Abstract: FR-PO615
Eosinophilic Granulomatosis With Polyangiitis (EGPA) Presents With Nephrotic Syndrome (NS) in the Course of the Disease
Session Information
- Glomerular Diseases: Lupus and Vasculitis
November 04, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1302 Glomerular Diseases: Immunology and Inflammation
Authors
- Guevara, Nehemias, St Barnabas Hospital, Bronx, New York, United States
- Flores Chang, Marjorie Mailing, St Barnabas Hospital, Bronx, New York, United States
- Ali, Mahmoud, St Barnabas Hospital, Bronx, New York, United States
- Ibrahim, Jamil, St Barnabas Hospital, Bronx, New York, United States
- Flores Chang, Bessy Suyin, St Barnabas Hospital, Bronx, New York, United States
Introduction
EGPA affects small and medium sizes vessels. Asthma, nasal, sinus symptoms, and peripheral neuropathy are hallmarks. Necrotizing pauci immune glomerulonephritis is the most common renal presentation seen in kidney biopsy, rarely associated with nephrotic range proteinuria (NRP). Here we are presenting a case of EGPA associated with NRP.
Case Description
A 54 year old male with a PMH HTN, EGPA, Jak 2+ myeloproliferative disorder, presented with 2 months of progressive dyspnea, nasal congestion, productive cough grey sputum with streaks of blood, palpitations, and fatigue. VS: BP: 177/138 mmHg, HR: 129 beats/min, RR:18 breaths/min, T: 98.4 F, SatO2: 97% on room air. PE: ill appearance, eyes anicteric, erythematous and edematous nasal turbinates, erythematous oropharynx with a non-bleeding ulcer on the right side, lungs: decreased breath sounds on bases, lower extremities with no edema, skin intact with no rashes. Works up remarkable for thrombocytosis of 765k, eosinophilia of 179k, elevated creatinine, and D-dimer (Table 1). CXR: unremarkable, EKG: sinus tachycardia, biatrial enlargement, left ventricular hypertrophy, concerning pulmonary embolism (PE), V/Q scan: matched defect in perfusion/ventilation at the left lower lobe superior segment; intermediate probability for PE, the patient was treated with therapeutic enoxaparin. CT Chest (Image 1) scattered bilateral centrilobular ill-defined ground glass nodules throughout the lungs. Course was complicated with worsening renal failure, a 50% drop in baseline renal function with NRP (3.5 gr first time presentation), concern for pulmonary/renal syndrome as an acute flare of EGPA. Renal biopsy showed: focal crescentic and diffuse sclerosing glomerulonephritis, pauci immune type (ANCA-associated), with no activity and mild chronicity. Immunosuppressive therapy with rituximab, mycophenolate mofetil, and steroids were initiated, with the slow recovery of renal function and decrease in proteinuria.
Discussion
EGPA and renal involvement are uncommon at 25%; it's associated with autoimmune activity, however, once renal is involved tubulointerstitial nephritis is the classical presentation, NS is rare in EGPA, final outcome and need for renal replacement therapy still unknown, our patient is still receiving immunosuppression with the slow recovery of renal function.
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