Fig 2 from Fogo et al AJKD, © National Kidney Foundation.

Recurrent FSGS

• Incidence: 20-40%
• Risk factors: Childhood onset, rapid progression, recurrence of FSGS in prior transplant
• Clinical Features: Nephrotic syndrome within weeks after transplantation.  Also, can have hematuria, hypertension, and AKI
• Treatment: Plasmapheresis, CNIs, Cyclophosphamide, Rituximab, and in some cases galactose
• Prognosis: 20% of transplant recipients experience recurrence-related graft loss after 5-10 years

De Novo FSGS

• Incidence: 10-20%
• Risk factors: Long-standing chronic vascular damage, allografts with severe vascular disease, grafts with chronic transplant GN, chronic CNI toxicity, viral infections such as hepatitis B, C, parvovirus B19, EBV and CMV, mTOR inhibitors
• Clinical features: Similar to recurrent FSGS
• Treatment: Treat secondary cause; if idiopathic, steroids, cyclophosphamide, IVIg, plasmapheresis, rituximab, cyclosporine, and possibly galactose
• Prognosis: 40% at 5 years graft survival after diagnosis.

De Novo Collapsing Glomerulopathy           

• Incidence: 0.6% of kidney allografts
• Risk factors:  HIV infected individuals, Parvovirus B19, mTOR inhibitors, HTLV-1 virus, pamidronate use, and ischemic/adaptive form of podocytopathy leading to FSGS
• Clinical features: Rapidly progressive nephrotic syndrome
• Treatment: Similar to above forms of FSGS
• Prognosis: 50% allograft loss, and median graft survival in the largest series is 38 weeks from time of biopsy.

Dr. Kenar Jhaveri
AJKD Blog Editor

To view the related installments on FSGS (freely available), please visit the Atlas of Renal Pathology II at AJKD.org.