Idiopathic portal hypertension complicating systemic sclerosis: A case report

被引：0

作者：

Moschos J. ^{[1
]}

Leontiadis G.I. ^{[1
]}

Kelly C. ^{[2
]}

Henry J. ^{[3
]}

Kadis S. ^{[1
]}

机构：

[1] Department of Gastroenterology, Queen Elizabeth Hospital, Gateshead NE6 9SX, Sheriff Hill

[2] Department of Rheumatology, Queen Elizabeth Hospital, Gateshead NE6 9SX, Sheriff Hill

[3] Department of Pathology, Queen Elizabeth Hospital, Gateshead NE6 9SX, Sheriff Hill

来源：

BMC Gastroenterology | / 5卷 / 1期

关键词：

Portal Vein; Portal Hypertension; Primary Biliary Cirrhosis; Systemic Sclerosis; Lansoprazole;

D O I：

10.1186/1471-230X-5-16

中图分类号：

学科分类号：

摘要：

Background: Patients with systemic sclerosis may develop mild abnormalities of liver function tests. More serious hepatic involvement has been well documented but is rare. Idiopathic portal hypertension had been reported only in a few female patients with systemic sclerosis. Case presentation: An 82-year-old man with known systemic sclerosis presented with melaena. Urgent gastroscopy revealed oesophageal varices, which re-started bleeding during the procedure and were treated ensocopically, with Sengstaken tube and glypressin. Liver function tests and coagulation were normal. Non-invasive liver screen (including hepatitis viral serology and autoantibodies) was negative. Ultrasound scan of the abdomen revealed a small liver with coarse texture and no focal lesion. Hepato-portal flow was demonstrated in the portal vein. The spleen was enlarged. A moderate amount of free peritoneal fluid was present. A CT scan confirmed the absence of portal vein thrombosis. One month following discharge the patient had a liver biopsy. Histological examination showed essentially normal liver tissue; there was no evidence of any excess inflammation and no features to suggest cirrhosis or drug-induced liver disease. Taking into account the above evaluation we concluded that the patient had idiopathic portal hypertension. Conclusion: Both male and female patients with systemic sclerosis may - rarely - develop idiopathic portal hypertension. © 2005 Moschos et al., licensee BioMed Central Ltd.

引用

页数：3

共 10 条

[1]

Youssef W.I., Tavill A.S., Connective tissue diseases and the liver, J. Clin. Gastroenterol., 35, pp. 345-349, (2002)

[2]

Walker N.J., Zurier R.B., Liver abnormalities in rheumatic diseases, Clin. Liver Dis., 6, pp. 933-946, (2002)

[3]

Saito K., Nakanuma Y., Takegoshi K., Ohta G., Obata Y., Okuda K., Kameda H., Non-specific immunological abnormalities and association of autoimmune diseases in idiopathic portal hypertension. A study by questionnaire, Hepatogastroenterology, 40, pp. 163-166, (1993)

[4]

Terada T., Nakanuma Y., Hoso M., Obata H., Expression of HLA-DR antigen on hepatic vascular endothelial cells in idiopathic portal hypertension, Clin. Exp. Immunol., 84, pp. 303-307, (1991)

[5]

D'Angelo W.A., Fries J.F., Masi A.T., Shulman L.E., Pathologic observations in systemic sclerosis (scleroderma): A study of fifty-eight autopsy cases and fifty-eight matched controls, Am. J. Med., 46, pp. 428-440, (1969)

[6]

Tsuneyama K., Harada K., Katayanagi K., Watanabe K., Kurumaya H., Minato H., Nakanuma Y., Overlap of idiopathic portal hypertension and scleroderma: Report of two autopsy cases and review of literature, J. Gastroenterol. Hepatol., 17, pp. 217-223, (2002)

[7]

Watanabe Y., Mizukami T., Egawa T., Okamoto S., Sakauchi M., Takita T., Suzuki N., Sakuma M., A case of progressive systemic sclerosis complicated by idiopathic portal hypertension with severe anaemia, Ryumachi, 39, pp. 586-590, (1999)

[8]

Ishii M., Katada Y., Idiopathic portal hypertension in a systemic sclerosis patient heterozygous for factor V Leiden mutation, Rheumatol. Int., 23, pp. 44-46, (2003)

[9]

Dhiman R.K., Chawla Y., Vasishta R.K., Kakkar N., Dilawari J.B., Trehan M.S., Puri P., Mitra S.K., Suri S., Non-cirrhotic portal fibrosis (Idiopathic portal hypertension): Experience with 151 patients and a review of the literature, J. Gastroenterol. Hepatol., 17, pp. 6-16, (2001)

[10]

Okuda K., Kono K., Ohnishi K., Kimura K., Omata M., Koen H., Nakajima Y., Musha H., Hirashima T., Takashi M., Et al., Clinical study of eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly, Gastroenterology, 86, pp. 600-610, (1984)

← 1 →