Objective To investigate the clinical features of patients with Castleman's disease (CD) and systemic lupus erythematosus (SLE). Methods According to the diagnostic information between 1994 to 2014 extracted from the database of the Medical Record Department of Peking Union Medical College Hospital (PUMCH),patients with CD and SLE were included. A thorough literature review utilizing the key words of "Castleman's disease","systemic lupus erythematosus","SLE",and "lupus" was performed in PubMed during the same period. Cases with detailed clinical information were included while cases without detailed information were excluded from the analysis of this study. Results Nine patients worldwide were available for analysis [2 cases from PUMCH,accounted for 0.03%(2/6502) of all patients diagnosed as SLE and 1.0% (2/100) of patients diagnosed as CD during the same period] with a male-to-female ratio of2:7. The median age at diagnosis of CD was 39.0 years (range:21- 60 years). All patients were diagnosed as multicentric CD with generalized peripheral lymphadenopathy. Pathologic examination showed a balanced distribution:plasma cell variant:hyaline-vascular variant:mixed variant=3:3:3. Fever was the most common symptom (88.9%,8/9). Blood system was the most commonly involved system (88.9%,8/9) and kidneys were the most commonly involved organ (88.9%,8/9). Autoimmune thrombocytopenia (AITP) was observed in 55.6% (5/9) of patients,which was significantly higher than the general SLE patients (15.0%) (P<0.01). None of the 9 patients had evidence of central nervous system involvement. Conclusions CD complicated by SLE is a rare clinical condition. Compared to the general SLE population,this subgroup of patients may have higher rate of AITP and lower rate of central nervous system involvement.