Unidade de Imunologia Clínica, Department of Medicine, Hospital de Santo António, Centro Hospitalar do Porto, Largo do Professor Abel Salazar, nº 2, 4099-001, Porto, Portugal.
Clin Rev Allergy Immunol. 2012 Dec;43(3):302-7. doi: 10.1007/s12016-012-8327-x.
Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection caused by the reactivation of JC virus and occurs in patients with severe primary or secondary immunosuppression. Recently, PML is becoming relevant in autoimmune disorders, particularly in patients treated with biologic agents. However, systemic lupus erythematosus (SLE) appears to be associated with susceptibility to PML that cannot be entirely explained by the immunosuppressive therapy. The authors present two patients with the diagnosis of SLE and PML: One had a heavy immunosuppressive therapy history, and the other had never experienced biologic or cytotoxic therapeutics. Both patients had a profound T-CD4+ lymphopenia during their clinical history. These two cases emphasize the importance of CD4+ lymphopenia in SLE patients with and without immunosuppressors regarding opportunistic infections.
进行性多灶性白质脑病（PML）是一种由 JC 病毒重新激活引起的罕见机会性感染，发生于严重原发性或继发性免疫抑制的患者中。最近，PML 在自身免疫性疾病中变得相关，特别是在接受生物制剂治疗的患者中。然而，系统性红斑狼疮（SLE）似乎与 PML 的易感性有关，这不能完全用免疫抑制治疗来解释。作者报告了两名 PML 合并 SLE 的患者：一名患者有重度免疫抑制治疗史，另一名患者从未接受过生物制剂或细胞毒性治疗。两名患者在其病史中均有严重的 T-CD4+淋巴细胞减少症。这两个病例强调了 CD4+淋巴细胞减少症在有和没有免疫抑制剂的 SLE 患者中对于机会性感染的重要性。
