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Chylothorax in dermatomyositis complicated with interstitial pneumonia

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Abstract

Chylothorax is a disease in which chyle leaks and accumulates in the thoracic cavity. Interstitial pneumonia and pneumomediastinum are common thoracic manifestations of dermatomyositis, but chylothorax complicated with dermatomyositis is not reported. We report a case of dermatomyositis with interstitial pneumonia complicated by chylothorax. A 77-year-old woman was diagnosed as dermatomyositis with Gottron’s papules, skin ulcers, anti-MDA5 antibody and rapid progressive interstitial pneumonia. Treatment with betamethasone, tacrolimus and intravenous high-dose cyclophosphamide was initiated, and her skin symptoms and interstitial pneumonia improved once. However, right-sided chylothorax began to accumulate and gradually increase, and at the same time, her interstitial pneumonia began to exacerbate, and skin ulcers began to reappear on her fingers and auricles. Although her chylothorax improved by fasting and parenteral nutrition, she died due to further exacerbations of dermatomyositis and interstitial pneumonia in spite of steroid pulse therapy, increase in the betamethasone dosage, additional intravenous high-dose cyclophosphamide and plasma pheresis. An autopsy showed no lesions such as malignant tumors in the thoracic cavity. This is the first report of chylothorax complicated by dermatomyositis with interstitial pneumonia.

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Authors and Affiliations

  1. Department of Rheumatology, Yodogawa Christian Hospital, 1-7-50 Kunijima, Higashiyodogawa-ku, Osaka, 533-0024, Japan

    Kentaro Isoda, Takao Kiboshi & Takeshi Shoda

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  1. Kentaro Isoda
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  2. Takao Kiboshi
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  3. Takeshi Shoda
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Correspondence to Kentaro Isoda.

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Isoda, K., Kiboshi, T. & Shoda, T. Chylothorax in dermatomyositis complicated with interstitial pneumonia. Rheumatol Int 37, 671–673 (2017). https://doi.org/10.1007/s00296-016-3613-1

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  • DOI: https://doi.org/10.1007/s00296-016-3613-1

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