小児リウマチ
Online ISSN : 2434-608X
Print ISSN : 2435-1105
TAFRO症候群様の臨床症状を呈し,心嚢および腹部 ドレナージを要した小児全身性エリテマトーデスの一例
浜田 佳奈中岸 保夫笠井 和子
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ジャーナル フリー

2019 年 10 巻 1 号 p. 3-10

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Edema when accompanied by systemic lupus erythematosus(SLE) is often caused by lupus nephritis. In addition, serositis is frequently associated with effusion in the pericardium and thoracic and peritoneal cavities. In the present case, unlike typical SLE, generalized edema was accompanied by severe effusion that required intensive care, including drainage of the pericardium and abdomen. The patient was an 8-year-old girl. She was diagnosed as having SLE at age 6 years and given steroid pulse and immunosuppressant therapies, but did not attain remission. During her clinical course, she was hospitalized twice for fever and respiratory failure. During the first hospitalization, she required noninvasive positive pressure ventilation for respiratory failure caused by pleural effusion and ascites. During the second hospitalization, in addition to respiratory management, she required pericardial and abdominal drainage to manage the effusion. Findings that were observed simultaneous to the anasarca included thrombocytopenia, fever, splenohepatomegaly, systematic lymph node swelling, and acute kidney failure, which led us to think of TAFRO(thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) syndrome. While SLE and other rheumatoid diseases are currently excluded from the criteria for defining TAFRO syndrome, they explain the clinical symptoms. The possibility of TAFRO syndrome may need to be considered even against the background of SLE.
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© 2019 一般社団法人 日本小児リウマチ学会
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