Abstract: PUB178
From Sicca to Severe: Pulmonary-Renal Syndrome in Sjogren Syndrome
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Shah, Bhoomi, Kaiser Permanente Southern California Department of Research & Evaluation, Los Angeles, California, United States
- Chao, Allen, Kaiser Permanente Southern California Department of Research & Evaluation, Los Angeles, California, United States
Introduction
Pulmonary-renal syndrome is a life-threatening clinical entity characterized by diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. It is most commonly associated with ANCA-associated vasculitis and anti-glomerular basement membrane disease. Primary Sjögren’s syndrome is a systemic autoimmune disorder primarily affecting exocrine glands; however, severe pulmonary and renal involvement is rare. We present a rare case of pulmonary-renal syndrome associated with primary Sjögren’s syndrome, highlighting the diagnostic challenges and importance of early recognition of systemic manifestations.
Case Description
A 69-year-old female with a history of xerostomia/xerophthalmia presented with progressive dyspnea, fatigue, and acute kidney injury. Initial laboratory evaluation demonstrated elevated serum creatinine of 1.9 mg/dL (baseline 1.2), nephritic urinary sediment with subnephrotic range proteinuria and hematuria. Serologic evaluation was notable for positive ANA, anti-SSA/Ro antibodies, and low C3 levels; ANCA and anti-GBM antibodies were negative. Kidney biopsy demonstrated intestitial nephritis with IgA nephropathy. Patient developed MRSA baceremia during the hospitalization and hence immunosuppression had to be delayed and unfortunately dialysis had to be initiated, on resolution of bacteremia patienet was treated with solumedrol and course complicated by diffuse alveolar hemorrhage confirmed by bronchoscopy and was treated with PLEX followed by rituximab.
Discussion
Pulmonary–renal syndrome is a rare but severe extraglandular manifestation of Sjögren’s syndrome and may mimic ANCA-associated vasculitis or anti-GBM disease. Early recognition is critical, as delayed diagnosis can lead to respiratory failure and irreversible kidney injury. Autoimmune serologies and tissue diagnosis remain essential for distinguishing Sjögren’s-related disease from other vasculitic processes. It is also notable that sjogren's can present with IgA deposits on biopsy. Immunosuppressive therapy is the mainstay of the treatment and PLEX can be used for cases with DAH.