Maruyama T, Koh C S, Inoue A, Yanagisawa N, Shinoda T
Department of Medicine (Neurology), Shinshu University School of Medicine.
Rinsho Shinkeigaku. 1991 Nov;31(11):1208-13.
We report a case of "steroid and immunosuppressant-resistant" polymyositis that was successfully treated with immunoadsorption. A 46-year-old man was admitted to Shinshu University Hospital on June 2, 1989, with the chief complaint of muscle weakness and pain. He had a 1.5-year history of frequent episodes of Raynaud's phenomenon. Five months before presentation, he noticed muscle weakness and pain in the proximal muscles of his extremities. One month after the onset of these symptoms, his body temperature increased to 38 degrees C and remained elevated until the following month. He subsequently entered a local hospital. He was found to have diminished strength in all limbs that was more marked in the proximal areas. The muscle weakness and pain progressed until the patient was no longer able to walk. Muscle biopsy of the left quadriceps femoris showed mononuclear cell infiltration in the endomyosium that was accompanied by phagocytosis and interstitial fibrosis. Based on these findings, a diagnosis of polymyositis was made. During hospitalization, the weakness continued to worsen despite pulse therapy with methylprednisolone 1000 mg per day for 3 days and the administration of prednisolone 100 mg and azathioprine 100 mg per day. The patient was transferred to our hospital for further evaluation and treatment. On physical examination, he demonstrated hair loss, soft palate aphthae, tonsillar injection, and cervical lymphadenopathy. Neurological examination revealed generalized weakness; the patient was barely able to move all of his limbs. All of the muscles in his extremities were swollen and hard, and he felt pain when they were grasped. Deep tendon reflexes were diffusely hypoactive.(ABSTRACT TRUNCATED AT 250 WORDS)