Pavlakou Paraskevi, Papasotiriou Marios, Ntrinias Theodoros, Kourakli Alexandra, Bratsiakou Adamantia, Goumenos Dimitrios S, Papachristou Evangelos
Department of Nephrology and Renal Transplantation, University Hospital of Patras, Achaia, Greece.
Hematology Division, Department of Internal Medicine, University Hospital of Patras, Achaia, Greece.
Front Med (Lausanne). 2021 Mar 12;8:665475. doi: 10.3389/fmed.2021.665475. eCollection 2021.
Lupus nephritis in the context of Systemic Lupus Erythematosus (SLE) is characterized by an unpredicted course with remissions and flare-ups. Among others, it remains a significant cause of end-stage kidney disease (ESKD) in relatively young patients. Therapeutic regimens with newer immunosuppressive agents have been introduced in order to control SLE clinical manifestations more efficiently and limit organ damage induced by immune complex formation and sustained inflammation. Treatment is usually long-term, and the cumulative impact of immunosuppression is expressed through the increased frequency of infections and neoplasms. However, if the observed immunity dysregulation is secondary and pharmaceutically induced or there is a pre-existing, primary immunodeficiency that shares common pathogenetic pathways with SLE's autoimmunity is not always clear. Herein, we present the case of a 39-year-old woman, that reached ESKD due to lupus nephritis. After an upper respiratory cytomegalovirus (CMV) infection and concomitant CMV reactivations the investigation revealed significant immunodeficiency. Not long after the initiation of intravenous immunoglobulin (IVIG) administration, patient received a cadaveric kidney transplant. IVIG was continued along with standard immunosuppression so that both recurrent infections and allograft rejection are avoided. Patient is closely monitored, and her post-transplant course is remarkably satisfying so far. ESKD patients with immunodeficiency syndromes should not be excluded by definition from kidney transplantation.
系统性红斑狼疮（SLE）背景下的狼疮性肾炎具有缓解和复发的不可预测病程。其中，它仍是相对年轻患者终末期肾病（ESKD）的重要原因。为了更有效地控制SLE临床表现并限制免疫复合物形成和持续炎症引起的器官损伤，已引入了使用新型免疫抑制剂的治疗方案。治疗通常是长期的，免疫抑制的累积影响通过感染和肿瘤发生频率增加表现出来。然而，观察到的免疫失调是继发性药物诱导的，还是存在与SLE自身免疫有共同致病途径的先前存在的原发性免疫缺陷，情况并不总是很清楚。在此，我们报告一例39岁因狼疮性肾炎发展为ESKD的女性病例。在一次上呼吸道巨细胞病毒（CMV）感染及随之而来的CMV再激活后，检查发现存在明显免疫缺陷。在开始静脉注射免疫球蛋白（IVIG）后不久，患者接受了尸体肾移植。IVIG与标准免疫抑制治疗同时继续使用，以避免反复感染和移植肾排斥反应。患者受到密切监测，到目前为止其移植后病程非常令人满意。患有免疫缺陷综合征的ESKD患者按定义不应被排除在肾移植之外。
