Safdar Osama Yousif, Basunbul Lama Islem, Alhazmi Lenah Sulaiman, Almughamisi Shahad Amro, Habib Laura Ahmed, Basaeed Amani Jamaan, Kalaktawi Nada M, Alharithi Elaf Turki, Aljaaly Hataan A, Alzahrani Walaa A
Pediatric Nephrology Department, King Abdulaziz University Hospital, Jeddah, Saudi Arabia.
Pediatric Department, King Abdulaziz University Hospital, Jeddah, Saudi Arabia.
Int Med Case Rep J. 2022 Oct 18;15:587-592. doi: 10.2147/IMCRJ.S347389. eCollection 2022.
Steroid-dependent nephrotic syndrome (SDNS) is a common type of childhood nephrotic syndrome. Remission following steroid therapy is achieved in 80-90% of the patients, while the remainder show steroid-resistant nephrotic syndrome (SRNS). Rituximab is an anti-CD20 chimeric monoclonal antibody with proven therapeutic effects in several diseases and has been used with great success in the treatment of NS since its discovery. We report a case of a 4-year-old girl diagnosed with SDNS at the age of 3. As treatment with steroids, enalapril, and mycophenolate failed to produce complete remission, rituximab was initiated, and remission was successfully achieved after administration of the first dose. Due to this response, rituximab therapy was continued; however, a day after being admitted to the nephrology ward for the second dose, she started to develop a high fever, which reached up to 40°C. In addition, she also displayed symptoms of upper respiratory tract infection and an ulcerated wound on her left cheek. The patient became drowsy with reactive pupils, cold peripheries, and weak peripheral pulses. The capillary refill time was prolonged to 3-4 seconds and it was decided to withhold the second dose of rituximab. The patient was shifted to the PICU as a case of septic shock secondary to facial cellulitis and started on inotropes (epinephrine and norepinephrine), meropenem, vancomycin, and hydrocortisone 15 mg. Thereafter, surgical debridement of the wound was carried out. The patient remained in remission with regard to nephrotic syndrome and was discharged in a healthy condition. In conclusion, rituximab used in conjunction with steroids and other immunosuppressants may increase the risk of serious infections like necrotizing fasciitis (NF). Further studies are needed to explore the relationship between rituximab and NF.
激素依赖型肾病综合征(SDNS)是儿童肾病综合征的一种常见类型。80% - 90%的患者在接受激素治疗后可实现缓解,而其余患者则表现为激素抵抗型肾病综合征(SRNS)。利妥昔单抗是一种抗CD20嵌合单克隆抗体,在多种疾病中已证实具有治疗效果,自发现以来在肾病综合征的治疗中取得了巨大成功。我们报告一例4岁女童,3岁时被诊断为SDNS。由于使用类固醇、依那普利和霉酚酸酯治疗未能实现完全缓解,遂开始使用利妥昔单抗,首剂给药后成功实现缓解。鉴于此反应,继续进行利妥昔单抗治疗;然而,在因第二剂入院肾病科病房一天后,她开始出现高热,体温高达40°C。此外,她还表现出上呼吸道感染症状以及左侧脸颊有一处溃疡伤口。患者变得嗜睡,瞳孔有反应,四肢冰冷,外周脉搏微弱。毛细血管再充盈时间延长至3 - 4秒,于是决定停用第二剂利妥昔单抗。患者因面部蜂窝织炎继发感染性休克被转至儿科重症监护病房(PICU),并开始使用血管活性药物(肾上腺素和去甲肾上腺素)、美罗培南、万古霉素以及15毫克氢化可的松治疗。此后,对伤口进行了外科清创。患者肾病综合征持续缓解,并健康出院。总之,利妥昔单抗与类固醇及其他免疫抑制剂联合使用可能会增加坏死性筋膜炎(NF)等严重感染的风险。需要进一步研究来探讨利妥昔单抗与NF之间的关系。