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rheum.sle.core.v1PRODUCTION
rheum.sle.core.v1

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

rheumatologychronicadult
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11/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Adult chronic SLE management — steady-state visits and treat-to-target [EULAR 2023 Fanouriakis]. Acute flare routes to rheum.sle-flare.core.v1

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Patient inputs (14)

MMF / CYC / MTX teratogenic; switch to AZA / HCQ / tacrolimus before conception [EULAR 2023 Fanouriakis]

Drives next-step escalation / de-escalation [EULAR 2023 treat-to-target]

Entry criterion (2019 EULAR/ACR ≥1:80 by IF Hep-2)

High specificity; correlates with renal flare [2019 EULAR/ACR Aringer ARD 2019]

Cytopenias common; classification + drug toxicity [2019 EULAR/ACR]

Lupus nephritis monitoring + drug dosing [KDIGO 2021 GN]

Active sediment screen [2024 ACR/EULAR LN]

Thrombotic + obstetric risk; influences VTE prophylaxis + pregnancy plan [EULAR 2023 Fanouriakis]

Trend marker for active disease [EULAR 2023 Fanouriakis]

Surveillance for nephritis; >0.5 prompts biopsy [2024 ACR/EULAR LN]

AAO 2024 baseline retinal screen + annual after 5 years on HCQ [AAO 2024]

High specificity; weighted in 2019 EULAR/ACR criteria [Aringer ARD 2019]

Accelerated atherosclerosis risk in SLE [EULAR 2023 Fanouriakis]

Steroid-induced diabetes monitoring [EULAR 2023]

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (6)

6 need judgement
  • informationalseverenew_proteinuria_on_routine (ACR 2024)
    New UPCR >0.5 g/g or active urine sediment on routine surveillance [2024 ACR/EULAR LN biopsy threshold]
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereaps_thrombosis_new
    New venous or arterial thrombosis in SLE patient with APS [ACR 2024]
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_with_active_disease
    Pregnancy detected in SLE patient with active disease or on teratogen [EULAR 2023 Fanouriakis]
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereserious_infection_during_immunosuppression
    Hospitalised infection, opportunistic infection, or sepsis in SLE patient [EULAR 2023]
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatehcq_retinopathy_warning
    Bull-eye maculopathy or significant change on retinal imaging [EULAR 2023]
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatecumulative_steroid_burden
    Prednisone >7.5 mg/d for >12 months OR cushingoid features [EULAR 2023 strong recommendation]
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

Chronic disease-modifying therapy (treat-to-target) (ACR 2024)
axis: sle_disease_modifying_chronic
Selected axis "Chronic disease-modifying therapy (treat-to-target) (ACR 2024)" by default fallback (first axis)
  • hydroxychloroquine
    first line
    antimalarial
    triggers: any_sle_diagnosis
    2023 EULAR strong: 5 mg/kg actual weight for ALL SLE patients; reduces flares, mortality, thrombosis
    rxcui 5521
  • methotrexate
    add on
    anti_metabolite
    triggers: joint_skin_dominant, inadequate_HCQ
    Steroid-sparing for arthritis / cutaneous predominance [EULAR 2023 Fanouriakis]
    rxcui 6851
  • mycophenolate_mofetil
    first line
    anti_metabolite
    triggers: lupus_nephritis_III_IV_V, severe_extrarenal
    2024 ACR/EULAR LN preferred induction + maintenance
    rxcui 68149
  • azathioprine
    add on
    anti_metabolite
    triggers: pregnancy_compatible, maintenance, mmf_intolerant
    Maintenance + pregnancy-compatible [EULAR 2023; 2024 ACR/EULAR LN]
    rxcui 1256
  • belimumab
    add on
    anti_BAFF_mab
    triggers: moderate_severe_active_despite_standard
    BLISS-52/76 + BLISS-LN; 2023 EULAR strong add-on for active SLE / lupus nephritis
    rxcui 1092437
  • anifrolumab
    add on
    anti_IFNAR_mab
    triggers: moderate_severe_active, skin_joint_dominant
    TULIP-1/2 NEJM 2020 / Lancet RD 2019
    rxcui 2565265
  • voclosporin
    add on
    calcineurin_inhibitor
    triggers: lupus_nephritis_class_III_IV_V, add_on_to_MMF
    AURORA / AURORA-2 — improved renal response when added to MMF (ACR 2024)
    rxcui 2475166
  • cyclophosphamide
    rescue
    alkylator
    triggers: rapidly_progressive_GN, NPSLE_severe, alveolar_hemorrhage
    Euro-Lupus low-dose IV pulse for severe induction [Houssiau ARD 2002; EULAR 2023]
    rxcui 3002
  • rituximab
    rescue
    anti_CD20_mab
    triggers: refractory_cytopenia, refractory_renal, NOBILITY_obinutuzumab_alternative
    Off-label rescue for refractory cytopenia + renal disease [EULAR 2023 conditional]
    rxcui 121191

outpatient playbook — drug actions (10)

  1. 1. HCQ universal
    5 mg/kg actual weight (typically 200-400 mg/d) • PO • daily
    trigger: All SLE patients (ACR 2024)
    2023 EULAR strong — universal
  2. 2. methotrexate (joint/skin dominant)
    15-25 mg PO/SC weekly + folate 1 mg daily • PO/SC • weekly
    trigger: Inadequate HCQ alone for arthritis or cutaneous (ACR 2024)
    Steroid-sparing for joint/skin [EULAR 2023 Fanouriakis]
  3. 3. MMF for moderate-severe
    1-1.5 g PO BID (target 2-3 g/d) • PO • BID
    trigger: Lupus nephritis or moderate-severe extrarenal (ACR 2024)
    Preferred induction + maintenance [2024 ACR/EULAR LN]
  4. 4. azathioprine (pregnancy-compatible)
    1.5-2.5 mg/kg/d after TPMT • PO • daily
    trigger: Pregnancy planning, MMF intolerant (ACR 2024)
    Pregnancy-compatible alternative [EULAR 2023; 2024 ACR/EULAR LN]
  5. 5. belimumab
    10 mg/kg IV monthly OR 200 mg SC weekly • IV/SC • monthly/weekly
    trigger: Moderate-severe active despite standard (ACR 2024)
    BLISS-52/76 + BLISS-LN (ACR 2024)
  6. 6. anifrolumab
    300 mg IV q4 weeks • IV • q4 weeks
    trigger: Moderate-severe with skin/joint dominant phenotype (ACR 2024)
    TULIP-1/2 NEJM 2020
  7. 7. voclosporin
    23.7 mg PO BID (with MMF) • PO • BID
    trigger: Lupus nephritis class III/IV/V add-on (ACR 2024)
    AURORA / AURORA-2 (ACR 2024)
  8. 8. aspirin if APS-positive (ACR 2024)
    81 mg PO daily • PO • daily
    trigger: AP antibody positive, no thrombotic event (ACR 2024)
    Primary thromboprophylaxis in APS-positive SLE [EULAR 2023 Fanouriakis]
  9. 9. warfarin if thrombotic APS
    INR 2-3 (3-4 if arterial / triple-positive) • PO • daily
    trigger: Prior thrombosis with APS (ACR 2024)
    Secondary prevention; DOACs not preferred for triple-positive APS [TRAPS Lancet Haematol 2016; EULAR 2023]
  10. 10. bone protection + PJP prophylaxis
    Calcium 1200 mg + vitamin D 800-1000 IU; bisphosphonate per DEXA + steroid; TMP-SMX if pred ≥20 mg ≥4 wks • PO • as listed
    trigger: Chronic steroid (ACR 2024)
    Standard prophylaxis bundle [EULAR 2023]

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Multi-system features (rash + joints + serositis + cytopenia) [2019 EULAR/ACR Aringer ARD 2019]; ANA ≥1:80 with compatible clinical features [2019 EULAR/ACR entry criterion]; Known SLE — routine treat-to-target visit [EULAR 2023 Fanouriakis].

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Systemic lupus erythematosus (chronic, treat-to-target)** (rheum.sle.core.v1).
Phenotype framing: Drug-induced lupus, mixed connective tissue disease, undifferentiated CTD, primary APS, RA-overlap, scleroderma overlap, viral mimics (parvo, EBV) [EULAR 2023]
Scope: Adult chronic SLE management — steady-state visits and treat-to-target [EULAR 2023 Fanouriakis]. Acute flare routes to rheum.sle-flare.core.v1

No severity triggers fired against current inputs.

Plan

Regimen axis: **Chronic disease-modifying therapy (treat-to-target) (ACR 2024)**.
1. hydroxychloroquine (antimalarial, first line) — 2023 EULAR strong: 5 mg/kg actual weight for ALL SLE patients; reduces flares, mortality, thrombosis
2. methotrexate (anti_metabolite, add on) — Steroid-sparing for arthritis / cutaneous predominance [EULAR 2023 Fanouriakis]
3. mycophenolate_mofetil (anti_metabolite, first line) — 2024 ACR/EULAR LN preferred induction + maintenance
4. azathioprine (anti_metabolite, add on) — Maintenance + pregnancy-compatible [EULAR 2023; 2024 ACR/EULAR LN]
5. belimumab (anti_BAFF_mab, add on) — BLISS-52/76 + BLISS-LN; 2023 EULAR strong add-on for active SLE / lupus nephritis
6. anifrolumab (anti_IFNAR_mab, add on) — TULIP-1/2 NEJM 2020 / Lancet RD 2019
7. voclosporin (calcineurin_inhibitor, add on) — AURORA / AURORA-2 — improved renal response when added to MMF (ACR 2024)
8. cyclophosphamide (alkylator, rescue) — Euro-Lupus low-dose IV pulse for severe induction [Houssiau ARD 2002; EULAR 2023]
9. rituximab (anti_CD20_mab, rescue) — Off-label rescue for refractory cytopenia + renal disease [EULAR 2023 conditional]

Setting playbook (outpatient) — Achieve LLDAS / DORIS, minimise glucocorticoid burden, prevent organ damage, plan pregnancy when stable, maintain bone + cardiovascular + cancer screening (ACR 2024)
10. HCQ universal 5 mg/kg actual weight (typically 200-400 mg/d) PO daily — All SLE patients (ACR 2024) (2023 EULAR strong — universal)
11. methotrexate (joint/skin dominant) 15-25 mg PO/SC weekly + folate 1 mg daily PO/SC weekly — Inadequate HCQ alone for arthritis or cutaneous (ACR 2024) (Steroid-sparing for joint/skin [EULAR 2023 Fanouriakis])
12. MMF for moderate-severe 1-1.5 g PO BID (target 2-3 g/d) PO BID — Lupus nephritis or moderate-severe extrarenal (ACR 2024) (Preferred induction + maintenance [2024 ACR/EULAR LN])
13. azathioprine (pregnancy-compatible) 1.5-2.5 mg/kg/d after TPMT PO daily — Pregnancy planning, MMF intolerant (ACR 2024) (Pregnancy-compatible alternative [EULAR 2023; 2024 ACR/EULAR LN])
14. belimumab 10 mg/kg IV monthly OR 200 mg SC weekly IV/SC monthly/weekly — Moderate-severe active despite standard (ACR 2024) (BLISS-52/76 + BLISS-LN (ACR 2024))
15. anifrolumab 300 mg IV q4 weeks IV q4 weeks — Moderate-severe with skin/joint dominant phenotype (ACR 2024) (TULIP-1/2 NEJM 2020)
16. voclosporin 23.7 mg PO BID (with MMF) PO BID — Lupus nephritis class III/IV/V add-on (ACR 2024) (AURORA / AURORA-2 (ACR 2024))
17. aspirin if APS-positive (ACR 2024) 81 mg PO daily PO daily — AP antibody positive, no thrombotic event (ACR 2024) (Primary thromboprophylaxis in APS-positive SLE [EULAR 2023 Fanouriakis])
18. warfarin if thrombotic APS INR 2-3 (3-4 if arterial / triple-positive) PO daily — Prior thrombosis with APS (ACR 2024) (Secondary prevention; DOACs not preferred for triple-positive APS [TRAPS Lancet Haematol 2016; EULAR 2023])
19. bone protection + PJP prophylaxis Calcium 1200 mg + vitamin D 800-1000 IU; bisphosphonate per DEXA + steroid; TMP-SMX if pred ≥20 mg ≥4 wks PO as listed — Chronic steroid (ACR 2024) (Standard prophylaxis bundle [EULAR 2023])

Non-pharmacologic actions:
- Sun protection (SPF 50+, broad-spectrum) (ACR 2024)
- Smoking cessation (ACR 2024)
- Cardiovascular risk modification (BP, lipids, weight) (ACR 2024)
- Vaccination optimisation (live vaccines NOT during high immunosuppression) (ACR 2024)
- Pregnancy planning when stable ≥6 months on compatible drugs (ACR 2024)
- Mental health support (ACR 2024)
- Adherence support (ACR 2024)

AVOID / contraindication checks:
- HCQ retinal screen baseline and annual after 5y (ACR 2024)
- MMF MTX CYC block in pregnancy (ACR 2024)
- CYC fertility counsel and leuprolide protection (ACR 2024)
- Rituximab HBV screen pre dose (ACR 2024)
- Voclosporin block with strong CYP3A inhibitors (ACR 2024)

Monitoring

Regimen monitoring:
- CBC BMP UA UPCR q3mo (ACR 2024)
- complement dsDNA q3-6mo (ACR 2024)
- HCQ retina screen baseline then annual after 5y (ACR 2024)
- lipid A1c annual (ACR 2024)
- DEXA q1-2y on chronic steroid (ACR 2024)

Setting (outpatient) monitoring:
- CBC, BMP, UA, UPCR q3 months minimum (ACR 2024)
- C3/C4, dsDNA q3-6 months (ACR 2024)
- HCQ retina baseline + annual after 5 years (ACR 2024)
- Lipid + A1c annually (ACR 2024)
- DEXA q1-2 years on chronic steroid (ACR 2024)
- Cancer screening per protocol (ACR 2024)

Follow-up plan: 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
- Close-out criterion: long-term plan + counselling complete

Monitoring phase: 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]

Disposition

Current setting: outpatient — Achieve LLDAS / DORIS, minimise glucocorticoid burden, prevent organ damage, plan pregnancy when stable, maintain bone + cardiovascular + cancer screening (ACR 2024)

Disposition criteria:
- Continue outpatient q3 months stable; q4-6 weeks during titration (ACR 2024)

Escalation triggers (move to higher acuity):
- New organ-threatening flare → switch to rheum.sle-flare.core.v1 pathway (ACR 2024)
- Pregnancy detection → MMF/CYC/MTX immediate switch + MFM (ACR 2024)
- New thrombosis → APS workup + anticoagulation (ACR 2024)
- Refractory disease → biologic add-on or rituximab off-label (ACR 2024)

Patient Action Plan

**SLE chronic self-management plan [EULAR 2023 Fanouriakis treat-to-target]**
Personalised values: active_organ_systems (ACR 2024), current_immunosuppression (ACR 2024), aps_status, pregnancy_intent.

**Stable / in remission (ACR 2024)** (green):
Triggers:
- No new symptoms
- Stable labs
- Taking medications as prescribed
Actions:
- Take HCQ every day (do not skip)
- Sun protection always (SPF 50+)
- Keep follow-up labs every 3 months
- Annual eye exam (or every 6-12 months after 5 years on HCQ)
- Vaccinations up to date — never live vaccine on high immunosuppression
- Quit smoking; manage BP, weight, lipids
- Plan pregnancy when stable ≥6 months on compatible drugs

**Possible flare or warning sign (ACR 2024)** (yellow):
Triggers:
- New rash, joint pain, oral ulcers, fatigue
- New foamy urine (proteinuria)
- New chest pain on breathing (pleuritis)
- Hair loss
- Recent missed doses
- New medication started
Actions:
- Continue current medications
- Call your rheumatologist within 1-3 days
- Avoid sun exposure
- Go to lab early for CBC, UA, UPCR, complement, dsDNA
Contact provider when:
- Symptoms not improving in 3-5 days
- New proteinuria
- Any new cardiopulmonary symptom

**Severe flare or emergency (ACR 2024)** (red):
Triggers:
- Severe headache, seizure, confusion, weakness
- Cough up blood, severe shortness of breath
- Severe abdominal pain, vomiting
- New thrombosis (leg pain/swelling, chest pain, neuro deficit)
- Severe rash with fever (DRESS, vasculitis)
- Pregnancy bleed or pre-eclampsia symptoms
- High fever + immunosuppression
Actions:
- Go to ED immediately
- Bring medication list including HCQ + immunosuppressants
- Tell ED about your SLE, immunosuppression, and APS status
Contact provider when:
- Always go to ED for these symptoms

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [SEVERE] New UPCR >0.5 g/g or active urine sediment on routine surveillance [2024 ACR/EULAR LN biopsy threshold]
- [SEVERE] New venous or arterial thrombosis in SLE patient with APS [ACR 2024]
- [SEVERE] Pregnancy detected in SLE patient with active disease or on teratogen [EULAR 2023 Fanouriakis]

Citations

- 2023 EULAR Recommendations for the Management of SLE (Fanouriakis et al, Ann Rheum Dis 2024) + 2024 ACR/EULAR Lupus Nephritis Guideline + 2019 EULAR/ACR SLE Classification Criteria + KDIGO 2021 GN [PMID:37827694](https://pubmed.ncbi.nlm.nih.gov/37827694/)
- Cited evidence (PMID 31385462) [PMID:31385462](https://pubmed.ncbi.nlm.nih.gov/31385462/)
- Cited evidence (PMID 33971155) [PMID:33971155](https://pubmed.ncbi.nlm.nih.gov/33971155/)
- Cited evidence (PMID 37466424) [PMID:37466424](https://pubmed.ncbi.nlm.nih.gov/37466424/)
- Cited evidence (PMID 32937045) [PMID:32937045](https://pubmed.ncbi.nlm.nih.gov/32937045/)

Last reconciled with current guidelines: 2026-05-22.
References
  • 2023 EULAR Recommendations for the Management of SLE (Fanouriakis et al, Ann Rheum Dis 2024) + 2024 ACR/EULAR Lupus Nephritis Guideline + 2019 EULAR/ACR SLE Classification Criteria + KDIGO 2021 GN — PMID:37827694
  • Cited evidence (PMID 31385462) — PMID:31385462
  • Cited evidence (PMID 33971155) — PMID:33971155
  • Cited evidence (PMID 37466424) — PMID:37466424
  • Cited evidence (PMID 32937045) — PMID:32937045