Anti-centromere antibody positivity with coexisting idiopathic portal hypertension and primary biliary cholangitis progressing to limited cutaneous systemic sclerosis: a case report

This is the first documented case of hepatic and systemic microvascular impairment observed in an anti-centromere antibody-positive patient. The pathological evidence of endothelial damage in the liver suggests that the "idiopathic" label of idiopathic portal hypertension may need reconsideration in the context of anti-centromere antibody-related pathophysiology, potentially warranting a unifying concept such as "anti-centromere antibody-related systemic microangiopathy syndrome." While our case may provide novel insights into anti-centromere antibody-driven microvascular dysfunction across multiple organ systems, the findings are preliminary. Future studies involving larger cohorts and detailed mechanistic analyses are necessary to confirm the systemic and hepatic effects of anti-centromere antibodies.