[Case report and discussion of a patient with polymyositis, fibrosing alveolitis and scleroderma-like involvement of the right hand].
It is important to know the diagnostic procedure and therapy of polymyositis (PM) inspite of its rare incidence. We describe a case with PM, interstitial lung disease, high titers of anti-Jo-1-antibodies and affection of the right hand, that could be followed over 11 years. The differentiation of PM from neurologic diseases and toxic myopathies is mentioned. The patient was successfully treated with cyclosporin over a period of 20 months, but he is now in remission while receiving steroids alone. We emphasize the importance of early treatment of PM with high-dose steroids and the addition of immunosuppressive drugs (azathioprin, methotrexate, cyclophosphamid) in cases with high disease activity or resistance to steroids. In the treatment of PM/DM immunoglobulins play an increasingly important role. The problems of long-term treatment with steroids are of special interest.
Wunderlin
B
B
Klinik Valens, Schweiz.
Knüsel
O
O
Kummer
H
H
D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D001706:Biopsy; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000008:administration & dosage / Q000009:adverse effects; D003937:Diagnosis, Differential; D004334:Drug Administration Schedule; D005500:Follow-Up Studies; D006229:Hand Dermatoses / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007116:Immunization, Passive; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects; D008168:Lung / Q000473:pathology; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology; D009460:Neurologic Examination; D017285:Polymyositis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D011658:Pulmonary Fibrosis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D016896:Treatment Outcome
D000974:Antibodies, Antinuclear; D007166:Immunosuppressive Agents; C046750:Jo-1 antibody; D016572:Cyclosporine
Fallbeschreibung und Diskussion eines Patienten mit Polymyositis, fibrosierender Alveolitis und sklerodermieähnlichem Befall der rechten Hand.