Patient handout

Focal Segmental Glomerulosclerosis

PRODUCTION

1. Your condition

This handout is for focal segmental glomerulosclerosis. Your care team identified this based on: nephrotic-range or sub-nephrotic proteinuria — upcr >3.5 g/g (primary) or 1-3.5 g/g (secondary) (kdigo 2021 gn).

Other reasons your team may use this plan: ckd with fsgs lesion on biopsy (kdigo 2021 gn); renal biopsy with focal segmental glomerulosclerosis + columbia variant classification (kdigo 2021 gn; d'agati 2004); african ancestry with proteinuric ckd — apol1 high-risk variant screening (kdigo 2021 gn).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

Medication	Starting dose	How	When	What it does
phenotype_workup_bundle	—	—	—	KDIGO 2021 GN — primary vs secondary identification dictates therapy; immunosuppression for secondary may be harmful

Plan: FSGS phenotype-driven ladder — phenotype ID → primary immunosuppression → CNI/rituximab/sparsentan → secondary RAS/SGLT2i/weight-loss/cART/drug-withdrawal (KDIGO 2021 GN; DUPLEX 2023)

3. When to call your provider

Contact your care team if any of the following happen:

Rising UPCR + falling eGFR despite ladder → re-biopsy + specialty referral (KDIGO 2021 GN)
Rapid eGFR decline + collapsing variant → admit + RPGN evaluation (KDIGO 2021 GN)
New fever + cytopenia on immunosuppression → ED (KDIGO 2021 GN)
CKD progression toward ESRD → transplant evaluation; route neph.ckd.core.v1 (KDIGO 2024 CKD)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

Primary idiopathic FSGS — sudden onset nephrotic syndrome + severe hypoalbuminemia + extensive foot-process effacement; suPAR/circulating factor mediated; steroid-responsive (~50%) (KDIGO 2021 GN)
APOL1-associated FSGS — homozygous or compound heterozygous high-risk variants in African ancestry; 6-10× FSGS risk + accelerated progression + transplant donor screening implications (KDIGO 2021 GN)
Genetic FSGS — NPHS1 (nephrin) / NPHS2 (podocin) / INF2 / TRPC6 / WT1 / ACTN4; young onset / familial / drug-resistant phenotype; minimal immunosuppression response (KDIGO 2021 GN)
Virus-associated FSGS — HIVAN (HIV-associated nephropathy, typically collapsing variant), HCV, parvovirus B19; cART for HIVAN often improves renal alone (KDIGO 2021 GN)
Drug-induced FSGS — heroin, lithium, anabolic steroids, IFN, pamidronate — withdraw offending agent; may resolve without immunosuppression (KDIGO 2021 GN)
Collapsing FSGS variant — global glomerular tuft collapse + podocyte hyperplasia; associated with HIV, parvovirus, SARS-CoV-2, APOL1 high-risk, IFN, pamidronate; worst prognosis (KDIGO 2021 GN; D'Agati 2004)(life-threatening)
Refractory primary FSGS — failure of steroid + CNI; consider rituximab off-label / sparsentan emerging / specialty referral (KDIGO 2021 GN; DUPLEX 2023)
Severe nephrotic complication in FSGS — AKI during nephrotic flare, VTE / renal vein thrombosis, SBP (KDIGO 2021 GN)

5. Follow-up

q3-6 month nephrology; transplant prep if eGFR <20-30; post-transplant FSGS recurrence counseling (~30% primary); CV/bone/fertility (KDIGO 2024 CKD)

6. Sources

Guideline: KDIGO 2021 Glomerular Diseases + DUPLEX sparsentan in FSGS (NEJM 2023) + KDIGO 2024 CKD