rheum.sle.core.v1

Systemic lupus erythematosus (chronic, treat-to-target)

rheumatologychronicadultoutpatienttransition

Start encounter →Print handoutPRODUCTION

Acute flare workflow lives on rheum.sle-flare.core.v1 (scaffold) — keep dossiers in sync as flare manifest matures. Pregnancy phenotype shares this dossier; consider rheum.sle-pregnancy.core.v1 split when manifest authored. PRODUCTION blockers: RxCUI verification (npm run research:rxnav:validate); HCQ retinal-screen reminder rule + voclosporin BP/Cr triple-monitor cadence need decision-rule wiring; no dedicated test file.

Entry points (3)

symptom
Multi-system features (rash + joints + serositis + cytopenia) [2019 EULAR/ACR Aringer ARD 2019]
multi_system_features
lab_abnormality
ANA ≥1:80 with compatible clinical features [2019 EULAR/ACR entry criterion]
ana_positive_with_features
problem_list
Known SLE — routine treat-to-target visit [EULAR 2023 Fanouriakis]
sle_known_diagnosis_routine

Required inputs (14)

ana_titerrequired
lab • used at INITIAL_WORKUP
Entry criterion (2019 EULAR/ACR ≥1:80 by IF Hep-2)
anti_dsdnarequired
lab • used at INITIAL_WORKUP
High specificity; correlates with renal flare [2019 EULAR/ACR Aringer ARD 2019]
anti_smith
lab • used at INITIAL_WORKUP
High specificity; weighted in 2019 EULAR/ACR criteria [Aringer ARD 2019]
complement_c3_c4required
lab • used at MONITORING
Trend marker for active disease [EULAR 2023 Fanouriakis]
cbc_with_diffrequired
lab • used at INITIAL_WORKUP
Cytopenias common; classification + drug toxicity [2019 EULAR/ACR]
creatininerequired
lab • used at INITIAL_WORKUP
Lupus nephritis monitoring + drug dosing [KDIGO 2021 GN]
urinalysis_with_microrequired
lab • used at INITIAL_WORKUP
Active sediment screen [2024 ACR/EULAR LN]
upcrrequired
lab • used at MONITORING
Surveillance for nephritis; >0.5 prompts biopsy [2024 ACR/EULAR LN]
lipid_panel
lab • used at MONITORING
Accelerated atherosclerosis risk in SLE [EULAR 2023 Fanouriakis]
a1c
lab • used at MONITORING
Steroid-induced diabetes monitoring [EULAR 2023]
anti_phospholipid_panelrequired
lab • used at INITIAL_WORKUP
Thrombotic + obstetric risk; influences VTE prophylaxis + pregnancy plan [EULAR 2023 Fanouriakis]
eye_exam_baseline_HCQrequired
imaging • used at TREATMENT
AAO 2024 baseline retinal screen + annual after 5 years on HCQ [AAO 2024]
pregnancy_intentionrequired
demographic • used at CONTEXT
MMF / CYC / MTX teratogenic; switch to AZA / HCQ / tacrolimus before conception [EULAR 2023 Fanouriakis]
current_immunosuppressionrequired
medication • used at CONTEXT
Drives next-step escalation / de-escalation [EULAR 2023 treat-to-target]

12-phase flow (11)

1FRAME
Adult chronic SLE management — steady-state visits and treat-to-target [EULAR 2023 Fanouriakis]. Acute flare routes to rheum.sle-flare.core.v1
advance: scope confirmed
2ENTRY
Routine titration visit OR new SLE candidate meeting 2019 EULAR/ACR criteria (≥10 points + ANA entry) [Aringer ARD 2019]
inputs: ana_titer
advance: classification + visit purpose set
3CONTEXT
Phenotype map (skin / joint / renal / hematologic / NPSLE / serositis), comorbidities, pregnancy plan, smoking, sun exposure, vaccination status [EULAR 2023]
inputs: anti_phospholipid_panel, pregnancy_intention, current_immunosuppression
advance: phenotype + reproductive plan documented
4INITIAL_WORKUP
Full immunology panel (ANA, dsDNA, Smith, RNP, SSA/SSB, complements), CBC, CMP, UA + UPCR, AP panel, lipid + A1c baseline [2019 EULAR/ACR + EULAR 2023]
inputs: ana_titer, anti_dsdna, cbc_with_diff, creatinine, urinalysis_with_micro
actions: panel.cbc, panel.renal, panel.ua
advance: baseline panel complete
5BRANCHING_WORKUP
Renal biopsy if proteinuria >0.5 / active sediment / unexplained AKI [2024 ACR/EULAR LN]; echo if pulmonary HTN suspected; DEXA baseline; APS workup if thrombosis or pregnancy loss
actions: workup.aps, workup.rpgn
advance: organ-specific workup booked
6DIFFERENTIAL
Drug-induced lupus, mixed connective tissue disease, undifferentiated CTD, primary APS, RA-overlap, scleroderma overlap, viral mimics (parvo, EBV) [EULAR 2023]
advance: differential resolved
7RISK_STRATIFICATION
Organ-dominant phenotype + APS status + flare frequency + cumulative steroid burden + atherosclerotic risk [EULAR 2023 Fanouriakis]
inputs: anti_phospholipid_panel
advance: risk profile assigned
8TREATMENT
HCQ 5 mg/kg actual weight for ALL [EULAR 2023 strong]. Mild skin/joint: topical + low-dose pred; consider methotrexate. Moderate / refractory: belimumab [BLISS Navarra Lancet 2011] or anifrolumab [TULIP-2 NEJM 2020] add-on. Lupus nephritis class III/IV: induction with MMF or Euro-Lupus IV CYC ± voclosporin add-on [AURORA Rovin Lancet 2021]. Severe NPSLE / refractory cytopenias / vasculitis: pulse methylpred + CYC or rituximab. Maintenance: MMF or AZA [2024 ACR/EULAR LN]. Aspirin if APS-positive non-thrombotic; warfarin if thrombotic APS [TRAPS Lancet Haematol 2016]. Vaccinations + bone health + sun protection + smoking cessation. Treat-to-target: LLDAS / DORIS remission [EULAR 2023]
inputs: eye_exam_baseline_HCQ
advance: individualised regimen documented
9DISPOSITION
Outpatient rheumatology q3 months stable, q4-6 weeks during titration; obstetrics co-management when pregnant [EULAR 2023]
advance: continuity arranged
10MONITORING
CBC, CMP, UA, UPCR q3 months minimum; complement + dsDNA q3-6 months; HCQ retina screen baseline + annual after 5 y [AAO 2024]; lipid + A1c annually; DEXA q1-2 years on chronic steroids; cardiovascular risk reassessment [EULAR 2023]
inputs: cbc_with_diff, creatinine, urinalysis_with_micro, upcr, complement_c3_c4
actions: panel.renal, panel.ua, panel.cbc
advance: monitoring schedule documented
11FOLLOWUP
LLDAS / DORIS targeted; minimise glucocorticoids ≤7.5 mg/d after first year [EULAR 2023]; pregnancy planning when stable ≥6 months on compatible drugs; transition planning for adolescents
advance: long-term plan + counselling complete