Interstitial lung diseases associated with amyopathic dermatomyositis

¹ Second Division, Dept of Internal Medicine
² Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Hamamatsu, 431-3192, Japan

^* To whom correspondence should be addressed. E-mail: suda{at}hama-med.ac.jp.

	Abstract

The aim of the present study was to clarify the clinical characteristics and prognosis of patients with interstitial lung disease associated with amyopathic dermatomyositis (ILD-ADM)

The study subjects consisted of 14 consecutive patients with ILD-ADM. They were classified into two categories, acute/subacute and chronic forms, according to the clinical presentation of ILD. The clinical features, responsiveness to therapy, and prognosis between the two forms were compared.

Nine ILD-ADM patients were categorized as the acute/subacute form, and five as the chronic form. PaO2 was significantly lower in the acute/subacute ILD than chronic ILD. On high-resolution computed tomography, ground-glass opacities were frequently found in the two forms, but consolidation was more common in acute/subacute ILD than chronic ILD. Bronchoalveolar lavage analysis showed higher numbers of total cells and lymphocytes in acute/subacute ILD than chronic ILD. Histologically, the most common finding was nonspecific interstitial pneumonia in the two forms, while diffuse alveolar damage was found only in acute/subacute ILD. Acute/subacute ILD was generally resistant to therapy, while chronic ILD responded well. Notably, the mortality of acute/subacute ILD was much higher than that of chronic ILD (67% vs. 0%, respectively).

In conclusion, ILD-ADM includes two different forms, acute/subacute and chronic forms, with distinct prognoses.

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