RESUMO
Abstract The prognosis for dermatomyositis (DM) with acute interstitial pneumonia (IP) is very poor. In the past 5 years, we have treated 10 DM patients with acute or subacute IP. Six cases were of acute-type IP, and 4 were of subacute-type IP. The treatment was a combination therapy of methylprednisolone (m-PSL) pulse therapy, cyclophosphamide (CPA) pulse therapy, oral cyclosporine A (CsA), and oral PSL. The outcome was 5 deaths and 5 survivals. All 5 cases of death had acute-type IP, four of which were complicated with pneumomediastinum, and these patients died within 40 days of IP onset. Furthermore, 4 of the 5 death cases were diagnosed with amyopathic DM, and one had hypomyopathic DM. The survivors comprised one case of acute-type IP with marked myositis, and 4 subacute cases. These results suggested that the prognosis for DM with IP might be dependent on the type of IP, the severity of the myositis, and the existence of pneumomediastinum. The rapid establishment of a more useful diagnostic technique and therapy for early-phase DM with acute IP is hoped for.
RESUMO
A 70-year-old woman was admitted to our hospital for the treatment of diffuse scleroderma and marked edema in the lower extremities. Renal biopsy revealed membranous change, interstitial nephritis, and intimal hyperplasia of the small arteries. The patient was diagnosed as having mixed connective tissue disease (MCTD) presenting with nephrotic syndrome (NS). She responded well to a combination treatment consisting of methylprednisolone (m-PSL) pulse therapy, oral PSL, and cyclosporine A (CsA). We speculated on the actual pathogenesis of NS in this case of MCTD.