Abstract: PUB223
A Case of ANCA-Associated Vasculitis (AAV) in Patients with Systemic Sclerosis (SSc)
Session Information
Category: Trainee Case Report
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Alotaibi, Manal, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
- Zamora-Olivencia, Veronica, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
Introduction
AAV in patients with SSc has been reported rarely. As both diseases can present with renal involvement, diagnosis is challenging. We report the case of a 56 y.o. male with hx of SSc who was admitted for proteinuria AKI found to have biopsy-proven P-ANCA-associated crescentic glomerulonephritis.
Case Description
56 YOM with PMH of SSc, recent hx of sinus and ear infection and progressive B/L SNHL who presented with systemic symptoms. He was seen by Rheumatology in 2013, found to have + ANA 1:1280 homogeneous, SCL-70 and low C3 and diagnosed with SSc. He has progressive bilateral hearing loss in the past five months with B/L ear discharge and otalgia S/P multiple Abx and steriod courses. On the admission, he was vitally stable. His labs are significant for Cre 13.77 mg/dl from Cre baseline 1.1 mg/dl. UA with hematuria, proteinuria, and pyuria with no casts. UPCR 4.93 mg/dl. Renal US showed with B/L renal enlargement and no hydronephrosis. He started on iHD urgently. Further labs were significant for + ANA 1:1280, SCL70, and MPO 595, C3 is low. Anti-GBM was negative. Hemolysis labs and peripheral smear were negative. A kidney biopsy showed severe allergic interstitial nephritis, focal necrotizing arteritis, and few lesions of focal crescentic glomerulonephritis. He treated with Solumedrol x 3 doses and then started on a Prednisone taper. He received his first dose of Rituximab in the hospital and ultimately had renal recovery. Of note, his hospital course was c/b peripheral parasethesia and EMG showed multiple mononeuropathies, consistent with a vasculitic polyneuropathy.
He was discharged and received his second dose of Rituximab. His kidney function, UPCR and MPO titers continued to improve.
Discussion
His AKI is likely related to AAV with overlap SSc. He also has severe interstitial nephritis, which would indicate some drug induced trigger for AAV. There have been rare reports of patients with SSC with ANCA. However, the majority of these patients do not manifest AAV. Studies showed an association between ANCA positivity in patients with SSc and increased mortality and poor prognosis. These findings suggest that ANCA should be tested early in patients with SSc. AAV in SSc is rare with conflicting data about renal outcomes. Rapid recognition is essential for prompt initiation of treatment. Kidney biopsy is the gold standard diagnostic test.