Clinical Commander

All dossiers
renal.fsgs.v1

Focal Segmental Glomerulosclerosis

nephrologychronicacuteadultoutpatientinpatient
Start encounter →Print handoutPRODUCTION

Promoted to INTEGRATED 2026-05-22 (shard-4 build campaign): added registry-resolving workups (hematuria, le_edema, rpgn); every evidence.pmid re-sourced and live-verified via PubMed; every regimen RxCUI reverse-looked-up live at RxNav. FSGS phenotypes: primary (permeability factor), secondary (adaptive/maladaptive), genetic (APOL1, podocyte genes), virus/drug-associated. Ladder: optimized supportive RAAS + SGLT2i; primary FSGS steroid + CNI; sparsentan (DUPLEX) emerging; secondary FSGS cause-directed. PMID anchors (live-verified PubMed 2026-05-22): 34556256 KDIGO 2021 Glomerular Diseases, 37921461 DUPLEX, 38490803 KDIGO 2024 CKD. Prior (cited as KDIGO 2021 GN) was a mis-attributed paper, corrected to 34556256; other fabricated PMIDs removed. RxCUI corrections (reverse-verified at RxNav): tacrolimus 42316, sparsentan 2630535 (prior 10318 resolved to tacrine; 2638750 was an invalid concept). Sibling routing: renal.minimal-change.v1, renal.membranous-nephropathy.v1, renal.rpgn.core.v1.

Entry points (5)

  • lab_abnormality
    Nephrotic-range or sub-nephrotic proteinuria — UPCR >3.5 g/g (primary) or 1-3.5 g/g (secondary) (KDIGO 2021 GN)
    nephrotic_or_subnephrotic_proteinuria
  • lab_abnormality
    CKD with FSGS lesion on biopsy (KDIGO 2021 GN)
    ckd_with_fsgs_biopsy
  • imaging
    Renal biopsy with focal segmental glomerulosclerosis + Columbia variant classification (KDIGO 2021 GN; D'Agati 2004)
    biopsy_fsgs_columbia_variant
  • history
    African ancestry with proteinuric CKD — APOL1 high-risk variant screening (KDIGO 2021 GN)
    apol1_high_risk_african_ancestry
  • problem_list
    FSGS on problem list (continuation visit)
    fsgs_known

Required inputs (12)

  • agerequired
    demographic • used at CONTEXT
    Age at presentation informs primary vs secondary likelihood + genetic panel decision (KDIGO 2021 GN)
  • race_ethnicityrequired
    demographic • used at CONTEXT
    African ancestry → APOL1 high-risk variant relevance for transplant + progression risk (KDIGO 2021 GN)
  • creatininerequired
    lab • used at CONTEXT
    eGFR + Δ-eGFR slope drives risk stratification + transplant timing (KDIGO 2021 GN)
  • urinalysisrequired
    lab • used at INITIAL_WORKUP
    Proteinuria + sediment (bland in pure FSGS) (KDIGO 2021 GN)
  • upcrrequired
    lab • used at INITIAL_WORKUP
    Proteinuria quantification — primary often >3.5 g/g, secondary <3.5 g/g (KDIGO 2021 GN)
  • serum_albuminrequired
    lab • used at INITIAL_WORKUP
    Severe hypoalbuminemia (<2.5 g/dL) suggests primary; preserved suggests secondary (KDIGO 2021 GN)
  • hiv_serologyrequired
    lab • used at INITIAL_WORKUP
    HIV-associated nephropathy (HIVAN) typically collapsing FSGS (KDIGO 2021 GN)
  • hcv_serology
    lab • used at INITIAL_WORKUP
    HCV-associated FSGS — DAA therapy can improve renal (KDIGO 2021 GN)
  • drug_exposure_heroin_lithiumrequired
    history • used at CONTEXT
    Drug-induced FSGS — heroin, lithium, anabolic steroids, IFN, pamidronate — withdrawal (KDIGO 2021 GN)
  • family_fsgs_or_ckd
    history • used at CONTEXT
    Genetic FSGS — NPHS1/NPHS2/INF2/TRPC6 — minimal immunosuppression response (KDIGO 2021 GN)
  • obesity_or_reduced_nephron_massrequired
    history • used at CONTEXT
    Adaptive secondary FSGS — obesity, reflux, unilateral kidney, sickle cell, oligomeganephronia (KDIGO 2021 GN)
  • sbprequired
    vital • used at CONTEXT
    BP target <130/80; ACEi/ARB renoprotection universal (KDIGO 2021 GN)

12-phase flow (12)

  1. 1FRAME
    Confirm FSGS lesion on biopsy + Columbia variant classification + assign primary vs secondary phenotype FIRST (KDIGO 2021 GN; D'Agati 2004)
    inputs: upcr, serum_albumin, creatinine
    advance: FSGS biopsy + phenotype assigned (KDIGO 2021 GN)
  2. 2ENTRY
    Nephrotic or sub-nephrotic proteinuria / CKD with biopsy-confirmed FSGS / APOL1 risk evaluation in African ancestry (KDIGO 2021 GN)
    inputs: upcr, creatinine
    advance: FSGS presentation phenotype identified (KDIGO 2021 GN)
  3. 3CONTEXT
    Capture age, race/ethnicity (APOL1), HIV/HCV, heroin/lithium/anabolic exposure, family history, obesity, reflux, BP (KDIGO 2021 GN)
    inputs: age, race_ethnicity, hiv_serology, drug_exposure_heroin_lithium, obesity_or_reduced_nephron_mass, sbp
    advance: Context captured (KDIGO 2021 GN)
  4. 4RED_FLAGS
    Collapsing FSGS variant / rapidly progressive eGFR decline / pulmonary-renal overlap / severe nephrotic complications (KDIGO 2021 GN)
    advance: No emergent phenotype OR routed appropriately (KDIGO 2021 GN)
  5. 5INITIAL_WORKUP
    UA + UPCR + albumin + lipids + BMP + HIV/HCV/HBV serology + parvovirus B19 if collapsing + complement (exclude lupus) + ANA + age-appropriate cancer screen (KDIGO 2021 GN)
    inputs: urinalysis, upcr, serum_albumin, creatinine, hiv_serology
    actions: panel.renal, panel.cbc, panel.lft, panel.lipid
    advance: Initial labs complete + viral causes screened (KDIGO 2021 GN)
  6. 6BRANCHING_WORKUP
    Renal biopsy with LM + IF + EM + Columbia variant classification (collapsing, tip, cellular, perihilar, NOS); APOL1 genotype if African ancestry; genetic panel if young onset / familial (KDIGO 2021 GN)
    advance: Biopsy + variant + APOL1/genetic results documented (KDIGO 2021 GN)
  7. 7DIFFERENTIAL
    Primary / secondary adaptive / APOL1-associated / genetic / HIVAN-collapsing / HCV / drug-induced / tip / cellular / perihilar / NOS (KDIGO 2021 GN)
    advance: Differential resolved + phenotype confirmed (KDIGO 2021 GN)
  8. 8RISK_STRATIFICATION
    Phenotype (primary vs secondary) + Columbia variant + proteinuria + eGFR slope + APOL1 risk + viral status (KDIGO 2021 GN)
    inputs: creatinine, upcr, race_ethnicity
    actions: calc.ckd_epi_2021
    advance: Risk tier + treatment plan set (KDIGO 2021 GN)
  9. 9TREATMENT
    Step 1 — phenotype identification (mandatory); Step 2 — primary FSGS prednisone 1 mg/kg/d × 16 wk minimum; Step 3 — primary CNI second-line + rituximab off-label refractory; Step 4 — sparsentan emerging (DUPLEX 2023 off-label); Step 5 — secondary RAS + weight loss + SGLT2i + cART for HIVAN + drug withdrawal (KDIGO 2021 GN)
    inputs: upcr, creatinine
    advance: Treatment tier executed (KDIGO 2021 GN)
  10. 10DISPOSITION
    Outpatient primary; admit for collapsing FSGS / rapid eGFR decline / severe nephrotic complications / HIVAN initiation (KDIGO 2021 GN)
    advance: Disposition set (KDIGO 2021 GN)
  11. 11MONITORING
    UPCR + eGFR + BP + albumin q3 months on therapy; CNI trough; rituximab CD20 / response (KDIGO 2021 GN)
    inputs: creatinine, upcr, serum_albumin
    actions: panel.renal
    advance: Monitoring schedule documented (KDIGO 2021 GN)
  12. 12FOLLOWUP
    q3-6 month nephrology; transplant prep if eGFR <20-30; post-transplant FSGS recurrence counseling (~30% primary); CV/bone/fertility (KDIGO 2024 CKD)
    advance: Long-term plan documented (KDIGO 2024 CKD)