Table 3.

Selected literatures of treatment regimen in fibrillary GN

TreatmentSample SizeRegimenOutcomes
Conservative treatment with RAAS blocking agents
 Case series (10)16ACEI/ARB2 CR, 2 PR, 8 PRD, 4 ESKD
 Case series (11)14ACEI/ARB1 CR, 1 PR, 2 PRD, 10 ESKD
Corticosteroid
 Case reports (61)3Prednisone 1 mg/kg per d3 CR
 Case report (70)1High dose steroidNR
 Case series (8)9NA9 NR
 Case series (10)8NA8 NR
 Case series (11)5NA5 NR
 Multi-institutional cohort (18)24NA16 (67%) ESKD at median follow-up of 28 mo
Cyclophosphamide
 Case series (11)3NA1 PR, 1 PRD, 1 ESKD
 Case report (71)2200 mg/d then 100 mg/d ×6 mo + steroid then azathioprine 50 mg/d2 PR
150 mg/d ×2 wk, then 800 mg/m2 pulse monthly ×6 mo, then azathioprine 50 mg/d
 Case report (72)1NAPR
 Case report (73)1100 mg/d ×1 yr + prednisone 40 mg/dPR
 Case report (25)11 mg/kg per day + prednisone 60 mg/d with tapering regimenImproved in creatinine but persistent proteinuria
 Multi-institutional cohort (18)9NA8 (89%) ESKD at median follow-up of 24 mo
Cyclosporine
 Case series (8)3NA1 PR, 2 NR
 Case series (10)2NA2 NR
 Case series (11)4NA4 NR
Mycophenolate mofetil
 Case report (74)1500 mg BID ×8 mo + prednisone 40 mg/dImprovement in serum Cr but persistent hematuria and proteinuria (patients died due to nonrenal cause 8 mo later)
 Case report (23)11 g BID + high dose steroidESKD
 Case series (10)1NAPR
Lenalidomide
 Case series (10)2NANR
Rapamycin
 Case series (10)1NANR
Bortezomib
 Case series (11)1NANR
Rituximab
 Case report (62)3RTX 375 mg/m2 ×4–8 doses or RTX 1 g IV ×2 dosesPR
 Case series (10)3NANR
 Case series (63)12RTX 1 g IV ×2 doses or 375 mg/m2 ×4 doses4 nonprogressors, 3 PRD, 5 ESKD
 Case series (11)7RTX 375 mg/m2 ×2–4 doses5 PR, 2 PRD
 Multi-institutional cohort (18)8NA1 ESKD at median follow-up time of 14 mo. Rituximab was significantly associated with lack of progression to ESKD (HR, 0.435)
 Pilot prospective trial (59)11RTX g IV ×2 doses and another 2 doses at 6 mo3 PR
  • RAAS, renin-angiotensin-aldosterone system; ACEI, angiotensin-converting enzyme inhibitor; ARB, angiotensin receptor blocker; CR, complete remission; PR, partial remission; PRD, persistent renal dysfunction; NR, no response; NA, not available; BID, twice per day; Cr, creatinine; RTX, rituximab; IV, intravenous; HR, hazard ratio.