02624nas a2200469 4500000000100000008004100001653001000042653001100052653001100063653000900074653001600083653001400099653001500113653002400128653001600152653002800168653001500196653002400211653004000235653002900275653002200304653001400326653002400340653001300364653001500377100001600392700001800408700001200426700001200438700001100450700001600461700002200477700001900499700001800518700001500536700001700551245007400568300001000642490000700652520148100659022001402140 2016 d10aAdult10aFemale10aHumans10aMale10aMiddle Aged10aPrognosis10aAdolescent10aProspective Studies10aYoung Adult10aKidney Failure, Chronic10aCreatinine10aDisease Progression10aGlomerulosclerosis, Focal Segmental10aImmunosuppressive Agents10aKidney Glomerulus10aPodocytes10aRemission Induction10aSteroids10aTacrolimus1 aYadav Ashok1 aSakhuja Vinay1 aKohli H1 aGupta K1 aJha V.1 aKumar Vivek1 aRaja Ramachandran1 aNada Ritambhra1 aKumar Ashwani1 aGoyal Ajay1 aRathi Manish00aA prospective study of collapsing focal segmental glomerulosclerosis. a894-80 v383 a
Collapsing focal segmental glomerulosclerosis (cFSGS) is characterized by rapid progression to end-stage renal disease (ESRD). We evaluated the clinicopathological spectrum of cFSGS and compared its clinical behavior to steroid and tacrolimus (TAC)-resistant noncollapsing focal segmental glomerulosclerosis (FSGS). All patients (>14 years) diagnosed with cFSGS were enrolled in the study. Staining for differentiated podocyte markers such as WT 1, PAX and KI67 were performed in all patients. The outcome and histological features of cFSGS was compared with a prospectively followed cohort of steroid and TAC-resistant noncollapsing FSGS. The study included 22 cFSGS patients and 19 cases of steroid and TAC-resistant FSGS. Complete remission, partial remission, steroid resistance, progression to ESRD and death were observed in 13.6%, 4.5%, 27.3%, 36.4% and 18.2% patients, respectively. Patients with cFSGS had higher serum creatinine and more advanced tubulointerstitial changes compared to resistant FSGS. Twenty-six percent of therapy resistant noncollapsing FSGS progressed to ESRD after two years of stopping TAC. However, there was no difference in progression to ESRD between cFSGS and therapy-resistant noncollapsing FSGS at the end of two years. Glomerular collapse in the setting of FSGS is poorly responsive to treatment and has a high rate of progression to ESRD. The long-term prognosis of cFSGS and steroid and TAC-resistant FSGS are similar.
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