Abstract: SA-PO870
A Diagnostic Dilemma of Lupus Nephritis and ANCA-Associated Vasculitis Overlap Syndrome
Session Information
- Glomerular Diseases: Case Reports - 2
October 26, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Remedios, Kimberly, Yale New Haven Health - Bridgeport Hospital, Bridgeport, Connecticut, United States
- Montalvo Campana, Mateo, Yale New Haven Health - Bridgeport Hospital, Bridgeport, Connecticut, United States
- Marnet, Erica, Yale New Haven Health - Bridgeport Hospital, Bridgeport, Connecticut, United States
- Gunasekaran, Deepthi, Yale University School of Medicine, New Haven, Connecticut, United States
Introduction
Systemic lupus nephritis (SLE) is characterized by the development of antinuclear antibodies (ANAs) and immune complex deposition in the kidney. Antineutrophilic cytoplasmic antibodies (ANCA) associated vasculitis (AAV) predominantly affects small vessels and is typically without immune complex deposition. It is challenging to determine if ANCA positivity in SLE patients is part of lupus nephritis (LN) , or whether two different processes occur concurrently
Case Description
An 18-year-old female, without co-morbidities presented with hematuria, dysuria, and headache for one week. She was febrile on presentation. Laboratory tests were significant for acute kidney injury (AKI) and anemia. Treatment started with intravenous fluids and antibiotics. Subsequently, she developed hemoptysis, with a consequent drop in hemoglobin. She became hypoxic, with worsening AKI and required intubation, mechanical ventilation and initiation of hemodialysis. Bronchoscopy showed DAH. She was initiated on steroids and plasma exchange for concern of rapidly progressive glomerulonephritis and pulmonary renal syndrome. Serologies were positive for antinuclear antibodies (1:320), anti-double stranded DNA (51 IU/ml) antibodies, Myeloperoxidase antibodies. Light microscopy showed mesangial hypercellularity with one fibrocellular crescent (Figure 1). IF was positive for IgA, IgM, IgG, C1q negative. She was started on cyclophosphamide and steroid. She was extubated and remained on dialysis
Discussion
It is a challenge to distinguish AAV from LN and the dilemma in treatment options for the two cases. ANCA positivity has been reported to be associated with more severe lupus nephritis in some cases while others have discounted it as a laboratory artefact. Her presentation of DAH is more often associated with ANCA vasculitis along with classic pathology of lupus nephritis in the form of immune complex deposition supports the diagnosis of SLE/ANCA vasculitis overlap syndrome
Figure 1: Light microscopy