MIXED CONNECTIVE-TISSUE DISEASE-ASSOCIATED WITH IDIOPATHIC PORTAL-HYPERTENSION AND CHRONIC THYROIDITIS

We report a patient with mixed connective tissue disease (MCTD) associ ated with idiopathic portal hypertension (IPH) and chronic thyroiditis . The patient was a 68-year-old Japanese woman who was admitted to our hospital for treatment of bleeding esophageal varices. She had previo usly exhibited Raynaud's phenomenon and had had arthritis for about 30 years. She also had had high titers anti-U1 of ribonucleoprotein (RNP ) anti-single strand-DNA autoantibodies for 2 years, and had been diag nosed with MCTD 1 year previously, The bleeding from esophageal varice s was successfully stopped by endoscopic injection sclerotherapy. Resu lts of laboratory examinations, imaging examinations, and laparoscopy, including liver biopsy, indicated that the esophageal varices were ca used by portal hypertension due to IPH. The patient also had a diffuse ly firm and enlarged goiter and hypothyroidism, and she exhibited anti -thyroid microsomal antibodies and anti-thyroglobulin antibodies, she was diagnosed as having a complication of chronic thyroiditis. This as sociation of MCTD, IPH, and chronic thyroiditis is quite rare and prov ides a unique opportunity to observe immunological involvement in the pathogenesis of IPH.