Frail Silk: Is the Hughes-Stovin Syndrome a Behcet Syndrome Subtype with Aneurysm-Involved Gene Variants?

ABSTRACT: Hughes-Stovin syndrome is a rare disease characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. The etiology and pathogenesis of HSS are incompletely known. The current consensus is that vasculitis underlies the pathogenic process, and pulmonary thrombosis follows arterial wall inflammation. As such, Hughes-Stovin syndrome may belong to the vascular cluster with lung involvement of Behçet syndrome, although oral aphtae, arthritis, and uveitis are rarely found. Behçet syndrome is a multifactorial polygenic disease with genetic, epigenetic, environmental, and mostly immunological contributors. The different Behçet syndrome phenotypes are presumably based upon different genetic determinants involving more than one pathogenic pathway. Hughes-Stovin syndrome may ha

SUBMITTER: Manole S 

PROVIDER: S-EPMC9968083 | biostudies-literature | 2023 Feb

REPOSITORIES: biostudies-literature