A decade of Gottron’s papules, inverse Gottron’s papules and mechanic’s hand in anti-MDA5-associated interstitial lung disease with clinically amyopathic dermatomyositis

摘要

A 70-year-old man who was a former smoker presented toour hospital with fever and hemoptysis for 2 weeks. He hada rash in his bilateral upper eyelids and papules over hiselbows and fingers for 10 years (Fig. 1A) but attributed it tohis occupation, transport business. He had never experienceddyspnea before this admission. On admission, his bodytemperature was 37.4?C, and SpO2 was 92% in room air.On physical examination, fine crackles were heard in thewhole chest in addition to Gottron’s papules, inverse Gottron’spapules and mechanic’s hands (Fig. 1A–C). Chest computedtomography scan revealed subpleural superior ground-glassopacities in bilateral lower lobes, indicating interstitial lungdisease (Fig. 1D). Laboratory examination showed creatininekinase, 86 IU/L; lactate dehydrogenase, 400 IU/L; KL-6,887 U/mL; anti-melanoma differentiation-associated protein5 (anti-MDA5) antibody, positive (150 indexes); anti-Jo-1 antibody, negative; antinuclear antibody, negative; negativedsDNA; anti-Sm antibody, negative; and anti-Scl-70antibody, negative. Consequently, he was diagnosed withanti-MDA5-associated interstitial lung disease along withclinically amyopathic dermatomyositis (CADM). Despite optimalimmunosuppressive and immunomodulatory therapy, his respiratorycondition worsened gradually, and he died 2 months afterthe diagnosis.