Triple Autoantibody Positivity in Isolated Portal Vein Thrombosis: Expanding the Spectrum of Autoimmune Vasculitic Overlap Syndrome
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Abstract
Background: Autoimmune-mediated vascular injury is an uncommon but important cause of non-cirrhotic portal hypertension (NCPH) and splanchnic vein thrombosis. The concurrent presence of C-ANCA, P-ANCA, and Anti-La (SSB) antibodies has been very rarely reported in association with portal vein thrombosis (PVT).
Case Presentation:
We report a rare case of a 55-year-old female with a known history of ascites for 2 years, presenting with worsening abdominal distension and malena for 15 days. Evaluation revealed non-cirrhotic portal hypertension with thrombosis extending from the portal vein to the confluence and right main portal vein. She tested positive for C-ANCA, P-ANCA, and Anti-La antibodies without systemic vasculitis or classic autoimmune disease features.
Conclusion:
This rare autoimmune overlap syndrome highlights the need to consider vasculitic and autoimmune mechanisms in unexplained PVT in non-cirrhotic patients. Early recognition and immunological testing can guide management and prevent complications.
Keywords: Autoimmune vasculitis, portal vein thrombosis, non-cirrhotic portal hypertension, C-ANCA, P-ANCA, Anti-La, overlap syndrome
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