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Dr mohammed kamal fsgs
Focal segmental glomerulosclerosis (FSGS) accounts for 20-40% of nephrotic syndrome cases in adults and children. It can be primary/idiopathic or secondary to various causes. Histologic variants include classic, collapsing, tip, perihilar, and cellular forms. Prognosis depends on factors like proteinuria level, kidney function, and fibrosis. While untreated FSGS often leads to kidney failure, treatment with steroids, immunosuppressants, or plasmapheresis can induce remission. Recurrence is a risk after kidney transplantation, so candidates are warned though transplantation is not precluded.
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Overview of FSGS by Dr. Mohammed Kamal, a nephrology specialist.
FSGS accounts for 20% of nephrotic syndrome in children and 40% in adults; incidence is 7/million.
FSGS is classified into primary (idiopathic) and secondary. Treatment approaches differ significantly.
Different histologic variants of FSGS including Classic, Collapsing, Cellular, and their diagnosis methods.
Different histologic variants of FSGS including Classic, Collapsing, Cellular, and their diagnosis methods.
Untreated primary FSGS often leads to ESRD. Prognostic factors include proteinuria and histologic findings.
Definitions for complete and partial response to therapy in FSGS, alongside metrics for remission.
Various treatment options including immunosuppression agents like steroids and their role in treating FSGS.
Plasmapheresis may have a limited but significant role in treating FSGS when combined with immunosuppression.
Transplantation considerations for FSGS patients, including recurrence risks and preventative measures.
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- 1. 1 Focal Segmental GlomeruloSclerosis Dr. MohammedKamal Nephrology Specialist New Mansoura General Hospital (international)
- 2. FSGS: Epidemiology Focal segmentalglomerulosclerosis accounts for approximately 20% of cases of the nephrotic syndrome in children and 40% of such cases in adults, with an estimated incidence of 7 per 1 million.
- 6. Classification Primary or idiopathicFSGS: Typically presents with the nephrotic syndrome. Secondary FSGS: Typically presents with non- nephrotic proteinuria and often renal insufficiency. Distinguishing between primary and secondary disease is particularly important because of the markedly different approach to therapy in the two disorders.
- 7. FSGS: Classification Permeability Factor(100% foot process effacement) Idiopathic Autoimmune (T-cell response) Toxins Genetic Abnormalities Infections (HIV, Parvo B-19) Obesity Heroin Nephropathy Familial Disease Drug Toxicity (pamidronate) Primary Secondary
- 11. Histologic variants Classic FSGS,also called FSGS NOS (Not Otherwise Specified), is the most common form. Collapsing variant Tip variant Perihilar variant Cellular variant Foot process effacement is relatively mild in FSGS NOS & perihilar variant.
- 12. Histologic Variants To makethe histologic Dx of FSGS the perihilar, cellular, tip and collapsing variants must be excluded. Light and Electron microscopy is necessary for accurate Dx. Light will show mesangial collapse and sclerosis. Electron will show diffuse fusion of the epithelial cell foot process.
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- 16. FSGS Tip Lesion Themost responsive to treatment.
- 17. FSGS collapsing variant Themost rapidly progressive form of FSGS. It does not typically respond to therapy. Ptns. require dialysis or a kidney transplant within one to two years despite treatment.
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- 19. Prognosis Untreated primary FSGSoften follows a progressive course to end-stage renal disease (ESRD). The rate of spontaneous complete remission among patients with nephrotic syndrome <10%. Spontaneous remission is more likely to occur among patients with normal kidney function and non-nephrotic proteinuria.
- 20. Prognosis Factors that appearto influence response to treatment and/or prognosis include: Degree of proteinuria. Renal dysfunction. Histologic findings. Interstitial fibrosis.
- 21. Prognostic Factors No NephroticSyndrome-85% 10 yr survival Nephrotic Syndrome- 60-90% 5 yr survival 30-55% 10 yr survival Massive Proteinuria- progress to ESRD w/in 5 yrs Interstitial Fibrosis- poor renal survival Collapsing Variant- worse prognosis (HIV) Glomerular Tip Lesion- More likely to respond to steroid therapy
- 22. Response to therapy Acomplete response is a reduction in proteinuria to <200 to 300 mg/day. A partial response in patients presenting with nephrotic range proteinuria is a reduction of ≥50 percent, and to less than 3.5 g/day. A relapse is return of proteinuria to ≥3.5 g/day in someone who had undergone a complete or partial remission.
- 23. Treatment • immunosuppression - Steroids -CNIۥۥs Cyclosporins / tacrolimus - MMF - Cyclophosphamide - Sirolimus - Rituximab - Abatacept • Thiazolidinedione • Pirfenidone • NSAIDS
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- 26. Plasmapheresis There may bea limited role for plasmapheresis in the treatment of primary FSGS, based in part upon studies in patients who have recurrent FSGS in the renal allograft. Removal of a circulating factor by plasmapheresis or a protein adsorption column can dramatically reduce proteinuria and, in some cases, induce complete remission.
- 27. Plasmapheresis Whether a patientwith primary FSGS might respond to plasmapheresis is thought to vary with the presence or absence, and the absolute level, of the circulating permeability factor. Plasmapheresis should be performed in conjunction with immunosuppression.
- 29. FSGS & kidneyTx summary: Recommendations; (A) Candidates with FSGS should be warned that there is a 20–40% risk of recurrence, and that 40–50% with recurrence lose their grafts. However, the risk of recurrent FSGS need not preclude transplantation. (B) A prior history of graft loss from recurrent FSGS should be considered at least a relative contraindication to living donor transplantation, due to the likelihood of recurrence (up to 80%). (C) Assays for a serum factor to predict recurrent FSGS have not been standardized or validated for clinical practice. (D) There are not yet sufficient data for or against the use of prophylactic plasma exchange or other measures to prevent recurrent FSGS.