Division of Kidney and Dialysis, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya City, Hyogo, 663-8501, Japan.
Division of Allergology and Rheumatology, Hyogo College of Medicine, Mukogawa-cho, Nishinomiya City, Hyogo, 663-8501, Japan.
BMC Nephrol. 2022 Jul 23;23(1):262. doi: 10.1186/s12882-022-02886-5.
TAFRO syndrome is an acute or subacute systemic inflammatory disease with no apparent cause, presenting with fever, generalized edema, thrombocytopenia, renal damage, anemia, and organ enlargement. Interleukin-6, vascular endothelial growth factor, and other cytokines are thought to be the etiologic agents that increase vascular permeability and cause the resulting organ damage. Only few reports of renal biopsy performed in patients with TAFRO syndrome exist.
A 61-year-old woman, with a history of Sjogren's syndrome, was admitted to our hospital with anasarca and abdominal distension. Based on the clinical course and various laboratory findings, we diagnosed TAFRO syndrome. Renal biopsy revealed thrombotic microangiopathy, including endothelial cell swelling, subendothelial space expansion, and mesangiolysis. She was treated with oral prednisolone and cyclosporine, with consequent resolution of anasarca, pleural effusion, and ascites, and improvement in renal function and urinary findings. The patient's platelet count also normalized after 2 months of treatment.
Given that only few reports of improvement in the systemic symptoms of TAFRO syndrome using steroids and cyclosporine exist, our study investigating the relationship between the pathogenesis of TAFRO syndrome and renal disorders, as well as treatment methods, provides valuable insights.
TAFRO 综合征是一种无明显病因的急性或亚急性全身性炎症性疾病，表现为发热、全身水肿、血小板减少、肾功能损害、贫血和器官肿大。白细胞介素-6、血管内皮生长因子等细胞因子被认为是增加血管通透性并导致相应器官损伤的致病因子。目前仅有少数关于 TAFRO 综合征患者行肾活检的报道。
一名 61 岁女性，有干燥综合征病史，因全身水肿和腹胀入院。根据临床经过和各种实验室检查结果，我们诊断为 TAFRO 综合征。肾活检显示血栓性微血管病，包括内皮细胞肿胀、内皮下腔扩张和系膜溶解。患者接受了口服泼尼松龙和环孢素治疗，随后全身水肿、胸腔积液和腹水消退，肾功能和尿液检查结果改善。治疗 2 个月后，患者的血小板计数也恢复正常。
鉴于仅少数报道称使用类固醇和环孢素可改善 TAFRO 综合征的全身症状，我们的研究调查了 TAFRO 综合征的发病机制与肾脏疾病之间的关系以及治疗方法，提供了有价值的见解。
