ATYPICAL CASE OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a multi-organ vasculitis. However, the hallmarks of disease are eosinophilia, chronic sinusitis, and asthma. Vasculitic organ involvement might be absent early on, making diagnosis challenging1. We present case patient atypical presentation serology for EGPA. CASE PRESENTATION: A 71-year-old male history non-asthmatic eosinophilic bronchitis, peripheral eosinophilia (peak 936 cells/uL; 17.6%) was admitted to hospital dyspnea rapidly progressive acute hypoxemic respiratory failure requiring transfer intensive care unit. He started on high flow nasal cannula (60LPM; FiO2 90%-100%). Bilateral crackles noted no pitting edema. Chest CT showed bilateral airspace ground glass opacities. tested negative twice SARS-CoV-2. White blood cell count, kidney function, brain natriuretic peptide were normal. Autoimmune workup ordered. empiric broad-spectrum antibiotic, gently diuresed. As his suggestive lung disease, in light significant oxygen requirement, decision made hold bronchoscopy start solumedrol at 125mg/day. On ICU Day 3, autoimmune elevated anti-nuclear antibody titer 1:160 speckled pattern, anti-myeloperoxidase (MPO) 49.4 (p-ANCA), anti-proteinase-3 (anti-PR3) titers 18.8 (c-ANCA). Infectious negative. The diagnosed possible cyclophosphamide addition steroids. Oxygen requirement continued improve. discharged home 4LPM oral steroid plan monthly infusion. Follow-up 2 months repeat chest marked improvement. DISCUSSION: manifestations EGPA dynamic, few presenting earlier than others2. With temporal spacing signs symptoms, it often late when more clues evident. In 1990, American College Rheumatology (ACR) established set 6 criteria: asthma, >10%, mononeuropathy or polyneuropathy, migratory pulmonary infiltrates, paranasal sinus abnormality, biopsy containing vessel extravascular eosinophils2. At least 4 out six these criteria must met have specificity 99.7%. p-ANCA (anti-MPO) around 70% patients active EGPA, whereas c-ANCA nearly absent3. Our 3 criteria, atypical, showing both c-ANCA. CONCLUSIONS: our not robust one. establishing presumed starting immunosuppressive therapy likely saved life. REFERENCE #1: Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome. Clinical study long-term follow-up 96 patients. Medicine (Baltimore) 1999; 78:26. #2: Masi AT, Hunder GG, Lie JT, 1990 classification syndrome (allergic granulomatosis angiitis). Arthritis Rheum 1990; 33:1094. #3: Sablé-Fourtassou R, Mahr A, Antineutrophil cytoplasmic antibodies Ann Intern Med 2005; 143:632 DISCLOSURES: No relevant relationships by Tony Abdo, source=Web Response Ahel El Haj Chehade, Ahmad Hassan,