Dermatomyositis Associated with Primary Biliary Cirrhosis

Autor: Akisaka, Masafumi, Adachi, Masanori, Suzuki, Makoto, Shokita, Hayashi, Sakugawa, Hiroshi, Saito, Atsushi
Jazyk: japonština
Rok vydání: 1993
Předmět:
Zdroj: 琉球医学会誌 = Ryukyu Medical Journal. 13(1):63-69
ISSN: 1346-888X
Popis: A 66-year-old woman was admitted to our hospital because of exacerbation of dermatomyositis. She had been treated as dermatomyositis since 1986. She also suffered from liver cirrhosis of unknown etiology and diabetes mellitus. She complained of fever, muscle weakness and myalgia in proximal extremities. Physical examination disclosed body temperature of 37℃, a sealy erythema on her forehead, purpuric rash of the eyelids, cyanosis, clubbing of the fingers and toes. Symmetrical atrophies in proximal limb muscle were evident and museles were tender to palpation. Auscultation of the heart revealed a midsystolic murmur in apex. Dry rales were heard in bilateral bases of the lung. Abdominal palpation showed a hepatosplenomegaly. Laboratory findings include erythrocyte sedimentation rate 100 mm/h, serum phosphokinase 326 U/L, serum GOT 98 IU/L, serum LDH 718 IU/L, serum total bilirubin 2.2 mg/dl, serum myoglobin 84 ng/dl. Antimitochondrial antibody was positive. The electromyogram study was normal but the muscle biopsy showed slight myopathic change. The liver biopsy demonstrated lymphocytes infiltration, destruction of limiting membranes, which were compatible with Primary Bilialy Cirrhosis(PBC). The final diagnosis of her illness ineluded 1) dermatomyositis, 2) PBC, 3) pulmonary fibrosis and 4) diabetes mellitus. Clinical manifestations gradually improved with predonisolone therapy. However, because of hypoxia and congestive heart failure, rehabilitation for muscle weakness could not be done sufficiently. Dermatomyositis associated with PBC is very rare, although 21 patients with polymyositis associated with PBC were reported to our best knowledge.
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Databáze: OpenAIRE