Case of idiopathic portal hypertension complicated with autoimmune hepatitis

Abstract

We report a case of idiopathic portal hypertension (IPH) complicated with autoimmune hepatitis. A 60-year-old woman was admitted to our hospital with esophageal and gastric varices in February 2010. Abdominal ultrasonography and computed tomography showed splenomegaly and collateral veins without evidence of liver cirrhosis. Laboratory examinations and liver biopsy indicated that the esophageal and gastric varices were caused by IPH. She underwent endoscopic injection sclerotherapy and partial splenic embolization. Two years after these therapies, laboratory examinations showed liver dysfunction with elevated levels of aspartate aminotransferase (180 IU/L), alanine aminotransferase (190 IU/L), γ-glutamyl transpeptidase (159 IU/L) and immunoglobulin G (2609 mg/dL). The titer of antinuclear antibodies was 1:320 and its pattern was homogeneous and speckled. Histological examination revealed plasma cell/lymphocyte infiltration and interface hepatitis in the portal tract. Based on these findings, a diagnosis of autoimmune hepatitis accompanied by IPH was made. After treatment with prednisolone (20 mg/day), liver functions were normalized immediately. Overlapping of IPH and AIH is extremely rare, but the present case is interesting considering the etiology of IPH because an autoimmune mechanism is thought to be involved in the pathogenesis of IPH.

Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

“…As autoimmune diseases frequently occur in middle‐aged females, this may potentially explain the older age at onset in female patients. Moreover, several cases of IPH with concomitant autoimmune hepatitis, mixed connective tissue disease, autoimmune polyglandular syndrome III, or Hashimoto's thyroiditis have been reported …”

“…According to the APASL recommendations, signs of cirrhosis or parenchymal injury and serum markers of hepatitis B or C need to be excluded for the diagnosis of IPH . However, cases of IPH with concomitant autoimmune hepatitis have been reported . Moreover, in our clinical practice, cases of IPH with pathological evidence of other concurrent liver diseases (especially non‐vascular liver diseases, such as hepatitis, drug‐induced liver injury, Gilbert disease, and bile duct disease) are not rare.…”

“…Moreover, several cases of IPH with concomitant autoimmune hepatitis, mixed connective tissue disease, autoimmune polyglandular syndrome III, or Hashimoto's thyroiditis have been reported. 12,[47][48][49] From a microcosmic perspective, the pathology of IPH is characterized by fibrotic expansion of intermediate-size portal tracts, round-shaped fibrous expansion of portal tracts, and narrowing, collapse, and loss of portal vein branches; in addition, aberrant vessels adjacent to portal tracts are a prominent feature in most peripheral portal tracts. 43 Thus, portal fibrosis and obliteration of the small portal vein may be the main pathological process that leads to portal hypertension in IPH.…”

“…[7][8][9][10] The etiology of IPH is still not clear. Some of the proposed etiological factors have included immunologic disorders, [11][12][13][14][15][16] chronic infection with some bacteria or human immunodeficiency virus, 13,[17][18][19] medication and toxins, [20][21][22][23][24] gene mutation, [25][26][27][28][29] and some other etiologies like dialysis. 30 The main clinical symptoms of IPH are those caused by portal hypertension, including splenomegaly, hypersplenism, hepatomegaly, gastrointestinal bleeding, ascites, and portal vein thrombosis (PVT).…”

Diagnosis and treatment guidelines for aberrant portal hemodynamics

“…[20][21][22][23][24] The etiology of the disease remains unclear, but hypotheses of intrahepatic peripheral portal thrombus, a spleen origin, and autoimmune disorders have been suggested. [25][26][27][28][29][30][31][32][33]…”