Abstract: FR-PO0755
An Unusual Presentation of ANCA-Associated Glomerulonephritis, Graves Disease, and Multiple Autoimmune Syndrome in a Patient with Morphea
Session Information
- Glomerular Diseases: ANCA Vasculitis, Anti-GBM Disease, and Crescentic GN
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Nazar, Tanya, St. Francis Medical Center, Monroe, Louisiana, United States
- Patel, Mizba Basheer, St. Francis Medical Center, Monroe, Louisiana, United States
- Pierce, Edward, St. Francis Medical Center, Monroe, Louisiana, United States
Introduction
Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of small vessel vasculitides with renal involvement, frequently presenting as rapidly progressive glomerulonephritis. Here we describe a rare presentation of multiple autoimmune syndrome (MAS) with coexisting autoimmune disorders including morphea, ANCA-associated glomerulonephritis, and Graves' disease; a combination not previously reported.
Case Description
A 60-year-old female with history of localized scleroderma (morphea) of the left breast presented with generalized weakness, arthralgia and bilateral leg edema. Initial labs revealed pancytopenia and renal failure with creatinine of 4.69 mg/dL and UPCR of 7,344 mg/g (reference <200 mg/g). She was initiated on dialysis. Renal biopsy demonstrated crescent formation in 5/16 glomeruli and mild immune deposition, consistent with ANCA-associated glomerulonephritis.
Further workup showed positive anti-dsDNA antibody, p-ANCA (1:80), elevated anti-MPO, and low C3. Thyroid testing reported subclinical hyperthyroidism with positive thyrotropin receptor antibody indicative of Graves' disease. The patient was started on prednisone, mycophenolate, and rituximab.
Discussion
This is a unique presentation of MAS in a patient with morphea. Emerging evidence supports morphea and systemic sclerosis (SSc) as a shared spectrum with a higher autoimmune risk. The co-occurrence of AAV with SSc is rare, and MPO-ANCA positivity is seen in 93% of such cases. Clinicians should maintain a high index of suspicion as AAV and scleroderma renal crisis require different treatments. The biopsy showed ANCA-associated glomerulonephritis with atypical immune deposition, a variant associated with higher proteinuria, lower C3, and poor renal outcomes. Per KDIGO 2024 guidelines, first-line therapy for AAV is prednisone with either cyclophosphamide or rituximab. Alternative therapy with mycophenolate is desirable in this overlap patient, as it reduces skin fibrosis in scleroderma in addition to immunosuppression. MPO-ANCA-positive crescentic glomerulonephritis has been reported in Graves' disease, highlighting the significance of screening for autoimmune thyroid disease in patients with AAV. This case emphasizes the need for AAV evaluation in patients with renal failure and other autoimmune conditions.