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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