Vol.21 No.3

Case Report

Polymyositis associated with autoimmune hepatitis, primary biliary cirrhosis, and autoimmune thrombocytopenic purpura

Authors

Yuko Kurihara1 , Takashi Shishido1 , Kayo Oku1 , Masashi Takamatsu1 , Hiroshi Ishiguro1 , Atsushi Suzuki1 , Tsunejirou Sekita1 , Toshihito Shinagawa2 , Tadayuki Ishihara3 , Ran Nakashima4 , Takao Fujii4 , Yutaka Okano1

  • Department of Medicine, Kawasaki Municipal Ida Hospital, 2-27-1 Ida, Nakahara-ku, Kawasaki 211-0035, Japan
  • Department of Pathology, Kawasaki Municipal Ida Hospital, Kawasaki, Japan
  • Department of Pathology, National Hospital Organization Hakone National Hospital, Odawara, Japan
  • Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan
Received:

11 August 2010

Accepted:

19 November 2010

Published online:

15 January 2011

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Abstract

We describe a 40-year-old woman with polymyositis (PM) who developed autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and autoimmune thrombocytopenic purpura (AITP) concurrently. About 4 years earlier, she suffered from muscle weakness probably due to PM. When she visited our hospital, she had polyarthritis, myalgia, symmetrical proximal limb-muscle weakness, elevated muscle enzymes, and myogenic abnormalities on electromyogram. Pathological findings obtained by muscle biopsy showed histological findings consistent with PM. Her serum liver enzymes were also elevated. The histology obtained by liver biopsy revealed the mixture findings of chronic active hepatitis and biliary cirrhosis. As antibodies to mitochondria M2 and liver/kidney microsome type 1 (LKM-1) were present, we concluded her liver disease was due to an overlap of AIH and PBC. Furthermore, purpura on the legs with thrombocytopenia appeared in parallel with liver dysfunction. She was diagnosed as having AITP by clinical and laboratory findings. Her serum showed a speckled pattern in immunofluorescence antinuclear antibody testing, but the antigen specificities were distinct from those of the known myositis-related autoantigens. This is a first case report of PM accompanied by AIH, PBC, and AITP. It was notable that there was an overlap of disease-associated immunological findings and immunogenetic backgrounds. This case provides a possible insight into the mechanisms and interplay of autoimmune diseases.

Key words

Polymyositis - Autoimmune hepatitis - Primary biliary cirrhosis - Human leukocyte antigen - Antinuclear antibodies