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Life-Threatening Variceal Bleeding in Antisynthetase Syndrome: Nodular Regenerative Hyperplasia and Porto-Sinusoidal
Jazmin Mexia Cabrales1, Hassan Sinan1, Bibhuti Adhikari1
1Department of Internal Medicine, NYC Health + Hospitals/Elmhurst, Icahn School of Medicine at Mount Sinai, Elmhurst, NY.
Abstract:
Antisynthetase syndrome (ASyS) is an autoimmune disorder that may be associated with hepatic involvement, such as nodular regenerative hyperplasia (NRH), a histologic manifestation of porto-sinusoidal vascular disorder, and complications such as variceal bleeding despite preserved liver function. We report a case of life-threatening variceal hemorrhage due to NRH-associated porto-sinusoidal vascular disorder in a patient with ASyS. This case highlights NRH as a cause of severe gastrointestinal bleeding in ASyS and underscores the need for diagnostic and management strategies distinct from those used in cirrhosis.
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