A Japanese-American female with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

Abstract

Patients with clinically amyopathic dermatomyositis (CADM) have a risk of developing rapidly progressive interstitial lung disease (ILD). CADM-ILD is associated with the anti-MDA-5 antibody. In the USA, however, patients with CADM have these antibodies less frequently than those in Japan. In addition, those with this disorder are less often complicated with rapidly progressive ILD than those in Japan. We present a case of a 42-year-old Japanese-American female with a 3-month history of a rash on her hands and face with joint pain. Based on the negative results from lupus tests, her primary care provider and a rheumatologist treated her with steroids, hydroxychloroquine, and methotrexate. During treatment, the patient started noticing shortness of breath because of pneumonia, which was revealed by a CT scan. The woman was finally diagnosed with acute respiratory failure due to CADM with ILD. She underwent a double lung transplant as well as treatment with multiple immunosuppressive agents and repeated plasma exchange but died 15 days after transplantation. Her clinical course is similar to that of Japanese patients with CADM-ILD. Outside Japan, primary care providers, rheumatologists, and dermatologists, as well as pulmonary physicians, may be less familiar with this disorder than those in Japan. Since CADM-ILD progresses very quickly and could be fatal, these doctors should be aware of this disease to treat such patients as soon as possible, particularly when seeing a patient of Japanese descent.

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Acknowledgments

The authors thank the patient’s sister for providing the medical records, radiographs, and lung specimens.

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Correspondence to Toshinori Takada.

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Takada, T., Asakawa, K. & Barrios, R. A Japanese-American female with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis. Clin Rheumatol 40, 1159–1165 (2021). https://doi.org/10.1007/s10067-020-05292-0

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Keywords

  • Anti-MDA-5 antibody
  • Clinically amyopathic dermatomyositis
  • Interstitial lung disease
  • Lung transplant
  • Plasma exchange