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Abstract: TH-PO663

A Rare Case of "Lupus-Like" Glomerulonephritis Presenting as Pulmonary Renal Syndrome (PRS)

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • He, Mingyue, Temple University Hospital, Philadelphia, Pennsylvania, United States
  • Koul, Sheetal, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, United States
  • Lee, Iris J., Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, United States
Introduction

PRS is a life-threatening condition characterized by rapidly progressive glomerulonephritis (RPGN) and diffuse alveolar hemorrhage (DAH). The main causes of PRS include ANCA associated vasculitis (AAV), anti-GBM disease, or immune complex-mediated glomerulonephritis (GN). Herein, we present a rare case of PRS with positive P-ANCA, but a “full house” crescentic GN on renal biopsy, with a negative ANA panel.

Case Description

A 59-year-old female with history of osteoarthritis presented with productive cough and dyspnea for two days. CT chest showed bibasilar ground glass opacities. Extensive infectious workup was negative. On the third day, she developed fever, hemoptysis, and rapidly progressive dyspnea necessitating intubation. Bronchoscopy revealed DAH. Her creatinine increased from 2.2 mg/dl on admission (baseline Cr 0.93 mg/dl) to 4.5 mg/dl within one week. Urinalysis revealed significant proteinuria (300 mg/dl) and RBC 40-60/HPF. Serologic workup revealed a high P-ANCA titer (> 1:640) and an elevated myeloperoxidase (MPO) antibody of 13. Anti-GBM, ANA, and anti-dsDNA were negative. Complements were normal. Kidney biopsy showed 19/69 glomeruli with cellular/fibrocellular crescent formation. Immunofluorescence (IF) revealed an intense “full house” staining. She was treated with plasmapheresis, steroid, and cyclophosphamide, resulting in the resolution of respiratory failure and improvement in Cr to 2.7 mg/dl upon discharge.

Discussion

Our patient’s clinical picture supports a diagnosis of AAV with renal involvement, which typically presents as a pauci-immune crescentic GN with negative IF on biopsy. The presence of a “full house” IF pattern, suggestive of Lupus nephritis, was unexpected in this case. While P-ANCA can be found in 10% of patients with SLE, the absence of ANA excludes the patient from meeting the new EULEAR/ACR criteria for SLE diagnosis.
SLE/AAV overlap syndrome has been reported as a rare entity, with biopsy features of both diseases. In such cases, the presence of ANCA antibody is often associated with more active lupus disease, higher ds DNA antibody levels, and worse renal function. Our case suggests the potential for AAV to overlap with other autoimmune syndromes, highlighting the importance of further studies to validate this association and develop a treatment strategy.