Chronic myositis with cardiomyopathy and respiratory failure associated with mild form of organ-specific autoimmune diseases.
We report the four patients with chronic myositis characterized by a very slow progression with cardiomyopathy and frequently with marked respiratory muscle weakness associated with other organ-specific autoimmune diseases such as primary biliary cirrhosis. The histopathology of the muscle showed many degenerative and regenerative fibers, but inflammatory-cell infiltration were minimal. The patients showed favorable response to high-dose corticosteroid treatment. Because of these clinical features, these patients are sometimes misdiagnosed as muscular dystrophy and not treated properly. It is important to distinguish this type of treatable myositis.
Department of Neurology, Brain Research Institute, Niigata University, Niigata City, Niigata, 951-8585, Japan. keiko@bri.niigata-u.ac.jp
K
K
Tanaka
A
A
Sato
K
K
Kasuga
M
M
Kanazawa
K
K
Yanagawa
M
M
Umeda
M
M
Tada
M
M
Tanaka
M
M
Nishizawa
D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications* / Q000175:diagnosis; D009202:Cardiomyopathies / Q000150:complications* / Q000175:diagnosis; D002908:Chronic Disease; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008875:Middle Aged; D009132:Muscles / Q000473:pathology; D009220:Myositis / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000473:pathology*; D011928:Raynaud Disease / Q000150:complications / Q000175:diagnosis; D012074:Remission Induction; D012131:Respiratory Insufficiency / Q000150:complications* / Q000175:diagnosis; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis; D016896:Treatment Outcome