12-phase flow (12)

1. 1FRAME
   SLE patient with renal involvement — organ-specific (renal-domain) flare engine. New-onset SLE without renal signs routes to rheum.sle.core.v1; non-renal flare to rheum.sle-flare.core.v1; pauci-immune GN to rheum.mpa.core.v1
   inputs: sle_diagnosis_confirmed
   advance: renal-domain LN scope confirmed
2. 2ENTRY
   Recognise renal trigger — proteinuria UPCR ≥0.5, active sediment (dysmorphic RBC/RBC casts), rising Cr, or rising dsDNA/low complement with renal signs
   inputs: sle_diagnosis_confirmed
   advance: renal flare trigger documented
3. 3CONTEXT
   Current ISD + HCQ adherence, prior biopsy class + induction history, RAAS blockade, BP control, infection screen, vaccination status, pregnancy/lactation, comorbidities
   inputs: current_immunosuppression, pregnancy_status
   advance: baseline ISD, adherence, pregnancy status captured
4. 4RED_FLAGS
   Rapidly progressive GN / AKI; nephrotic with thrombosis (membranous + APS); severe HTN; concurrent extrarenal life-threatening flare (NPSLE, diffuse alveolar hemorrhage, severe cytopenias); pregnancy with active LN — escalate + rule out infection mimic / drug toxicity
   inputs: creatinine, sbp, cbc_with_diff
   actions: calc.news2
   advance: red flags acted on; mimics screened
5. 5INITIAL_WORKUP
   Creatinine + eGFR, UA with micro (dysmorphic RBC/casts), UPCR/24h protein, C3/C4, anti-dsDNA, CBC + diff, albumin, LFT, lipids, AP panel; infection screen (cultures, CMV/HSV PCR if immunosuppressed); rule out drug-induced + TMA
   inputs: creatinine, upcr, urinalysis_with_micro, complement_c3_c4, anti_dsdna, serum_albumin, cbc_with_diff
   actions: workup.rpgn, panel.renal, panel.ua, panel.inflammation, panel.cbc
   advance: renal workup sent + infection / TMA screen complete
6. 6BRANCHING_WORKUP
   RENAL BIOPSY is central — ISN/RPS 2018 class I–VI with activity vs chronicity indices; AP panel + ADAMTS13/schistocytes if TMA; renal US to exclude obstruction/size; APS-nephropathy histology if AP-positive; distinguish active vs chronic/sclerotic lesions
   inputs: anti_phospholipid_panel
   actions: workup.rpgn, workup.hepatorenal_syndrome
   advance: renal biopsy obtained / class assigned, or contraindication documented
7. 7DIFFERENTIAL
   Class I/II minimal/mesangial; class III/IV (±V) proliferative; pure class V membranous; class VI sclerotic. Distinguish from infection mimicking flare, drug toxicity (CNI/NSAID), TMA / APS-nephropathy, pauci-immune ANCA GN, hypertensive nephrosclerosis, preeclampsia in pregnancy
   inputs: renal_biopsy_isn_rps_class
   advance: class assigned + mimics excluded / co-managed
8. 8RISK_STRATIFICATION
   ISN/RPS class + activity/chronicity indices drive intensity & prognosis: III/IV ± V = aggressive induction; pure V = nephrotic/thrombosis-focused; VI = supportive/ESKD planning. BILAG renal-domain activity (organ-specific SLE flare); high chronicity index → guarded renal prognosis
   inputs: renal_biopsy_isn_rps_class, creatinine, upcr
   actions: calc.bilag, calc.qsofa
   advance: class-driven severity + prognosis set
9. 9TREATMENT
   Background for ALL: hydroxychloroquine (renal + survival benefit) + RAAS blockade + BP/proteinuria target + CV/bone/infection prophylaxis + vaccination pre-ISD. Proliferative III/IV (±V) induction: glucocorticoid (pulse MP for severe then rapidly tapered low-dose oral — steroid-minimisation) + EITHER mycophenolate OR low-dose IV cyclophosphamide (Euro-Lupus), increasingly TRIPLE therapy adding belimumab (BLISS-LN) or a CNI (voclosporin AURORA / tacrolimus). Pure membranous V: MMF + GC ± CNI, anticoagulate if nephrotic / APS. Maintenance: MMF (preferred) or azathioprine + low-dose GC + HCQ for ≥3 yr. Refractory → rituximab / obinutuzumab, switch agents. Pregnancy: continue HCQ, MMF/CYC CONTRAINDICATED → azathioprine/tacrolimus + low-dose GC + aspirin, MFM co-management. Biopsy — NOT serology — decides therapy
   inputs: renal_biopsy_isn_rps_class, pregnancy_status, serum_albumin
   advance: class-specific induction + background + steroid-minimisation plan documented
10. 10DISPOSITION
    RPGN/AKI, nephrotic+thrombosis, severe extrarenal flare, pregnancy with active LN → admit (ICU if RPGN with hyperkalemia/uremia, DAH, NPSLE, CAPS). Stable proteinuric LN → expedited nephrology + rheumatology co-management outpatient
    inputs: creatinine
    advance: level of care + nephrology/rheumatology co-management set
11. 11MONITORING
    UPCR + creatinine + eGFR (q2 wk during induction, then q1–3 mo), anti-dsDNA / C3-C4 q1–3 mo, BP + proteinuria target, CNI trough levels (voclosporin/tacrolimus), CBC/LFT for ISD myelotoxicity, CYC nadir at 7–14 d, infection surveillance, HCQ retina screen, pregnancy renal + fetal surveillance
    inputs: upcr, creatinine, complement_c3_c4
    actions: panel.renal, panel.ua, panel.cbc
    advance: complete renal response by 6–12 mo (partial by 3–6 mo) or refractory pathway entered
12. 12FOLLOWUP
    Prolonged maintenance ≥3 yr (MMF/AZA + low-dose GC + HCQ); treat-to-target proteinuria <0.5–0.7 g/g; CV + infection risk modification; vaccinations; ESKD/transplant planning + post-transplant LN recurrence surveillance; pregnancy planning when stable ≥6 mo on compatible drugs
    advance: long-term maintenance + ESKD/pregnancy plan documented