FULMINANT ORGANIZING PNEUMONIA SECONDARY TO MDA5-ASSOCIATED AMYOPATHIC DERMATOMYOSITIS: A CASE REPORT AND REVIEW OF THE LITERATURE

Abstract

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: Fulminant Organizing Pneumonia (OP) is a rare, clinicopathologic diagnosis considered to have good prognosis with early recognition and steroid therapy. There is a paucity of literature on rapidly progressive presentations [1,3,5,6]. We present the case of a 44-year-old woman who presented with fevers and arthralgias found to have Anti-MDA5 amyopathic dermatomyositis who developed fulminant organizing pneumonia and hypoxemic respiratory failure requiring extracorporeal membrane oxygenation (ECMO). CASE PRESENTATION: The patient was a 44-year-old female without significant past medical history who presented with one month of worsening rash, recurring high fevers, hoarseness, dysphagia, and arthralgias. The rash started on the dorsum of the hands, the distal interphalangeal joints (DIP) and proximal interphalangeal joints (PIP). Within five days, the patient had escalating oxygen requirements requiring mechanical ventilation. Fiberoptic bronchoscopy revealed bilateral vocal cord paralysis and hyperemic airways. Cultures from lavage revealed pan-sensitive klebsiella and patient was started on the appropriate antimicrobial therapy. Computed Tomography Angiography (CTA) at this time was negative for pulmonary embolism (PE) but demonstrated bibasilar airspace disease with small areas of cavitation. A high risk video assisted thoracoscopic lung biopsy was performed and pulse steroids were started pending the results. Lung biopsy demonstrated hyaline membranes and organizing pneumonia. Despite treatment, she did not improve, developed worsening hypoxemia, which required transfer for ECMO. Patient was found to have Anti-MDA5 Antibodies in the serum and was diagnosed with amyopathic dermatomyositis. Despite maximal medical therapy, the patient passed away. DISCUSSION: This is the fourth case report to describe a fulminant onset OP in a patient with MDA5-associated amyopathic dermatomyositis [7,10]. Rapidly progressive OP leading to acute respiratory failure and ARDS has been previously described [5]. This variant of dermatomyositis is not even assayed on certain laboratory “dermatomyositis panels” which can hinder diagnostic clarity. There is controversy with regards to the appropriate timing and choice treatment of MDA5 associated dermatomyositis, but recent literature suggests early administration of Rituximab [12]. This introduces a clinical quandary. These patients require urgent diagnoses and prompt treatment to prevent progression of lung injury, but are often too unstable for lung biopsy or require tests which take several days to return. Rapidly progressive OP secondary to this rare variant of amyopathic dermatomyositis is exceedingly rare, with only a few reported cases [7,10]. CONCLUSIONS: In the setting of acute respiratory failure and a diagnosis of organizing pneumonia the use of early immunologic agents should be considered, even without a definitive immunologic/rheumatologic diagnosis. Reference #1: Kobayashi T, Kitaichi M, Tachibana K, et al. A Cryptogenic Case of Fulminant Fibrosing Organizing Pneumonia. Intern Med. 2017;56(10):1185-1191. Reference #2: Wells, A. U. (2001). Cryptogenic Organizing Pneumonia. Seminars in Respiratory and Critical Care Medicine, 22(04), 449–460. Reference #3: Cohen AJ, King TE, Downey GP. Rapidly progressive bronchiolitis obliterans with organizing pneumonia. Am J Respir Crit Care Med. 1994;149(6):1670-5. DISCLOSURES: No relevant relationships by Bhavna Abbi, source=Web Response Advisory Committee Member relationship with Ambu Please note: $1-$1000 Added 03/29/2019 by Olumayowa Abe, source=Web Response, value=Honoraria No relevant relationships by Christian Castaneda, source=Web Response No relevant relationships by Jerry Centeno, source=Web Response No relevant relationships by Erik Perez, source=Web Response No relevant relationships by John Prudenti, source=Web Response