TAFRO syndrome with renal biopsy successfully treated with steroids and cyclosporine: a case report.

BACKGROUND: 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. CASE PRESENTATION: 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. CONCLUSIONS: 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.

Inhibition of the Mammalian Target of Rapamycin May Augment the Increase in Soluble Klotho Levels in Renal Transplantation Recipients.

BACKGROUND/AIMS: α-Klotho is mainly expressed in the kidneys, and its soluble form can prevent vascular calcifications. Inhibition of the mammalian target of rapamycin (mTOR) upregulates Klotho. We assessed serial changes in the levels of soluble Klotho (sKlotho) in recipients before and after renal transplantation and investigated the effects of an mTOR inhibitor. METHODS: Serum sKlotho levels were measured in 36 recipients before and 1 year after transplantation and compared between those taking everolimus and those not taking everolimus. RESULTS: sKlotho levels were higher after transplantation than before transplantation (369.3 vs. 211.8 pg/mL). After transplantation, sKlotho levels were significantly higher in recipients taking everolimus than in those not taking everolimus (536.7 vs. 332.4 pg/mL). CONCLUSION: Our results suggest that mTOR inhibition may augment the increase in sKlotho levels in transplant recipients. Further studies are needed to examine whether mTOR inhibitors suppress the development of vascular complications via upregulation of Klotho expression in renal transplant recipients.

Serum levels of intact parathyroid hormone is a prognostic indicator of dialyzed patients: the Nishinomiya study.

Patients with chronic kidney disease on dialysis are at higher risk for cardiovascular disease (CVD), which is the greatest cause of mortality. The target range of serum intact parathyroid hormone (iPTH) for prognosis, 60 to 240 pg/mL, was recommended by the Japanese Society for Dialysis Therapy guidelines. To investigate the impact of this iPTH target on CVD, dialysis patients were enrolled. A total 287 participants were observed. At the start of the study, serum iPTH levels, routine laboratory parameters, and certain factors related to CVD were evaluated. A survival analysis (Kaplan-Meier curve) was used. After 10 years of follow-up, 19.2% of patients had CVD. The subjects were divided into three groups according to their iPTH level at baseline based on the target range of 60 to 240 pg/mL: Low, Middle, and High groups. CVD was more common in the High and Low groups compared to the Middle group. A lower risk of CVD was evident in the extended dialysis patients with a range of 60 to 240 pg/mL iPTH. Further studies are needed to evaluate the impact of the iPTH level on poor outcome.