Abstract: SA-PO0797
Moyamoya-Pattern Vasculopathy as Initial Manifestation of Systemic Lupus Erythematosus with Possible Antiphospholipid Syndrome
Session Information
- Glomerular Diseases: Lupus Nephritis, Monoclonal Gammopathy-Related Disease, and Transplantation
October 24, 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
- Patel, Vraj, Temple University Health System Inc, Philadelphia, Pennsylvania, United States
- Aggarwal, Kanika, Temple University Health System Inc, Philadelphia, Pennsylvania, United States
- Boyle, Suzanne, Temple University Health System Inc, Philadelphia, Pennsylvania, United States
- Koul, Sheetal, Temple University Health System Inc, Philadelphia, Pennsylvania, United States
Introduction
Moyamoya disease is a diagnosis of exclusion because characteristic angiographic findings may also occur secondary to systemic disorders. Autoimmune diseases, including systemic lupus erythematosus (SLE), can produce an indistinguishable vasculopathy more appropriately classified as moyamoya syndrome(MMS), with important clinical implications.
Case Description
A 26-year-old woman was referred for evaluation of elevated creatinine (1.67 mg/dL) and proteinuria (urine protein-to-creatinine ratio 864 mg/g). Kidney biopsy demonstrated membranoproliferative glomerulonephritis with “full-house” immunofluorescence staining and severe interstitial fibrosis (60–70%), highly suggestive of lupus nephritis. Serologic evaluation revealed ANA positivity (1:640) with equivocal anti-dsDNA antibodies, supporting a diagnosis of SLE. Her history was notable for ischemic stroke at age 21. Cerebral angiography at that time demonstrated severe stenosis/occlusion of the distal right M1 segment with reconstitution of distal M2 branches, interpreted as moyamoya-pattern vasculopathy. Autoimmune evaluation then showed discordant ANA testing, with positive ELISA but negative indirect immunofluorescence. Serum creatinine was normal, although urinalysis showed 1+ proteinuria. She subsequently underwent right indirect internal carotid–external carotid bypass surgery. Hypercoagulable workup demonstrated positive lupus anticoagulant screening, positive beta-2 glycoprotein IgG, low-medium positive anticardiolipin IgG, and indeterminate anticardiolipin IgM. However, repeat testing after 12 weeks was not obtained, precluding formal diagnosis of antiphospholipid syndrome (APS).
Discussion
Incomplete autoimmune evaluation in a young patient with moyamoya-pattern vasculopathy delayed recognition of systemic disease. Five years after her cerebrovascular event, kidney biopsy confirmed lupus nephritis in previously unrecognized SLE. Concurrent antiphospholipid antibody positivity raised concern for APS-associated vasculopathy, although classification criteria could not be fulfilled without repeat confirmatory testing. The marked tubulointerstitial fibrosis at presentation suggests prolonged subclinical autoimmune disease preceding definitive diagnosis. MMS may represent the earliest manifestation of SLE, and timely recognition may alter surveillance, immunosuppressive management, and long-term outcomes.