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.