Abstract: PO1614
A Severe Presentation of Systemic Lupus Erythematosus (SLE) and ANCA-Associated Vasculitis (AAV) Overlap Syndrome
Session Information
- Glomerular Diseases: Clinicopathological Features and Outcomes in IgAN, Lupus Nephritis, and Vasculitis
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Khil, Jaclyn, Kaiser Permanente Oakland Medical Center, Oakland, California, United States
- Nguyen, Thuy Minh, Kaiser Permanente Oakland Medical Center, Oakland, California, United States
- Zheng, Sijie, Kaiser Permanente Oakland Medical Center, Oakland, California, United States
Introduction
An increasingly recognized overlap syndrome (OS) of lupus nephritis and ANCA positive crescentic glomerulonephritis is rare and presents treatment challenges. It's not clear whether to treat as LN or ANCA vasculitis. Here we describe a severe presentation of OS, the subsequent management and positive outcome.
Case Description
A 42 y.o. woman with no PMH presented with 3 weeks of cough, dyspnea, fever, malaise, AKI and hypoxia. Her hgb was 6.3 g/dL and creatinine 17.4 mg/dl (normal 1.5 years prior). UA showed large hgb and >182 RBCs and UPCR 5.72 g/g. Serologies were significant for elevated DsDNA, RNP ab, Smith ab and positive ANA with low complement levels. MPO ab was elevated to >8.0 (<=0.9) and anti GBM negative. Cardiolipin ab was elevated but beta 2 glycoprotein ab and lupus anticoagulant ab were negative. Chest CT showed multifocal opacities. She was urgently started on hemodialysis and solumedrol 1g for 3 days then prednisone 60mg daily. On day 2 of admission she underwent renal biopsy. Her course was complicated by hemoptysis and bronchoalveolar lavage showed diffuse alveolar hemorrhage (DAH). She was initiated on plasmapheresis. Biopsy demonstrated necrotizing crescentic GN with immunofluorescence showing full house pattern and complex deposits with strong ANA staining of nuclei. Given the biopsy results and serologies the patient was diagnosed with LN/AAV overlap. The patient was started on Cytoxan 750mg monthly administration. She completed 7 daily sessions of plasmapheresis and renal function recovered sufficiently to stop dialysis. Two months after discharge, her renal function continued to improve with her most recent creatinine down to 2 mg/dl.
Discussion
LN and AAV overlap is rare and there are no guidelines regarding management of these patients. This case stresses the importance of having a high suspicion of LN/AAV when a young, female patient presents with new onset renal failure and DAH. The patient benefited from early, aggressive treatment targeting both disease processes including early initiation of high dose steroids. Secondly, plasma exchange should be initiated emergently with severe presentation, including DAH. Plasmapheresis did not reduce incidence of ESKD in PEXIVAS trial, however, we suspect in this case it contributed to the good outcome. Lastly, little is known about outcomes in these patients, but this is an example of a severe presentation with a positive outcome.