A 46-year-old male demonstrated edematous fingers on both hands in November 2003, and
interstitial pneumonia was noted on chest X-ray during a medical check-up in December 2003. Since
muscular weakness and
fever developed thereafter, and
interstitial pneumonia was aggravated on chest X-ray and CT, the patient was admitted to our hospital on March 25, 2004. Heliotrope-like
erythema, and Gottron's sign were noted. Laboratory findings showed the following ; LDH 876 U/l ; CK 224 U/l ; CRP 5.68 mg/dl ; and KL-6 3270 U/ml.
Autoantibodies such as
anti-Jo-1 antibody were all negative. Chest X-ray and CT showed ground-glass opacity in the bilateral lower dorsal regions of the lung, and reduced volume of the inferior lobe. He was diagnosed as having
dermatomyositis (DM) associated with progressive
interstitial pneumonia. Although a combination of
steroid pulse
therapy and
Cyclosporin-A were administered, the pulmonary lesions became aggravated. Additional intravenous
Cyclophosphamide (IVCY) was initiated on the 6th hospital day, and
interstitial pneumonia was markedly improved. Cases of progressive
interstitial pneumonia associated with DM that are negative for
anti-Jo-1 antibody and show a low ratio of CK/LDH are resistant to various treatments. Our case suggested that combination
therapy with
steroid,
Cyclosporin-A, and IVCY is useful for the treatment of progressive
interstitial pneumonia with DM.