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

Clinical Rheumatology
Toshinori TakadaRoberto Barrios

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 phy...Continue Reading

References

Jul 14, 2006·The European Respiratory Journal·T SudaK Chida
Mar 10, 2010·Transplant International : Official Journal of the European Society for Organ Transplantation·Tsuyoshi ShojiHiroshi Date
May 3, 2011·Journal of the American Academy of Dermatology·David FiorentinoLivia Casciola-Rosen
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Nov 7, 2017·Arthritis & Rheumatology·Ingrid E LundbergUNKNOWN International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyosit
Nov 28, 2017·RMD Open·Matteo BottaiUNKNOWN International Myositis Classification Criteria Project consortium, the Euromyositis register and the Juvenile Dermatomyositi

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