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[Therapeutic efficacy of cyclosporin A in four cases of amyopathic dermatomyositis with rapidly progressive interstitial pneumonia].

OBJECTIVES
We investigated the clinical features of amyopathic dermatomyositis (ADM) with rapidly progressive interstitial pneumonia (IP), and the effectiveness of cyclosporin A (CsA) for IP.


METHODS
In our four patients who were accorded with the notion of amyopathic dermatomyositis proposed by Euwer and treated with CsA for IP, and eight reported patients for whom we could obtain details of the clinical course, we investigated the relationship between the clinical features of them at the start of CsA administration and the clinical effectiveness of CsA.


RESULTS
When we investigated the effectiveness of CsA in our four patients and eight reported patients, we could judge that effective cases were four and ineffective cases were eight. We compared the PaO2 at the start of CsA administration between effective and ineffective cases, the average PaO2 at the start of CsA administration was 65.7 mmHg in four effective cases, while it was 51.0 mmHg in eight ineffective cases. There was a statistically significant difference between the groups.


CONCLUSIONS
Because our results suggested that many ineffective cases were treated with CsA after they had developed severe respiratory failure, we think that CsA should be given to ADM patients with rapidly progressive IP before the progression of respiratory failure.