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MODERN RHEUMATOLOGY Vol.13 No.4

>MR13-4

Clinical study of 10 cases of acute or subacute interstitial pneumonia associated with dermatomyositis
Hiro Kuroda1 , Hiroshi Morinaga1, Chikage Satoh1, Akira Miyata1 and Katsutoshi Sunami2
(1) Department of Internal Medicine, Chugoku Central Hospital of the Mutual Aid Association of Public School Teachers, 6-3-1 Nishifukatsu-cho, Fukuyama, 721-8581, Japan
(2) Department of Orthopedic Surgery, Chugoku Central Hospital of the Mutual Aid Association of Public School Teachers, Fukuyama, Japan
 
Received: 25 June 2002 Accepted: 28 March 2003
 
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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.
 
Key words
Acute interstitial pneumonia (AIP) - Combination therapy - Dermatomyositis (DM) - Pneumomediastinum
 
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