Systemic Sclerosis and Idiopathic Portal Hypertension: Report of a Case and Review of the Literature

SIMPLE SUMMARY: The presence of liver involvement in systemic sclerosis (SSc) is considered atypical, besides the eventual coexistence of other autoimmune hepatic disorders. However, the occurrence of syndromes called idiopathic portal hypertension (IPH) and regenerative nodular hyperplasia (RNH) have been anecdotally reported in the literature. We described a case of SSc complicated by IPH and we reviewed the literature on the topic. No specific SSc pattern linked to IPH emerged, even though the reports often described IPH in patients with limited skin subset SSc. Coexistence of prothrombotic states and overlap with other hepatic diseases could facilitate IPH onset. In spite of IPH being a rare condition, the rheumatologists should consider IPH as a possible hepatic complication in SSc patients. ABSTRACT: The presence of liver involvement in systemic sclerosis (SSc) is considered atypical, besides the possible coexistence of other autoimmune hepatic disorders. However, the occurrence of portal hypertension and, more specifically, of the syndromes called idiopathic portal hypertension (IPH) and regenerative nodular hyperplasia (RNH) have been anecdotally reported in the literature for SSc patients. We described a case of SSc woman complicated by IPH; moreover, we reviewed the literature on the topic. A 61-year-old female SSc patient was admitted to our hospital because of the onset of ascites. SSc, as a limited skin subset of disease with anticentromere antibodies, was diagnosed 11 years previously, with no significant visceral involvement. We excluded possible causes of portal hypertension, namely chronic infections, autoimmune hepatic diseases, neoplasia, thrombosis of portal vein, and Budd–Chiari syndrome. Finally, IPH was diagnosed. A review of the literature identified a number of case reports or case series that described IPH in the course of SSc. No specific SSc pattern linked to IPH emerged, even though reports from the literature often described the limited skin subset. Coexistence of prothrombotic states and overlap with other hepatic diseases could facilitate IPH onset. Besides being a rare condition, the onset of IPH in SSc patients is an occurrence that should be taken into account.