NEW ONSET GRANULOMATOSIS WITH POLYANGIITIS PRESENTING AS PULMONARY-RENAL SYNDROME FOLLOWING COVID-19 INFECTION

SESSION TITLE: Signs and Symptoms of Chest Diseases Case Report Posters 3 SESSION TYPE: Case Report Posters PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: Pulmonary renal syndrome(PRS) is a life-threatening condition characterized by the presence of both diffuse alveolar hemorrhage (DAH) and rapidly progressive glomerulonephritis (RPGN) and is often associated with ANCA-associated vasculitis. Prompt recognition is difficult but essential for this rare disease as untreated PRS has a mortality rate approaching 25-50%. Here we show a case of new onset granulomatosis with polyangiitis (GPA) presenting as PRS following COVID-19 infection. CASE PRESENTATION: A 41 year-old female with no significant past medical history presented with acute hypoxic respiratory failure after several days of shortness of breath and cough with intermittent hemoptysis. She had been diagnosed with COVID-19 at home 3 weeks ago. Initial labs were notable for a creatinine of 3.58mg/dL, hemoglobin of 7.1g/dL as well as a D-Dimer of 6368ng/mL. COVID-19 PCR was negative. Chest X-ray (CXR) on admission showed multifocal bilateral airspace opacities suspicious for atypical pneumonia. Patient was initially admitted for the management of Covid pneumonia but developed large volume hemoptysis and had a computed tomography pulmonary embolism (CT PE) study done, which showed bilateral diffuse groundglass opacities with relative sparing of the periphery concerning for DAH. Patient received multiple doses of nebulized tranexamic acid and was transferred to the intensive care unit where she was intubated. She subsequently had a bronchoscopy with bronchoalveolar lavage done which confirmed the diagnosis of DAH. Autoimmune workup was notable for c-ANCA positive at 1:1280 and proteinase 3 antibody > 8.0. Renal biopsy confirmed diagnosis of RPGN secondary to small vessel vasculitis, consistent with granulomatosis with polyangiitis. Initial therapy consisted of 7 rounds of plasmapheresis, high dose steroids, and cyclophosphamide. Her hemoptysis improved with immunosuppression, and after extubation, she was able to be discharged home without oxygen. DISCUSSION: PRS exhibits considerable heterogeneity in clinical presentation both in severity and prognosis. Distinguishing PRS from pulmonary infection is essential and can be challenging due to similarities in presentation. In this case it was initially felt that her intermittent hemoptysis was in the setting of COVID-19 pneumonia even though her admission PCR was negative. Although her D-Dimer was elevated CT PE was initially deferred as her elevated Dimer could be explained by the presence of a pneumonia. It was only after she developed large volume hemoptysis that a CT was pursued to rule out a pulmonary embolism. Development of GPA after COVID-19 is extremely rare and the pathophysiology is unknown, however there is precedence for infections triggering autoimmune syndromes such as with reactive arthritis. One theory is that SARS-CoV-2 antigens may trigger antibodies and antigen-antibody complexes that target the endothelium, leading to vasculitis. CONCLUSIONS: Early recognition of PRS depends on a high index of clinical suspicion due to many similarities with the presentation of pneumonia and other lung infections. REFERENCE #1: McCabe C, Jones Q, Nikolopoulou A, Wathen C, Luqmani R. Pulmonary-renal syndromes: An update for respiratory physicians. Vol. 105, Respiratory Medicine. 2011. p. 1413–21. REFERENCE #2: Selvaraj V, Moustafa A, Dapaah-Afriyie K, Birkenbach MP. COVID-19-induced granulomatosis with polyangiitis. BMJ Case Rep. 2021 Mar 18;14(3). REFERENCE #3: Karampoor S, Afrashteh F, Rahmani S, Laali A. Eosinophilic granulomatosis with polyangiitis after COVID-19: A case report. Respir Med Case Rep. 2022 Jan 1;38. DISCLOSURES: No relevant relationships by Mark Birkenbach No relevant relationships by Gerardo Carino No relevant relationships by Ayan Purkayastha No relevant relationships by Griffin Reed