Morita Keishi, Shibano Takayuki, Maekawa Kohei, Hattori Masuji, Hida Nobuyuki, Nakamura Shiro, Takeshima Yasuhiro
Department of Pediatrics, Hyogo College of Medicine, Nishinomiya, Japan.
Division of Internal Medicine, Department of Inflammatory Bowel Disease, Hyogo College of Medicine, Nishinomiya, Japan.
CEN Case Rep. 2019 Feb;8(1):55-60. doi: 10.1007/s13730-018-0364-8. Epub 2018 Sep 24.
Rituximab (RTX) is increasingly used for the treatment of refractory nephrotic syndrome due to its inhibitory effect on B cells which extends the period of remission, while lowering the dose of steroids needed for disease management. However, RTX can lead to various side effects, including Crohn's disease. Herein, we describe a case of a 15-year-old boy with refractory nephrotic syndrome diagnosed at age 9 years who developed Crohn's disease following RTX treatment. RTX was initiated in this patient at the age of 13 years 6 months due to occurrence of 12 relapses of nephrotic syndrome over a 4-year period, despite treatment using cyclosporine, steroid pulse therapy, and mycophenolate mofetil. The patient received 4 doses of RTX over a 2-year period (dose, 375 mg/m). Although the treatment was effective in extending the disease-free duration up to 6 months, at the age of 15 years 9 months, the patient developed abdominal pain, associated with frequent watery stools and rapid weight loss. Based on clinical and endoscopic findings, he was diagnosed with Crohn's disease and treated using infliximab. Remission of Crohn's disease was achieved with this treatment, with no further relapse of nephrotic syndrome. Infliximab is thought to extend the remission period of nephrotic syndrome. In this case, we propose that Crohn's disease was caused by an abnormal immune tolerance, secondary to the use of RTX, although the exact underlying mechanism remains to be clarified. Therefore, inflammatory bowel disease should be considered if severe abdominal symptoms with weight loss following RTX administration are observed.
利妥昔单抗(RTX)因其对B细胞的抑制作用而越来越多地用于治疗难治性肾病综合征,这种抑制作用可延长缓解期,同时降低疾病管理所需的类固醇剂量。然而,RTX可导致各种副作用,包括克罗恩病。在此,我们描述了一例15岁男孩的病例,该男孩9岁时被诊断为难治性肾病综合征,在接受RTX治疗后患上了克罗恩病。该患者在13岁6个月时开始使用RTX,因为尽管使用了环孢素、类固醇冲击疗法和霉酚酸酯进行治疗,但在4年期间肾病综合征仍复发了12次。患者在2年期间接受了4剂RTX(剂量为375mg/m²)。尽管该治疗有效地将无病持续时间延长至6个月,但在15岁9个月时,患者出现腹痛,伴有频繁水样便和体重快速下降。根据临床和内镜检查结果,他被诊断为克罗恩病,并使用英夫利昔单抗进行治疗。通过该治疗实现了克罗恩病的缓解,肾病综合征未再复发。英夫利昔单抗被认为可延长肾病综合征的缓解期。在本病例中,我们认为克罗恩病是由使用RTX继发的异常免疫耐受引起的,尽管确切的潜在机制仍有待阐明。因此,如果在使用RTX后观察到伴有体重减轻的严重腹部症状,应考虑炎症性肠病。