PubMed · 5714522
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D Colomb, D David. 1968-12-08. [Dermatomyositis].. https://pubmed.ncbi.nlm.nih.gov/5714522/
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Dermatomyositis is a systemic autoimmune disease which belongs to the group of idiopathic inflammatory myopathies. The disease is rare with an incidence of 0.1-1/100,000 and a prevalence of 1-6/100,000. Women are affected twice as often as men. In some patients the disease presents with dermatologic changes weeks to months before the myopathy arises. It was observed that in some patients the myositis develops much later and sometimes not at all. Therefore, the term of dermatomyositis sine myositis used in earlier literature has been changed to amyopathic dermatomyositis. The most important question is whether the patient needs systemic therapy with its possible side effects yet possibly preventing the appearance of myositis or only local therapy for the skin manifestations is necessary. The goal of this article is to summarize the latest findings in amyopathic dermatomyositis.
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We report findings in 70 patients with both diffuse interstitial lung disease and either polymyositis (PM) or dermatomyositis (DM). Initial presentations were most commonly either musculoskeletal (arthralgias, myalgias, and weakness) or pulmonary (cough, dyspnea, and fever) symptoms alone; in only 15 patients (21.4%) did both occur simultaneously. Pulmonary disease usually took the form of acute to subacute antibiotic-resistant community-acquired pneumonia. Chest radiographs and computed tomography most commonly demonstrated bilateral irregular linear opacities involving the lung bases; occasionally consolidation was present. Jo-1 antibody was present in 19 (38%) of 50 patients tested. Synchronous associated malignancy was present in 4 of 70 patients (5.7%). Surgical lung biopsies disclosed nonspecific interstitial pneumonia (NSIP) in 18 of 22 patients (81.8%), organizing diffuse alveolar damage (DAD) in 2, bronchiolitis obliterans organizing pneumonia (BOOP) in 1, and usual interstitial pneumonia (UIP) in 1. Treatment usually included prednisone in 40-60 mg/d dosages for initial control, followed by lower dose prednisone plus an immunosuppressive agent such as azathioprine or methotrexate for disease suppression. Survival was significantly better than that observed for historical control subjects with idiopathic UIP, and was more consistent with survival previously reported in idiopathic NSIP. There was no difference in survival between Jo-1 positive and Jo-1 negative groups.