Lymphedema and retroperitoneal fibrosis / 中华风湿病学杂志

ABSTRACT

Objective:To improve the understanding of the relationship between lymphedema and retroperitoneal fibrosis (RPF).Methods:Four cases with lymphedema and RPF in Beijing Shijitan Hospital Capital Medical University were reported. The diagnosis and treatment were analyzed and discussed.Results:All four patients had lymphedema onset and imaging showed suspicious RPF. One case of non-Hodgkin's lymphoma was confirmed by lymph node biopsy. The malignant lesions were excluded in the other two cases by pathology or positron emission tomography (PET)/computerized tomography (CT). They were proved to be idiopathic retroperitoneal fibrosis after treatment with glucocorticoid combined immunosuppressive agents. Another case was systemic amyloidosis mimicking retroperitoneal fibrosis.Conclusion:Lymphatic involvement in RPF is relatively rare, and the possibility of RPF should be considered when patients develop lymphedema. Even if the initial diagnosis is RPF, we should be wary of tumors or other diseases. Imaging examination should be performed, and tissue biopsy should be used if necessary, so as to facilitate early diagnosis and treatment and improve the prognosis of patients.