As a result, the steroid dose could be reduced earlier by the combination of steroid therapy and LDL-A. The remission rate was further increased in a follow-up study 2 years later, suggesting that the prognosis of even FSGS with refractory NS is favorable if remission can be achieved [8]. A survey concerning the long-term outcome was conducted primarily by the Japanese Society of Kidney and Lipids with the cooperation of 36 facilities, involving 94 patients with refractory nephrotic syndrome including 41 patients with FSGS and 28 patients with refractory minimal change nephrotic syndrome (MCNS) who underwent LDL-A in 1999 and thereafter [9]. The profiles of the FSGS

and MCNS patients were as follows: male/female ratio: 24/16 and 14/14; mean age: 43 ± 19.6 and 35.7 ± 18.7 years; initial/recurrence ratio: 20/15 and 12/14; number of LDL-A trials: 8.25 ± 2.87 and 8.00 ± 5.57; Lumacaftor ic50 and ratio between those who underwent kidney transplantation and those who did not: 7/24 and 0/25, respectively. In terms of the frequency of use of various drugs, steroids were used

in 88 and 93 %, steroid pulse therapy was performed in 29 and 57 % (the prescription was the same as that before the initiation of LDL-A, except in 1 patient with MCNS), immunosuppressants were used in 41 and 46 %, CyA was employed in 29 and 36 %, and statins were used in 44 and 36 %, respectively. The percentages of patients who were included in the category of type I ICR find more after 2 years were 62 and 95 %, and those after 5 years were 87 and 80 %, respectively. Those of FSGS are shown in Figure 2. The response became more favorable as the time from the onset of NS to the introduction of LDL-A decreased. Fig. 2 Retrospective survey of outcome

of FGS patients with refractory NS treated by LDL-apheresis. Two-year outcome of 29 FSGS patients (a) and 5-year outcome of 15 patients (b) are shown Since the above studies were retrospective, a prospective cohort study (Prospective Observational Survey on the Long-Term Effects of http://www.selleck.co.jp/products/BafilomycinA1.html LDL-A on Drug-Resistant Nephrotic Syndrome (POLARIS)) was initiated by the Japanese Society of Kidney and Lipids. In the preliminary analysis, almost the same remission rate was obtained, even in prospective study (under submission). As shown in Table 2, on the basis of reported results of retrospective studies, LDL-A has been effective for inducing remission in nearly 50 % of patients with various diseases including FSGS that was refractory to NS, with a high level of safety. As noted in recently renewed guidelines for NS in Japan (2013), LDL-A should be selected as an option for the strategy to treat refractory NS. Table 2 Clinical efficacy of LDL-apheresis for nephrotic syndrome (Summary of Clinical Studies before 2007)   Muso et al. Nephron 2001 89 408–415 Stenvinkel et al. Eur J Clin Invest 2000 30 866–870 Yokoyama et al. Clin Nephrol 1998 50 1–7 Muso et al. NDT 1994 9 2257-264 Sakai et al. Jin To Touseki 1994 33 321–328 Hattori et al.