Clinically amyopathic dermatomyositis with rapidly progressive interstitial pneumonia: The relation between the...

A 67-year-old woman exhibited chilblain-like erythema on the bilateral auricles, erythema with hyperkeratosis on the sides of fingers and the bilateral elbows, and erythema on the extensor/flexor sides of the finger joints and the iliac regions. Fine crackles were audible on the dorsal side of the bilateral lower lung regions. We diagnosed clinically amyopathic dermatomyositis accompanied by rapidly progressive interstitial pneumonia. While we initiated immunosuppressive therapy, the respiratory failure showed no responses. We performed endotoxin adsorption therapy using polymyxin B-immobilized fiber column direct hemoperfusion (PMX-DHP). After the first PMX-DHP session, the respiratory condition was slightly improved but it soon deteriorated. At the second PMX-DHP session, subcutaneous and mediastinal emphysema was noted. The patient died, showing no improvement in the respiratory condition. We measured the levels of serum interleukin (IL)-6, tumor necrosis factor (TNF)-α and IL-10 before and after PMX-DHP and compared them with previously reported levels. The levels of TNF-α and IL-10 showed no or slight changes, but those of IL-6 at the beginning of the treatment were higher compared with previous reports. After the first PMX-DHP, the IL-6 level decreased with slight improvement in respiratory condition. However, after the second PMX-DHP, it increased markedly with exacerbation of the respiratory failure. The serum IL-6 level is considered to be effective for the evaluation of the disease activity and prognosis under the treatment by PMX-DHP.