A case of merged idiopathic portal hypertension in course of mixed connective tissue disease

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.…”

“…For example, platelet-derived growth factor (PDGF), connective tissue growth factor (CTGF) and other cytokines are found to play role in the development of such disorders. These cytokines may result in pathological changes resembling IPH [6] , such as peri-biliary fibrosis in the portal tracts and increased myofibroblastic spindle cell activity in the portal tracts. As previously noted by Nakanuma et al [10] , the increased expression of adhesion molecules in portal venous endothelial lining cells may also reflect the secondary effects of these autoimmune diseases.…”

Idiopathic Portal Hypertension in a Patient with Mixed Connective Tissue Disease and Protein C Deficiency

“…Tokushige et al found that patients with IPH have high serum immunoglobulin levels (1) and activated T cells (2). Several reports have suggested that MCTD plays a role in the pathogenesis of IPH (3)(4)(5)(6)(7)(8). It was speculated that persistently elevated inflammatory cytokine levels in sera are correlated with a high risk of endothelial damage in patients with MCTD (9).…”

“…It was speculated that persistently elevated inflammatory cytokine levels in sera are correlated with a high risk of endothelial damage in patients with MCTD (9). However, IPH associated with MCTD has rarely been reported; there have only been six reports of IPH in MCTD patients (3)(4)(5)(6)(7)(8) (Table 1), and five of the six cases were complicated by esophageal varices. The liver biopsy findings of these six cases revealed inflammatory cell infiltration and mild or severe fibrosis in the portal areas, combined with narrowing or obstruction of the portal vein.…”

“…Although the pathogenesis of IPH is still unknown, IPH is believed to have an association with immunological abnormalities (1,2). However, IPH associated with MCTD is rare (3)(4)(5)(6)(7)(8).…”