Michel M, Habibi A, Godeau B, Bachir D, Lahary A, Galacteros F, Fifi-Mah A, Arfi S
Department of Internal Medicine, Henri-Mondor University Hospital, Créteil, France.
Semin Arthritis Rheum. 2008 Dec;38(3):228-40. doi: 10.1016/j.semarthrit.2007.10.003. Epub 2008 Jan 4.
To analyze the main characteristics of adults with sickle cell disease (SCD) and concurrent connective tissue disease (CTD).
A retrospective investigational study was performed. CTD was diagnosed according to standard international criteria. Severity of SCD was assessed by a clinical severity score.
Thirty patients, 23 women (76%) and 7 men, with hemoglobin S/S (n = 25) or S/C (n = 5) SCD were included. The subtypes of CTD were rheumatoid arthritis (RA) (n = 15), definite systemic lupus erythematosus or "incomplete lupus" requiring treatment (n = 13), primary Sjögren's syndrome with central nervous system involvement (n = 1), and systemic sclerosis (n = 1). Twenty-five of the 30 patients (83%) received steroid treatment, and 15 (50%) received at least 1 immunosuppressive agent (methotrexate in 14 cases) to control CTD. Four RA patients were given antitumor necrosis factor (TNF)alpha and 1 was treated with rituximab without SCD exacerbation. After a median follow-up of 4.5 years [range: 6 months to 30 years] from CTD diagnosis, 11 of the 25 (44%) patients receiving steroids had at least 1 episode of severe infection (mostly due to Staphylococcus aureus or Escherichia coli). SCD exacerbated in 13 of the 30 (43%) patients after CTD onset; 12 of these patients were receiving prednisone and/or methotrexate. Six patients (20%) had died from sepsis (n = 2), stroke (n = 2), or acute chest syndrome (n = 2).
CTD-related clinical manifestations and outcome were not particularly severe in patients with SCD. However, those with active CTD and undergoing steroid +/- methotrexate treatment had more serious SCD-related manifestations, a higher rate of severe infections, and an overall patient mortality rate of 20%. Thus, the management of patients with CTD and underlying SCD should consider the risk/benefit ratio of each treatment and steroid-sparing strategies should be implemented.
分析患有镰状细胞病（SCD）并并发结缔组织病（CTD）的成年人的主要特征。
进行了一项回顾性研究。CTD根据国际标准进行诊断。SCD的严重程度通过临床严重程度评分进行评估。
纳入了30例患者，其中23例女性（76%）和7例男性，患有血红蛋白S/S（n = 25）或S/C（n = 5）的SCD。CTD的亚型包括类风湿关节炎（RA）（n = 15）、需要治疗的明确系统性红斑狼疮或“不完全性狼疮”（n = 13）、累及中枢神经系统的原发性干燥综合征（n = 1）和系统性硬化症（n = 1）。30例患者中有25例（83%）接受了类固醇治疗，15例（50%）接受了至少一种免疫抑制剂（14例使用甲氨蝶呤）以控制CTD。4例RA患者接受了抗肿瘤坏死因子（TNF）α治疗，1例接受了利妥昔单抗治疗，且SCD未加重。自CTD诊断后中位随访4.5年[范围：6个月至30年]，25例接受类固醇治疗的患者中有11例（44%）至少发生过1次严重感染（主要由金黄色葡萄球菌或大肠杆菌引起）。30例患者中有13例（43%）在CTD发病后SCD加重；其中12例患者正在接受泼尼松和/或甲氨蝶呤治疗。6例患者（20%）死于败血症（n = 2）、中风（n = 2）或急性胸部综合征（n = 2）。
SCD患者中CTD相关的临床表现和结局并非特别严重。然而，患有活动性CTD且正在接受类固醇±甲氨蝶呤治疗的患者有更严重的SCD相关表现、更高的严重感染率，总体患者死亡率为20%。因此，对CTD合并潜在SCD患者的管理应考虑每种治疗的风险/获益比，并应实施类固醇节省策略。
