WITHDRAWN

Abstract

SESSION TITLE: Systemic Diseases Causing Pulmonary Havoc SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is an immune-mediated disease characterized by necrotizing vasculitis of the small blood vessels. Pulmonary involvement is common in AAV. Nodules and pleural effusions are the most common pulmonary finding in AAV. Fibrosing interstitial lung disease (ILD) is rarely associated with AAV, and it has a significant prognostication impact. We present a case of usual interstitial pneumonia (UIP) found in a patient with anti-Myloperoxidase (MPO) ANCA and rapidly progressive disease. CASE PRESENTATION: A 71-year-old female presented to the hospital with progressive exertional dyspnea and weakness for several months. Her physical exam was remarkable for bilateral basilar crackles. Oxygen saturation was 91% on ambient air. Chest radiograph showed lower zones interstitial infiltrates. Computed tomography of the chest showed diffuse reticular opacities in both lungs with honeycombing and traction bronchiectasis consistent with definite usual interstitial pneumonia (UIP) pattern. Laboratory evaluation revealed elevated serum creatinine (5.73 mg/dL). Immunology tests showed elevated anti-MPO ANCA titers. A renal biopsy showed the presence of pauci-immune crescentic glomerulonephritis, confirming the diagnosis of ANCA-mediated vasculitis. The patient was treated with plasmapheresis and 500 mg of intravenous methylprednisolone for three days, followed by cyclophosphamide and a medium dose of prednisone. Her kidney function improved, and she was then discharged to follow-up with pulmonary clinic for initiation of anti-fibrotic. DISCUSSION: ANCA-associated vasculitis (AAV) is a small-vessel vasculitis that can involve multiple organs. Pulmonary involvement has been reported in 25%–80% of cases. Sometimes, it can be the initial manifestation of the disease. The most common form of pulmonary involvement in AAV is diffuse pulmonary nodules, with or without cavitation and pleural effusions. Occasionally, patients can present with fibrosing-ILD, particularly with anti-MPO. This presentation is more prevalent in males and the elderly. Among AAV patients with ILD, UIP is the most frequent radiographic pattern. Non-specific interstitial pneumonia (NSIP) pattern can also be seen. ILD has been linked to increased mortality in AAV patients, particularly those with a UIP pattern. Treatment of fibrosing-ILD in AAV patients is not well established, with a questionable role for immunosuppression. Anti-fibrotic agents might be helpful in patients with a UIP pattern. CONCLUSIONS: UIP is rarely associated with AAV, and it can be the leading diagnostic manifestation. A careful clinical history and laboratory evaluation should be performed in patients with UIP to identify and treat any underlying systemic disease. UIP is a poor prognostic sign in AAV. Clinical trials are needed to establish the role of anti-fibrotic in treating this condition. Reference #1: Maillet T, Goletto T, Beltramo G, Dupuy H, Jouneau S, Borie R, Crestani B, Cottin V, Blockmans D, Lazaro E, Naccache JM, Pugnet G, Nunes H, de Menthon M, Devilliers H, Bonniaud P, Puéchal X, Mouthon L, Bonnotte B, Guillevin L, Terrier B, Samson M; French Vasculitis Study Group (FVSG). Usual interstitial pneumonia in ANCA-associated vasculitis: A poor prognostic factor. J Autoimmun. 2020 Jan;106:102338. doi: 10.1016/j.jaut.2019.102338. Epub 2019 Sep 27. PMID: 31570253. Reference #2: Mohammad AJ, Mortensen KH, Babar J, Smith R, Jones RB, Nakagomi D, Sivasothy P, Jayne DRW. Pulmonary Involvement in Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis: The Influence of ANCA Subtype. J Rheumatol. 2017 Oct;44(10):1458-1467. doi: 10.3899/jrheum.161224. Epub 2017 Aug 1. PMID: 28765242. Reference #3: Zhou P, Ma J, Wang G. Impact of interstitial lung disease on mortality in ANCA-associated vasculitis: A systematic literature review and meta-analysis. Chron Respir Dis. 2021 Jan-Dec;18:1479973121994562. doi: 10.1177/1479973121994562. PMID: 33605150; PMCID: PMC7897823. DISCLOSURES: No relevant relationships by Mais Abdou No relevant relationships by Khaled Alshabani