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Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the lack of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by interstitial pneumonia that shows a rapid progressive course associated with a poor prognosis. We report a 49-year-old patient who presented with nonspecific interstitial pneumonia (NSIP) associated with ADM. The patient failed to respond to prednisolone and immunosuppressive therapy and died. Although idiopathic NSIP is known to have a better prognosis, NSIP in ADM could be a fatal disease. Therefore, we should appropriately treat interstitial pneumonia in ADM even if it is NSIP.

Nonspecific Interstitial Pneumonia with Poor Prognosis Associated with Amyopathic Dermatomyositis