Wake Forest Health Sciences, Department of Internal Medicine, Section on Rheumatology and Immunology, Medical Center Blvd, Winston Salem, NC, 27157, USA.
Wake Forest Health Sciences, Department of Internal Medicine, Section on Infectious Disease, Medical Center Blvd, Winston Salem, NC, 27157, USA.
Curr Rheumatol Rep. 2017 Jun;19(6):35. doi: 10.1007/s11926-017-0664-6.
Immunosuppressive therapy for connective tissue diseases (CTDs) is steadily becoming more intense. The resultant impairment in cell-mediated immunity has been accompanied by an increasing risk for opportunistic infection (OI). Pneumocystis pneumonia (PCP) has been recognized as an OI in patients with CTDs, but specific risk factors and precise indications for PCP prophylaxis remain poorly defined. This review was undertaken to update information on the risk of PCP in patients with CTDs and to examine current guidelines for PCP prophylaxis in this population.
Data on the occurrence of PCP and indications for prophylaxis in patients with CTDs is sparse. Large systematic reviews did not incorporate patients with CTD secondary to the lack of randomized control trials. Upon reviewing guidelines published since 2015, prophylaxis for PCP is recommended only for patients with ANCA-positive vasculitis, specifically granulomatosis with polyangiitis (GPA), who are undergoing intense induction therapy. Evidence-based recommendations for the prophylaxis of PCP in patients with CTDs cannot be provided. There is expert consensus that PCP prophylaxis is warranted in patients with GPA undergoing induction therapy. Prophylaxis should perhaps also be considered for other CTD patients who are receiving similar intense immunosuppressive therapy especially if they are lymphopenic or have a low CD4 count.
结缔组织疾病（CTD）的免疫抑制治疗正逐渐变得更加强化。随之而来的细胞介导免疫损伤伴发机会性感染（OI）风险增加。肺孢子菌肺炎（PCP）已被认为是 CTD 患者的 OI，但 PCP 预防的具体危险因素和确切适应证仍未明确。本综述旨在更新 CTD 患者发生 PCP 的风险信息，并探讨该人群中 PCP 预防的现行指南。
关于 CTD 患者发生 PCP 和预防适应证的数据很少。由于缺乏随机对照试验，大型系统综述并未纳入 CTD 继发于抗中性粒细胞胞质抗体（ANCA）阳性血管炎的患者。在审查 2015 年以来发布的指南后，仅建议正在接受强化诱导治疗的 ANCA 阳性血管炎（尤其是肉芽肿性多血管炎）患者预防 PCP。无法为 CTD 患者提供基于证据的 PCP 预防建议。专家共识认为，正在接受诱导治疗的 GPA 患者需要 PCP 预防。对于接受类似强化免疫抑制治疗的其他 CTD 患者，或许也应考虑预防 PCP，特别是那些存在淋巴细胞减少或 CD4 计数较低的患者。
