2: Bando M, Ohno S, Hosono T, et al. Risk of acute exacerbation after video-assisted thorascopic lung biopsy for interstitial lung disease. J Bronchology Interv Pulmonol 2009;16(4):229-35. DISCLOSURES: No relevant relationships by Jessica Boehmler, source=Web Response No relevant relationships by Nathan Brewster, source=Web Response No relevant relationships by Kareem Godil, source=Web Response No relevant relationships by Breanna Goldner, source=Web Response No relevant relationships by Ethan Stern, source=Web Response

A CASE OF CHRONIC PULMONARY ASPERGILLOSIS IN A HEALTHY IMMUNOCOMPETENT PATIENT

SESSION TITLE: Chest Infections: Find the Fungus SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/21/2019 3:15 PM - 4:15 PM INTRODUCTION: Chronic pulmonary aspergillosis (CPA), and specifically its most common form – chronic cavitary pulmonary aspergillosis (CCPA) - is an uncommon pulmonary disease (1). It may be seen in either patients are who are immunocompromised or patients who are immunocompetent with underlying lung disease. We present a case of CCPA complicated by recurrent hemoptysis necessitating surgical resection in a healthy 19-year-old female without known underlying lung disease. CASE PRESENTATION: A 19 year-old female presented with one month of hemoptysis. She was healthy, without any known medical problems until age 17 when she was found to have a mycetoma and cavitary pneumonia. She underwent an extensive workup, including HIV and Quantiferon Gold testing, at that time which was negative and was treated with antibiotics. She was unable to complete a prolonged course of voriconazole due to financial constraints. Interval imaging demonstrated multiple cavitary lesions with air fluid levels and soft tissue density consistent with aspergilloma. She was asymptomatic between age 17 and 19, when she began to experience hemoptysis. The patient presented to our hospital with persistent hemoptysis. She underwent robotic video-assisted thorascopic surgery (VATS) with right lower lobectomy. The parenchyma was noted to contain numerous cystic cavities. Histological examination demonstrated free-floating aspergillus hyphae within a cyst wall. Although not definitive, the aspergilloma may have formed in an area of interlobar pulmonary sequestration or congenital cystic adenoid malformation. Five days following surgery, the patient had no further hemoptysis and was discharged home. DISCUSSION: CCPA is a common form of CPA, which also encompasses aspergillus nodules, simple aspergilloma, and subacute invasive aspergillosis (SAIA) or chronic necrotizing pulmonary aspergillosis. Left untreated, CCPA may progress to chronic fibrosing pulmonary aspergillosis (CFPA), which is characterized by extensive pulmonary fibrosis. It is exceedingly unusual for CCPA to develop in immunocompetent individuals without underlying pulmonary disease. Our patient is a young and healthy female who had no prior respiratory symptoms who presented at the age of 17 with an already formed aspergilloma. Oral antifungal therapy is considered standard of care for treatment of CCPA (1). Response is slow and requires a minimum of four months. Hemoptysis may necessitate surgical resection in those with adequate pulmonary function. CONCLUSIONS: CCPA may occur in imunnocompetent patients with no prior history of lung disease. The possibility of the presence of a previously unknown and asymptomatic congenital malformation may increase the risk for the development of CPA. Reference #1: Denning DW, Cadranel J, Beigelman-Aubry C, et al. Chronic pulmonary aspergillosis: rationale and clinical guidelines for diagnosis and management. Eur Respir J 2016;47:45-68. DISCLOSURES: No relevant relationships by Craig Fryman, source=Web Response No relevant relationships by Sameer Khanijo, source=Web Response No relevant relationships by Kelvin Wong, source=Web Response

INCREASING DIAGNOSTIC YIELD WITH ARGON PLASMA COAGULATION

SESSION TITLE: Wednesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Endobronchial biopsy can often be complicated by bleeding. In certain cases, the procedure must be aborted due to bleeding complications. We present a case of using argon plasma coagulation to increase biopsy yield and allow for adequate tissue sampling and diagnosis CASE PRESENTATION: An 87-year-old woman presented to us with complaints of 15-pound weight loss along with worsening cough and night sweats. On initial workup her chest x-ray noted a nodular density in the left upper lung zone. She underwent computed tomography of her chest in which a large mass like density was seen in the left upper lobe. Bronchoscopy with bronchoalveolar lavage as well as brush biopsy was scheduled given the size and location of the lesions. On inspection, there was diffuse inflammation of the apicoposterior branch of the left upper lobe, along with severe obstruction. Brushing of the lesions was attempted, but copious amounts of bleeding occurred. Epinephrine was instilled to achieve hemostasis, and the procedure was aborted. Given the complications, the samples obtained were non-diagnostic. She was rescheduled for bronchoscopy, but in addition to brushing, and forceps biopsy we employed argon plasma coagulation (APC) as well as endobronchial ultrasound with transbronchial biopsy. Upon forceps biopsy, bleeding complications were encountered. However, multiple samples were obtained, and argon plasma was used to achieve hemostasis as well as tumor debulking. In addition, areas on inflammation seen on the left lower lobe were sampled with cytology brushing. Samples obtained from EBUS-TBNA, brushing, and biopsy was diagnostic for non-small cell lung cancer, and PDL-1 negative. She underwent palliative radiation therapy. Given her staging of IIIA, she opted to enroll in home hospice. DISCUSSION: Bleeding complications from both endobronchial to transbronchial biopsies have been well documented. A variety of methods to achieve hemostasis have also been listed methods to control post-biopsy bleeding. These include topicalized adrenaline, ice-cold saline, direct tamponade with the bronchoscope, and balloon tamponade. Bleeding complications are likely to occur especially in the setting of inflamed for vascular tissue. In addition, given the amount of tissue needed for both diagnosis and tumor markers, multiple samples are required; the ERS recently suggested as much as six samples. APC has been documented to achieve hemostasis, especially with regards malignancy. Argon plasma coagulation is a form on non-contact electrocoagulation, and allows rapid coagulation of target tissue. The European Respiratory society recently suggested APC may be used as an adjunct for post-biopsy bleeding and hemostasis. However, the data is limited in such cases. CONCLUSIONS: This case highlights the planned of APC to increase diagnostic yield, with the preparation of hemostasis after multiple biopsies. Reference #1: Bernasconi M, Koegelenberg CFN, Koutsokera A, et al. Iatrogenic bleeding during flexible bronchoscopy: risk factors, prophylactic measures and management. ERJ Open Res. 2017;3(2):00084-2016. Published 2017 Jun 21. https://doi.org/10.1183/23120541.00084-2016 Reference #2: Bolliger CT, Sutedja TG, Strausz J, Freitag L. Therapeutic bronchoscopy with immediate effect: laser, electrocautery, argon plasma coagulation and stents. Eur Respir J. 2006;27(6):1258-71. Reference #3: Ofiara LM, Navasakulpong A, Ezer N, Gonzalez AV. The importance of a satisfactory biopsy for the diagnosis of lung cancer in the era of personalized treatment. Curr Oncol. 2012;19(Suppl 1):S16-23. DISCLOSURES: No relevant relationships by Danial Arshed, source=Web Response No relevant relationships by Christopher Lee, source=Web Response no disclosure on file for Lourdes Sanso; no disclosure on file for Anthony Smith

ATTACK OF THE FOAMY MACS: DIFFUSE PANBRONCHIOLITIS IN A CAUCASIAN WOMAN

SESSION TITLE: Pulmonary Pathology II SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/21/2019 3:15 PM - 4:15 PM INTRODUCTION: Diffuse Panbronchiolitis (DPB) is a rare condition most commonly seen in patients of Asian descent (1). We identified a Caucasian patient with alpha-1antitrypsin (α1AT) MS genotype, who was diagnosed with DPB after multi-disciplinary discussion (MDD). CASE PRESENTATION: A 41 y/o woman with a diagnosis of hypersensitivity pneumonitis (HP) was referred for environmental exposure evaluation by an occupational/environmental pulmonologist to find the cause of her HP. She had presented five years previously with cough productive of yellow sputum. She reported a history of seasonal allergies. Her cough did not improve with bronchodilator use. Prednisone did provide some relief at a dose of 40 mg daily but was discontinued due to insomnia and edema. Her chest CT showed tubular bronchiectasis in both lower lobes and peribronchial thickening with peripheral infiltrates. Surgical lung biopsy demonstrated features thought to be consistent with HP. Comprehensive environmental exposure history was unrevealing. Physical exam revealed intermittent coughing, diffuse crackles and expiratory low-pitched wheezing. Due to discordance between her clinical & exposure history, chest imaging and pathology, her case was presented at MDD conference. During this MDD, thoracic radiology impression was that her imaging represented an airways-centered process such as bronchiolitis. Thoracic pathology review found bronchiolocentric lymphocytic infiltrations with germinal centers distributed in the middle of the secondary lobule, interstitial lymphocytes, and foamy macrophages in the interstitium, sparing the alveolar spaces. The massive lymphocytic infiltration in a bronchiolocentric pattern along with interstitial foamy macrophages was consistent with the unique pathologic pattern of DPB. Additional laboratory tests found normal α1AT level (125), but abnormal genotype (MS). CFTR gene sequencing and autoimmune antibodies were normal. CBC showed no leukocytosis or eosinophilia. She was treated with erythromycin and noted improvement in cough. DISCUSSION: DPB can be a rare cause of cough in non-Asian patients. α1AT MS genotype has been associated with airway disease and may be the reason for this patient’s presentation (2). Here, MDD yielded a refinement in clinical diagnosis, which led to alteration in care. Treatment for DPB includes chronic macrolide use, a therapy different from traditional therapies for HP, and would have not been instituted without this diagnostic refinement. CONCLUSIONS: This case highlights the utility of MDD approach in increasing diagnostic confidence of rare pulmonary diseases. Reference #1: Poletti V, et al. Diffuse panbronchiolitis. Eur Respir J 2006; 28:862. Reference #2: Sandford AJ, et al. Z and S mutations of the α1-antitrypsin gene and the risk of chronic obstructive pulmonary disease. Am J Respir Cell Mol Biol 1999; 20: 287–291. DISCLOSURES: No relevant relationships by Cheryl Augenstein, source=Web Response grant recipient relationship with Department of Defense, Department of Veterans Affairs, CDC Please note: >$100000 Added 03/18/2019 by Stella Hines, source=Web Response, value=Grant/Research Support No relevant relationships by Mary Richert, source=Web Response No relevant relationships by Bethany Weiler-Lisowski, source=Web Response No relevant relationships by Sarah Williams, source=Web Response

A PATIENT WITH ACUTE CHRONIC RESPIRATORY FAILURE AND BILATERAL PULMONARY INFILTRATES

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Differential diagnosis of bilateral diffuse pulmonary infiltrate is broad and includes both infectious and noninfectious etiologies, such as vasculitis, allergic bronchopulmonary aspergillosis, interstitial lung disease and autoimmune disease. CASE PRESENTATION: A 45 year old male with a presumptive diagnosis of hypersensitivity pneumonitis presented with worsening shortness of breath and was admitted to the hospital. He received steroids and 5 day course of levofloxacin, but remained hypoxic requiring up to 10 liter oxygen. Due to his worsening respiratory status, he was transferred to the intensive care unit (ICU). On admission to the ICU, chest radiograph showed progressing patchy airspace disease diffusely throughout both lungs. CT chest revealed nonspecific diffuse and patchy ground glass opacity with interlobular septal thickening and superimposed consolidative opacities throughout both lungs, in a crazy paving pattern. He continued treatment with intravenous steroids but had minimal improvement in his respiratory status. He underwent bronchoscopy with bronchoalveolar lavage (BAL) on day 5, which showed scant thick secretions with occasional streak of blood. Based on his imaging results and bronchoscopy appearance, the top differential for his acute on chronic respiratory failure was pulmonary alveolar proteinosis (PAP). He subsequently underwent bilateral total lung lavage during which large amount of debris was cleared from both lungs, and periodic acid-Schiff (PAS) stain showed clumps of granular acellular material suggestive of intra-alveolar proteinosis. Culture from BAL also showed polymicrobial growth with streptococcus anginosus and rothia dentocariosa, and he completed 7 day course of antibiotic therapy, initially with cefepime and then transitioned to ceftriaxone. By the time of discharge, his oxygen requirement was 3L/min via nasal cannula and he was started on GM CSF. DISCUSSION: PAP is a rare disease characterized by accumulation of PAS-positive lipoproteinaceous material in the distal air spaces due to impaired surfactant clearance. Auto-immune PAP is the most common etiology with estimated prevalence of 4 to 40 cases per million. CT scan is the major tool in diagnosis of PAP with patterns of ground-glass opacities, septal reticulations and parenchymal consolidation being highly suggestive of the disease. Diagnosis requires BAL which reveals milky fluid and positive PAS-staining granules. The mainstay of treatment remains symptomatic total lung lavage with GM CSF as an adjunct. CONCLUSIONS: In patients with acute on chronic respiratory failure, the appearance of diffuse ground-glass opacities, septal reticulations and parenchymal consolidation should raise concern for PAP. Proper diagnostic workup includes BAL with PAS staining. Reference #1: Singh I, Kaner R. A Woman with Asthma and Ground-Glass Opacities. Ann Am Thorac Soc. 2017;14(1):140-144. Reference #2: Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med. 1958;258(23):1123-42. Reference #3: Borie R, Danel C, Debray MP, et al. Pulmonary alveolar proteinosis. Eur Respir Rev. 2011;20(120):98-107. DISCLOSURES: No relevant relationships by Alice Gallo De Moraes, source=Web Response No relevant relationships by Melissa Myers, source=Web Response No relevant relationships by Zhenmei Zhang, source=Web Response

AN UNUSUAL CAUSE OF ACUTE EOSINOPHILIC PNEUMONIA

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: We report an unusual case of interstitial lung disease in a patient suffering from alcoholism. CASE PRESENTATION: A 44 year old woman with a history of alcoholism presented with a 5 day history of shortness of breath, fevers and malaise. She was found to be hypoxemic and computed tomography of the chest showed bilateral patchy ground glass opacities with interlobular septal thickening and subpleural sparing. She had no exposures and her only medication was monthly subcutaneous naltrexone to maintain sobriety. She had received 4 doses, the most recent being the day of her onset of symptoms. Laboratory testing was significant for leukocytosis and elevated peripheral eosinophilia. Additional testing was negative for autoimmune or vasculitis markers, and parasitic or filarial presence. Bronchoalveolar lavage (BAL) was performed and revealed eosinophilic predominance of 49%. Pathology from transbronchial biopsies demonstrated eosinophilic pneumonia with intra-alveolar fibrin deposition. Steroids were initiated and her imaging abnormalities and oxygen requirement resolved within 48 hours. All of the above findings were felt to be most consistent with acute eosinophilic pneumonia secondary to naltrexone. The patient was cautioned to not be re-exposed to the same medication again. DISCUSSION: Acute eosinophilic pneumonia (AEP) is a febrile illness with hypoxemic respiratory failure and eosinophilic infiltration of the pulmonary parenchyma (1). Characteristic BAL shows marked eosinophilia in the absence of infection, atopy or asthma. AEP is mostly idiopathic but other causes include hypersensitivity to drugs, inhaled agents, or environmental exposures (2). Naltrexone acts by blocking the mu-opioid receptor and modifying the hypothalamic-pituitary-adrenal axis to suppress alcohol consumption. The subcutaneous form of this medication is thought to aid in adherence as it is administered once monthly and achieves a steady therapeutic level. In a randomized control trial showing the efficacy of long-acting injectable naltrexone, there was one serious adverse event of AEP judged to possibly be related to the study medication (3). In AEP, patients typically present with an acute onset of fever, nonproductive cough and dyspnea of less than 4 weeks duration. Diffuse bilateral pulmonary infiltrates are seen on imaging. Pathologic features include acute and organizing diffuse alveolar damage, as well as interstitial and alveolar eosinophils. The mainstay of treatment is systemic glucocorticoids. Most patients improve rapidly in response to glucocorticoids within 24-48 hours (2). CONCLUSIONS: In our case, the patient’s clinical history, radiographic, laboratory and BAL findings were consistent with AEP. Other causes were ruled out and thus AEP was determined to be secondary to long-acting injectable naltrexone. Providers should recognize that AEP is an extremely rare, but reported adverse effect of this drug. Reference #1: Allen JN, Pacht ER, Gadek JE, et al. Acute eosinophilic pneumonia as a reversible cause of noninfectious respiratory failure. N Engl J Med.1989;321:569–574. Reference #2: Rhee CK, Min KH, Yim NY, Lee JE, Lee NR, Chung MP, Jeon K. Clinical characteristics and corticosteroid treatment of acute eosinophilic pneumonia. Eur Respir J. 2013;41(2):402 Reference #3: Garbutt JC, Kranzler HR, O’Malley SS, et al. Efficacy and Tolerability of Long-Acting Injectable Naltrexone for Alcohol Dependence: A Randomized Controlled Trial. JAMA. 2005;293(13):1617–1625. DISCLOSURES: No relevant relationships by Jeannine Foster, source=Web Response No relevant relationships by Thoris Pan, source=Web Response No relevant relationships by Haala Rokadia, source=Web Response

FREE FLOATING RIGHT HEART THROMBI AND PULMONARY EMBOLI: A CASE SERIES

SESSION TITLE: Monday Fellow Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Free floating right heart thrombi (FFRHT) often portend imminent pulmonary embolism (PE) and warrant immediate intervention given their association with high mortality rates - ranging from 27% to 44% with up to 20% mortality within the first day of admission by some reports. Management remains controversial without specific society guidelines, and multiple surgical and pharmacologic treatments have previously been described. Here we present three cases of patients with FFRHT with markedly different management and outcomes. CASE PRESENTATION: Case 1: A 70-year-old man with recent ankle fracture presented with dyspnea and dizziness and was found to have a femoral vein thrombus. Transthoracic echo (TTE) showed right ventricular dilatation and a FFRHT (Fig. 1). He underwent emergent surgical embolectomy with extraction of multiple extensive clots and temporary placement of a right ventricular assist device (RVAD). The patient was later discharged from the hospital with no functional impairment. Case 2: A 74-year-old male dialysis patient presented with fever and pain at his fistula site and received empiric treatment for sepsis. The following day, he developed respiratory failure which progressed to cardiac arrest and underwent six minutes of CPR with ROSC. TTE showed biventricular failure with minimal cardiac activity and a visible RHT (Fig. 2). He received tissue plasminogen activator (tPA) with some improvement on repeat TTE, but soon succumbed to his illness. Case 3: A 49-year-old woman with Hodgkin’s lymphoma and recent left hip replacement presented with dyspnea and calf pain, and was found to have femoral vein thrombosis as well as large bilateral central submassive PE (Fig. 3). Therapeutic anticoagulation was started and TTE showed a large mobile thrombus in the right atrium, which appeared to traverse the inter-atrial septum with a mobile component in the left atrium. She was given tPA and serial repeat TTE showed resolution of RHT and right heart strain. The patient was discharged from the hospital in good condition on oral anticoagulation. DISCUSSION: RHT can be type A "clot in transit” (originating in the periphery) or type B (originating in the right heart). Type A RHT are strongly associated with PE. The existing literature on their management is controversial - while association with PE and high mortality is clear, few reports compare outcomes from anticoagulation alone to thrombolysis or surgical embolectomy (with or without RVAD placement). Incidence of FFRHT is likely drastically underestimated, and is expected to continue rising as bedside cardiac ultrasound becomes more prevalent in the initial assessment of patients with PE. CONCLUSIONS: Bedside cardiac ultrasonography is vital in assessing patients with PE to improve detection of FFRHT. Management of these patients with either thrombolysis or surgical embolectomy should be considered in addition to anticoagulation. Reference #1: Barrios D, Chavant J, Jimenez D, Bertoletti L, Rosa-Salazar V, Muriel A, et al. Treatment of right heart thrombi associated with acute pulmonary embolism. Am J Med. 2017;130:588-595 Reference #2: Koc M, Kostrubiec M, Elikowski W, Meneveau N, Lankeit M, Grifoni S, et al. Outcome of patients in right heart thrombi: the right heart thrombi european registry. Eur Respir J. 2016; 47:869-875 Reference #3: Rose PS, Punjabi NM, Pearse DB. Treatment of right heart thrombo-emboli. Chest. 2002;121:806-814 DISCLOSURES: Speaker/Speaker's Bureau relationship with pfizer Please note: $5001 - $20000 Added 03/20/2019 by ron goldenberg, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Janssen Please note: $5001 - $20000 Added 03/20/2019 by ron goldenberg, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with BMS Please note: $5001 - $20000 Added 03/20/2019 by ron goldenberg, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with melinta Please note: $1001 - $5000 Added 03/20/2019 by ron goldenberg, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with allergan Please note: $5001 - $20000 Added 03/20/2019 by ron goldenberg, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with merck Please note: $5001 - $20000 Added 03/20/2019 by ron goldenberg, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with achaogen Please note: $5001 - $20000 Added 03/20/2019 by ron goldenberg, source=Web Response, value=Honoraria No relevant relationships by Ingrid Gunther, source=Web Response No relevant relationships by Ali Hafiz, source=Web Response No relevant relationships by Susan Mirabal, source=Web Response My spouse/partner as a Advisory Committee Member relationship with CORECEPT Please note: $1001 - $5000 Added 03/15/2019 by Vikramjit Mukherjee, source=Web Response, value=Consulting fee No relevant relationships by Odai Sinokrot, source=Web Response No relevant relationships by James Tsay, source=Web Response No relevant relationships by Winnie Yan, source=Web Response

"Balloon pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension: is Europe behind?" Irene M. Lang and Hiromi Matsubara. Eur Respir J 2019; 53: 1900843.

"Balloon pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension: is Europe behind?" Irene M. Lang and Hiromi Matsubara. Eur Respir J 2019; 53: 1900843.

EXTRAORDINARY EXTRA NODAL LYMPHOMA

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: There are over 20 sub types of non-Hodgkin Lymphoma (NHL), marginal zone lymphoma being the 3rd most common. Mucosa-associated lymphoid tissue lymphoma is an extra nodal marginal zone lymphoma (EMZL). We present a case of indolent nodal marginal zone lymphoma progressing to pulmonary EMZL with diffuse tracheobronchial involvement. CASE PRESENTATION: An 86-year-old female was seen in her oncologist’s office for follow up of indolent abdominal nodal marginal zone B cell lymphoma which had been stable for the last 5 years. She reported dry cough, exertional dyspnea and intermittent wheezing for the last 2 weeks. Medical history was unremarkable otherwise. Vital signs and physical exam were within normal limits. Baseline labs including CBC, CMP and chest x-ray were unremarkable. The patient was evaluated with contrast enhanced CT scan of the chest, abdomen and pelvis which revealed endobronchial lesion in the right middle and lower lobes with post obstructive atelectasis of the right middle lobe, and mediastinal, bilateral hilar and axillary lymphadenopathy. These findings were new compared to the previous CT scans. An endoscopic bronchial ultrasound was done which revealed diffuse mucosal involvement of the tracheobronchial tree in the form of nodular mucosa, and narrowing of the bronchus intermedius. Biopsy of the abnormal mucosa and thoracic lymphadenopathy was done and was consistent with marginal zone B cell lymphoma. Our patient was recommended induction with RCVP (rituximab, cyclophosphamide, vincristine, and prednisone) for 1-2 cycles for followed by maintenance with rituximab, revlimid or both. DISCUSSION: Marginal zone lymphoma (MZL) is a group of indolent NHL B-cell lymphomas, which accounts for approximately 8% of all NHL cases. The average age at diagnosis is 60 years, and it is slightly more common in women than in men. The most common form is EMZL. Common sites for EMZL are stomach, small intestine, salivary gland, thyroid and lungs. When present, EMZL of the lung will present as single or multiple nodules or consolidations on imaging. Treatment depends on the extent of the disease. Options are radiation, surgical resection, immunotherapy (anti-CD 20 monoclonal antibody) alone or in combination with chemotherapy. CONCLUSIONS: EMZL shares clinical features with chronic infections and metastatic cancer. It should be high in the differential in patients with consolidation and thoracic lymphadenopathy. Diffuse involvement of the airways including the upper airways is extremely rare which was present in our case. Also, our patient did not have any underlying autoimmune or infectious etiology that is found in most of the cases of EMZL. Long term success with any therapy is limited requiring close observation. Reference #1: Borie R, Wislez M, Antoine M, Bergman CC, Thieblemont C, Cadranel J. Pulmonary mucosa-associated lymphoid tissue lymphoma revisited. Eur Respir J 47: 1244-1260, 2016. Reference #2: Kawaguchi T, Himeji D, Kawano N, Shimao Y, Marutsuka K. Internal Medicine. 2018 Mar 9; 57(15): 2233-2236. Reference #3: Zinzani PL. The many faces of marginal zone lymphoma. Hematology Am Soc Hematol Educ Program1: 426-432, 2012. DISCLOSURES: No relevant relationships by Shujaa Faryad, source=Web Response No relevant relationships by Rajamurugan Meenakshisundaram, source=Web Response

ANTI-SYNTHETASE SYNDROME ASSOCIATED WITH PULMONARY VENO-OCCLUSIVE DISEASE

SESSION TITLE: Pulmonary Vascular Disease SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/22/2019 3:45 PM - 4:45 PM INTRODUCTION: Anti-synthetase syndrome (ASS) is a heterogeneous connective tissue disease, characterized by an association with interstitial lung disease (ILD) and/or inflammatory myositis and the presence of anti-aminoacyl-tRNA-synthetase antibodies. Pulmonary hypertension (PH), a progressive pulmonary vascular disease, defined by elevation of pulmonary arterial pressure, has been reported with a prevalence of 7.9% in patients with ASS. However, ASS associated with PH due to Pulmonary veno-occlusive disease (PVOD) has never before been described in literature. We present a novel case of anti-synthetase syndrome associated with PVOD. CASE PRESENTATION: 55 year old man with history of ILD presented with hypoxic respiratory failure and right heart failure. He was positive for ANA (1:160), and Anti-Jo (226 AU/mL) antibodies and hence was diagnosed with Anti-synthetase syndrome. Heart catheterization was done which showed, preserved LV function, and PA 100/45 mmHg with mean of 65, PAOP of 8 mmHg, PVR of 21 WU, and CO 2.7 L/min. Findings of elevated pressures out of proportion to his ILD, led to a diagnosis of pre-capillary PH and he was started on therapy with Milrinone, Macitentan and Sildenafil which he tolerated. Later in the hospitalization, IV Epoprostenol was added which resulted in acutely worsening hemodynamics and CT Chest remarkable for worsening pulmonary edema, pleural effusions and lymphadenopathy concerning for PVOD. He was deemed not a candidate for lung transplant. IV Epoprostanol was de-escalated, however at that time patient opted for comfort measures and expired. On autopsy, bilateral lungs revealed definitive evidence of PVOD, including vascular myxoid fibrosis, intimal hyperplasia, and venous occlusion in a background of pulmonary parenchyma with mature fibrosis. Changes suggestive of pulmonary arterial hypertension including arterial intimal thickening were also observed. DISCUSSION: PVOD is a rare form of group 1 PH, that has never before been described in association with anti-synthetase syndrome. The pathologic hallmark of PVOD is the extensive and diffuse occlusion of pulmonary veins by fibrous tissue. A definite diagnosis of PVOD requires histologic analysis of a lung sample. Patients with PVOD may either be refractory to pulmonary arterial hypertension (PAH)-specific therapy or deteriorate with the start of therapy. It is difficult to clinically distinguish patients with PVOD from PAH prior to start of therapy. CT of the chest can suggest PVOD in the setting of PH when it shows nodular ground-glass opacities, thickened septal lines, and lymph node enlargement, however in patients who present with decompensated heart function such details are difficult to distinguish on imaging. CONCLUSIONS: Anti-synthetase syndrome can be associated with PVOD, and it should be suspected in patients that have evidence of pre-capillary disease, and who deteriorate with initiation of PH therapies. Reference #1: Hervier B, Meyer A, Dieval C, et al. Pulmonary hypertension in antisynthetase syndrome: prevalence, aetiology and survival. Eur Respir J. 2013;42(5):1271-1282. Reference #2: Montani D, Achouh L, Dorfmuller P, et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore). 2008;87(4):220-233. Reference #3: Holcomb BW Jr, Loyd JE, Ely EW, Johnson J, Robbins IM. Pulmonary veno-occlusive disease: a case series and new observations. Chest. 2000;118:1671–1679. DISCLOSURES: No relevant relationships by Ali Ataya, source=Web Response No relevant relationships by Lauryn Benninger, source=Web Response No relevant relationships by Garrett Fitzpatrick, source=Web Response No relevant relationships by Saminder Kalra, source=Web Response No relevant relationships by Dana Kay, source=Web Response No relevant relationships by Ramon Valentin, source=Web Response

MEPOLIZUMAB AS A NOVEL TREATMENT FOR ASTHMA WITH ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS

SESSION TITLE: Monday Fellow Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: A 69-year-old black female with difficult to control asthma was found to have allergic bronchopulmonary aspergillosis (ABPA). While omalizumab can be used to treat ABPA, we elected to use mepolizumab due to the degree of peripheral eosinophilia relative to IgE. This demonstrates a novel use for mepolizumab. CASE PRESENTATION: A patient with no known history of lung disease presented in November 2015 with dyspnea. An Aspergillus precipitins panel was negative at that time. We followed her over the next four years noting the development of a peripheral eosinophilia (see table). During this time, she experienced two episodes of lobar collapse from mucus plugging which were both associated with viral pneumonia. After an extensive evaluation for alternative causes of poorly controlled asthma with significant peripheral eosinophilia, a repeat aspergillosis precipitins panel was obtained in October 2018 which was now positive. In October, 2018, the patient was started on prednisone and voriconazole without clinical or spirometry improvement. In January, 2019, she began treatment with mepolizumab. At follow-up, she demonstrated subjective improvement, a normal peripheral eosinophilia, and improved imaging with her most recent chest x-ray (on the left) compared to one at diagnosis with ABPA (on the right). A comparison of FEF 25-75% on PFTs from October, 2019 and February, 2019 demonstrated an increase of 0.33 liters, an improvement of 20% of predicted value. DISCUSSION: ABPA is a non-invasive form of pulmonary aspergillosis that can develop in patients with chronic lung diseases such as asthma. It is characterized by activation of lymphocytes along a T-helper 2 pathway resulting in recruitment of eosinophils via cytokines including IL-4, IL-5, and IL-13. ABPA can lead to epithelial damage, bronchiectasis, and ultimately fibrosis if left untreated. Omalizumab is a monoclonal antibody directed against IgE and has been used as add on treatment for ABPA. Mepolizumab is a monoclonal antibody directed against IL-5. Given this patient’s relatively lower IgE levels as compared to her profound peripheral eosinophilia, mepolizumab was chosen over omalizumab in order to target IL-5. One case report is present in the literature describing this as a successfully treatment option. To date, there are no clear head-to-head studies comparing these two treatment modalities. CONCLUSIONS: This case demonstrates an alternative biological treatment for uncontrolled asthma due to allergic bronchopulmonary aspergillosis using mepolizumab in the setting of significant peripheral eosinophilia with a significant clinical/radiological/spirometer improvement. Reference #1: Patterson KC, and Strek ME. Diagnosis and Treatment of Pulmonary Aspergillosis Syndromes. Chest. 146;5: 1358 – 1368. Reference #2: Kousha M, Tadi R, and Soubani AO. Pulmonary Aspergillosis: A Clinical Review. Eur Respir Rev. 20;121: 156 – 174. Reference #3: Oda N, Miyahara N, et al. Severe Asthma Concomitant with Allergic Bronchopulmonary Aspergillosis Successfully Treated with Mepolizumab. Allergology International. 67 (2018) 521 – 523. DISCLOSURES: No relevant relationships by Tyson Sjulin, source=Web Response No relevant relationships by William Wadzinski, source=Web Response

PULMONARY-RENAL SYNDROME: URINE TROUBLE WHEN YOU DEVELOP ALVEOLAR HEMORRHAGE

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Acute ANCA vasculitis with in-hospital development of alveolar hemorrhage results in significant mortality of up to 24%. Generally, diffuse alveolar hemorrhage has worsening prognostic outcomes in patients age > 60 years, particularly when associated with GFR < 60, tobacco abuse, cardiovascular disease, shock, and mechanical ventilation. Furthermore, ANCA-associated vasculitis has a 90% 2-year mortality if left untreated, which significantly decreases to 74% 5-year mortality in patients receiving prompt corticosteroid therapy. It is important to promptly recognize and treat late onset ANCA vasculitis, especially in patients requiring critical care admission. CASE PRESENTATION: We present an 80-year-old woman with medical history of hypothyroidism, diabetes mellitus type II, and hypertension. She was initially admitted for treatment of Clostridium difficile colitis. Despite clinical improvement of diarrheal illness after treatment with oral vancomycin, she was noted to have worsening acute kidney injury on hospital day 2. On hospital day 4, she had an acute drop in hemoglobin. On day 7, the patient's respiratory status began to decompensate with increasing hypoxia and work of breathing. She was transitioned to an intermediate level of care for high flow nasal cannula, and ultimately was placed on non-invasive positive pressure ventilation for refractory hypoxemia. Computed tomography (CT) imaging was notable for bilateral infiltrates and the patient was treated with broad spectrum antibiotics with concern for pneumonia, though she remained afebrile. She never experienced hemoptysis. On hospital day 10, the patient further declined requiring urgent intubation. Upon intubation, significant bloody secretions were noted. This was further evaluated with bronchoscopy with serial BAL aliquots, which proved to be progressively bloody, consistent with diffuse alveolar hemorrhage. Treatment with pulse dose steroids and plasma exchange was initiated. Serologic workup was consistent with granulomatosis with polyangiitis (GPA) as demonstrated by positive c-ANCA titer 1:640. The patient was started on rituximab, successfully extubated and discharged from intensive care in stable condition on hospital day 22. DISCUSSION: This case is especially interesting considering the new diagnosis of GPA in the inpatient setting following an admission for C. difficile colitis. She had no hemoptysis, no bloody diarrhea, prior diagnosis of CKD, nor other manifestations concerning for GPA. Her presentation was acute and atypical, and her mortality was ameliorated due to urgent intubation and immediate treatment. CONCLUSIONS: By initiating prompt steroid therapy and plasma exchange, this 80-year-old woman with significantly impaired GFR, shock requiring multiple vasopressors, and invasive ventilatory support, defied the expected outcome and significant mortality associated with late-onset granulomatosis with polyangiitis. Reference #1: de Prost N, Parrot A, Picard C, Ancel PY, Mayaud C, Fartoukh M, et al. Diffuse alveolar haemorrhage: Factors associated with in-hospital and long-term mortality. Eur Respir J. 2010;35:1303–11 Reference #2: Biscetti F, Carbonella A, Parisi F, et al. The prognostic significance of the Birmingham Vasculitis Activity Score (BVAS) with systemic vasculitis patients transferred to the intensive care unit (ICU). Medicine (Baltimore). 2016;95(48):e5506 DISCLOSURES: No relevant relationships by Meagan Marshburn, source=Web Response No relevant relationships by Sarah Ellen Stephens, source=Web Response No relevant relationships by Amanda Westbrook, source=Web Response