Ferreira De Matos C, De Almeida Chaves S, Potin P, Syrykh C, Piel-Julian M-L, Sailler L, Moulis G
Service de médecine interne, CHU de Toulouse, URM, CHU Purpan, place du Dr Baylac, TSA 40031, 31059 Toulouse cedex 9, France.
Service de médecine interne, CHU de Toulouse, URM, CHU Purpan, place du Dr Baylac, TSA 40031, 31059 Toulouse cedex 9, France.
Rev Med Interne. 2023 Feb;44(2):85-88. doi: 10.1016/j.revmed.2022.08.006. Epub 2022 Aug 30.
Castleman disease is a rare lymphoproliferation, which may mimic systemic lupus. Conversely, systemic lupus sometimes presents like an hematological malignancy. In these cases, a "Castleman-like" histology has been exceptionally described.
A 55-year-old female treated by methotrexate for systemic lupus with skin and joint involvement presented weight loss, polyadenopathy and clinical signs of lupus flare. Biology showed pancytopenia, complement activation, and positive anti-DNA antibodies. PET/CT showed hypermetabolic polyadenopathy. The lymph node biopsy showed "Castleman-like" features. Treatment with corticosteroids and azathioprine resulted in complete remission.
Systemic lupus and Castleman disease may share common clinical, biological, and histological features. The presence of specific elements of systemic lupus flare and the remission obtained by low-dose corticosteroids results in considering the diagnosis of Castleman-like systemic lupus and avoiding treatment intensification.
Castleman病是一种罕见的淋巴组织增生性疾病,可能类似系统性红斑狼疮。相反,系统性红斑狼疮有时表现得像血液系统恶性肿瘤。在这些病例中,曾有过关于“Castleman样”组织学的特殊描述。
一名55岁女性,因皮肤和关节受累的系统性红斑狼疮接受甲氨蝶呤治疗,出现体重减轻、多处淋巴结肿大以及狼疮病情复发的临床症状。实验室检查显示全血细胞减少、补体激活以及抗DNA抗体阳性。PET/CT显示多处淋巴结代谢活跃。淋巴结活检显示具有“Castleman样”特征。使用皮质类固醇和硫唑嘌呤治疗后实现完全缓解。
系统性红斑狼疮和Castleman病可能具有共同的临床、实验室检查及组织学特征。存在系统性红斑狼疮病情复发的特定因素,以及低剂量皮质类固醇治疗取得缓解,使得考虑诊断为Castleman样系统性红斑狼疮,并避免强化治疗。