Clinically Amyopathic Dermatomyositis with Interstitial Pneumonia That Was Successfully Treated with Plasma Exchange

Patients with clinically amyopathic dermatomyositis (CADM), a subset of dermatomyositis characterized by a lack of muscle involvement, frequently develop rapidly progressive and treatment-resistant interstitial lung disease. We report the case of a 49-year-old man who was diagnosed with CADM. He dev...

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Bibliographic Details
Published in:Internal Medicine 2018/07/01, Vol.57(13), pp.1935-1938
Main Authors: Yagishita, Mizuki, Kondo, Yuya, Terasaki, Toshihiko, Terasaki, Mayu, Shimizu, Masaru, Honda, Fumika, Oyama, Ayako, Takahashi, Hiroyuki, Yokosawa, Masahiro, Asashima, Hiromitsu, Hagiwara, Shinya, Tsuboi, Hiroto, Matsumoto, Isao, Sumida, Takayuki
Format: Article
Language:English
Subjects:
Case Report
clinically amyopathic dermatomyositis
Cyclophosphamide
Cyclosporins
Dermatomyositis
Dermatomyositis - complications
Dermatomyositis - therapy
Humans
Immunosuppressive agents
Interferon
Internal medicine
interstitial lung disease
Lung diseases
Lung Diseases, Interstitial - classification
Lung Diseases, Interstitial - therapy
Male
Medical treatment
Methylprednisolone
Middle Aged
Muscles
Plasma
plasma exchange
Plasma Exchange - methods
Pneumonia
Respiratory therapy
Steroids
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Description
Summary:Patients with clinically amyopathic dermatomyositis (CADM), a subset of dermatomyositis characterized by a lack of muscle involvement, frequently develop rapidly progressive and treatment-resistant interstitial lung disease. We report the case of a 49-year-old man who was diagnosed with CADM. He developed interstitial pneumonia, which did not respond to combination therapy with methylprednisolone pulse therapy, cyclophosphamide, and cyclosporine. We therefore attempted plasma exchange. After 7 courses of therapeutic plasma exchange, the interstitial pneumonia gradually improved. This case suggests that plasma exchange might be an effective therapeutic option for patients with progressive interstitial lung disease in steroid- and immunosuppressive therapy-refractive CADM.
ISSN:0918-2918
1349-7235
DOI:10.2169/internalmedicine.0297-17