title: A case of merged idiopathic portal hypertension in course of mixed connective tissue disease.
abstract: We report a case of idiopathic portal hypertension (IPH) complicated with mixed connective tissue disease (MCTD). The patient, a 41-year-old woman, was admitted to another hospital because of tarry stools in March 2000. Emergency endoscopy revealed bleeding from the esophageal varices, and she was referred to our hospital. Twelve years before, she had been diagnosed as having MCTD, because she exhibited Raynaud's phenomenon, pleuritis, pericarditis, and had high titers of anti nuclear antibody (ANA) and anti-U1 ribonucleoprotein (RNP). Laboratory examinations, imaging examinations, and liver biopsy indicated that the esophageal varices were caused by IPH. The association of IPH and MCTD is very rare; to the best of our knowledge, only five cases of MCTD, associated with pulmonary hypertension (PH), have been reported.
authors.affiliation: Department of Internal Medicine II, Fukushima Medical University School of Medicine, 1-Hikarigaoka, Fukushima 960-1295, Japan.
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Rai
authors.forename: Hiromasa
authors.initials: H
authors.lastname: Ohira
authors.forename: Etsuko
authors.initials: E
authors.lastname: Fukaya
authors.forename: Kazumichi
authors.initials: K
authors.lastname: Abe
authors.forename: Junko
authors.initials: J
authors.lastname: Yokokawa
authors.forename: Junko
authors.initials: J
authors.lastname: Takiguchi
authors.forename: Shoichiro
authors.initials: S
authors.lastname: Shishido
authors.forename: Yukio
authors.initials: Y
authors.lastname: Sato