SHM Converge Scientific Abstract ePoster Gallery-Beyond Sjogren's: When Mpo-Anca Unmasks Multiorgan Vasculitis

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This case highlights why tissue diagnosis matters more than serology alone in complex autoimmune presentations. A 64-year-old woman had 3 months of progressive bilateral calf/foot pain and weakness, fatigue, weight loss, intermittent fevers, gross hematuria, anemia, inflammatory markers, hepatocellular/cholestatic liver injury, hepatomegaly, proteinuria, and multiple positive autoantibodies including ANA, SSA/SSB, MPO-ANCA, RF, and ASMA.<br /><br />Although the serologies could suggest Sjögren’s syndrome, autoimmune hepatitis, or a Sjögren’s–ANCA overlap syndrome, the patient’s kidney and liver biopsies clarified the diagnosis. Kidney biopsy showed pauci-immune crescentic glomerulonephritis with severe small-vessel vasculitis, consistent with MPO-ANCA–associated vasculitis (AAV). Liver biopsy showed portal tract arteritis/vasculitis, obliterative portal venopathy, and nodular regenerative hyperplasia, consistent with porto-sinusoidal vascular disease (PSVD), not autoimmune hepatitis.<br /><br />The case demonstrates that multiple positive autoantibodies do not necessarily mean multiple autoimmune diseases. Formal diagnostic criteria and organ pathology are essential because treatment decisions differ substantially. For example, Sjögren’s renal disease usually causes tubulointerstitial nephritis, not the hematuria and proteinuria seen here, and PSVD requires monitoring for portal hypertension rather than treatment as autoimmune hepatitis.<br /><br />The patient was treated promptly with IV methylprednisolone followed by prednisone and rituximab induction, along with PJP prophylaxis and multidisciplinary follow-up. Her course was complicated by steroid-induced diabetes.<br /><br />Key takeaways: biopsy should be strongly considered when proteinuria and hematuria suggest glomerular disease, or when liver injury with hepatomegaly suggests vascular liver disease; early immunosuppression and coordinated multidisciplinary care can prevent irreversible organ damage.

Asset Subtitle

Christopher Loh

Meta Tag

Author List Christopher Loh

Category Clinical Vignettes

Concept Tissue Diagnosis

Concept Kidney Biopsy

Concept ANCA-Associated Vasculitis

Concept Pauci-Immune Crescentic Glomerulonephritis

Concept MPO-ANCA Vasculitis

Distinguished Non-Finalist

Presenter Organization Stony Brook University Hospital

Presenting Author Christopher Loh

Track Adult

Keywords

tissue diagnosis

autoimmune serology

MPO-ANCA vasculitis

pauci-immune crescentic glomerulonephritis

portal-sinusoidal vascular disease

liver biopsy

kidney biopsy

Sjögren syndrome overlap

rituximab treatment

proteinuria hematuria

Tissue Diagnosis

Kidney Biopsy

ANCA-Associated Vasculitis

Pauci-Immune Crescentic Glomerulonephritis

MPO-ANCA Vasculitis