Non-cirrhotic Idiopathic portal hypertension in systemic sclerosis patients: report of one case and a systematic review of previous case reports.

Abstract

Background: The overlap of non-cirrhotic idiopathic portal hypertension (NCIPH) and systemic sclerosis (SSc) is rare. This article reports one case of a patient with SSc developing NCIPH and presents a systematic review of previously reported cases.

Methods: CARE guidelines and the PRISMA statement were applied.

Results: We report the case of a 52 year-old woman, presenting, in 2015, diffuse cutaneous scleroderma (SSc), treated with oral prednisolone and monthly intravenous cyclophosphamide. Three months later, she developed a scleroderma renal crisis, requiring hemodialysis for 18 months. Since 2017 she has not been on immunosuppressive treatment for SSc, the cutaneous involvement improved, and she has a stable Kdigo 3 chronic kidney disease. In 2019, she developed ascites. During investigation, NCIPH leading to small and medium esophageal varices and collateral circulation was diagnosed. Currently, the patient is undergoing prophylactic endoscopic band ligation of the esophageal varices and presents a stable condition. In the systematic review, 18 papers reporting 20 cases of NCIPH associated with SSc were included. Seventeen (81%) patients were women, with [Mean (SD)]: 56.71 (12.97) years. Classification of SSc was (N = 15): 10 limited, 4 diffuse, and 1 sin scleroderma. Clinical presentation of NCIPH was esophageal and/or gastric varices [19 (90,5%)], ascites [10 (47,6%)], and upper gastrointestinal bleeding [9 (42,8%)]. NCIPH was treated with diuretics [n = 9 (42,8%)], endoscopic esophageal varices sclerosis or band ligation [n = 7 (35%)], and beta-blockers [n = 4 (19%)]. Recovery of symptoms, or stabilization of clinical condition was reported in nine patients. Despite the death of seven patients, only one was attributed to the hepatic condition.

Conclusions: NCIPH has been rarely reported in SSc patients. NCIPH prognosis in SSc is good. Due to the scarcity of cases reporting the occurrence of both diseases, the characteristics of SSc patients at risk of developing NCIPH remain unclear.