Idiopathic portal hypertension associated with systemic lupus erythematosus

Abstract

A case of idiopathic portal hypertension (IPH) associated with systemic lupus erythematosus (SLE) is reported in a 38-year-old man who had been diagnosed with SLE and treated for 18 years. Esophageal varices. found in 1994 on endoscopic examination, had been followed up for 2 years. On July 16, 1996, he was admitted to Nagoya University Hospital because there was a high risk of bleeding from the esophageal varices due to severe thrombocytopenia. As partial splenic embolization had temporarily controlled the thrombocytopenia, splenectomy and devascularization of the stomach vessels were performed after endoscopic ligation of the esophageal varices. Histological specimens of wedge biopsied liver showed chronic inactive hepatitis without cirrhosis. The presence of anticardiolipin antibody, indicated by positivity for lupus anticoagulant, was suggestive of the presence of a common immunological mechanism in the etiology of SLE and IPH.

Clinical characteristics of idiopathic portal hypertension

“…However, it is widely accepted that autoimmune diseases (especially connective tissue diseases) increases the prevalence of IPH in certain patient groups. The most important IPH associated diseases are mixed connective tissue disease (6 cases [6] ), systemic sclerosis (20 cases [7] ) and systemic lupus erythematosus (16 cases [8] ). In addition, a Japanese survey [9] found that hypergammaglobulinemia is the most common presenting autoimmune dysfunction (26% of all included IPH patients), along with chronic thyroiditis as the most common autoimmune disease.…”

Idiopathic Portal Hypertension: Report of a Case

“…In one fourth of the cases, esophageal varices are not completely eradicated after transabdominal devascularization procedure, and adjuvant endoscopic sclerotherapy can be performed afterwards with satisfactory results [14,15]. Some authors [15,19,32] support the idea of combining endoscopic treatment (EIS or EVL) with non-shunt operation for esophageal varices due to IPH. This case report demonstrates a complete eradication of varices after Hassab-Paquet procedure.…”

“…The exact causes of IPH remain unclear, but some studies underlined a potential role of immunological abnormalities in these patients [1,31,32]. A large survey of IPH in Japan disclosed that 11.9% of cases were associated with one or two autoimmune diseases and 26.3% of them showed hypergammaglobulinemia [31].…”

Non‐cirrhotic portal hypertension: Why is it so common in India?

“…In Japan, autoimmune disorders are frequently associated with IPH, such as systemic lupus erythematosus (LE) with positive LE cell phenomenon, 33,34 lupus anticoagulant, progressive systemic sclerosis, chronic thyroiditis, mixed connective tissue disease and Raynaud's phenomenon. [35][36][37] In fact, in this same issue of the Journal of Gastroenterology and Hepatology, two cases of IPH-associated scleroderma are described.…”