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rheum.sle-flare.core.v1PRODUCTION
rheum.sle-flare.core.v1

SLE flare (acute)

rheumatologyacuteadult
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12/12 authored

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

Current phase

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Detailed

Confirmed SLE patient with acute flare. New diagnosis routes to rheum.sle.core.v1; APS / pure thrombosis to hem.aps.core.v1

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flare scope confirmed

Patient inputs (12)

HCQ adherence, MMF / AZA / belimumab / anifrolumab on board (EULAR 2023 Fanouriakis)

MMF / CYC / methotrexate teratogenic; flare may herald pre-eclampsia (EULAR 2023 Fanouriakis)

Differentiates flare from new-onset SLE; informs flare type (EULAR 2023 Fanouriakis)

Cytopenias define hematologic flare; rule out infection mimic (EULAR 2023)

Lupus nephritis monitoring; drug dosing (ACR 2024 lupus)

Active sediment (RBC casts, WBC, dysmorphic RBCs) = lupus nephritis flare (ACR 2024 lupus)

UPCR >0.5 induction therapy decision; >3 severe (ACR 2024 lupus nephritis)

Falling C3/C4 supports active disease (EULAR 2023 Fanouriakis)

Rising titer correlates with renal flare (EULAR 2023)

CRP elevation in SLE is unusual without infection / serositis — important infection / mimics flag (EULAR 2023)

Drives mild / moderate / severe classification + therapy intensity (EULAR 2023; ACR 2024)

Thrombotic phenotype risk; affects anticoagulation and pregnancy management (EULAR 2023; ACR 2024)

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

Severity triggers (7)

7 need judgement
  • informationallife_threateningalveolar_hemorrhage
    Hypoxia + hemoptysis + diffuse pulmonary infiltrates + falling Hb
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningnpsle_severe
    Psychosis, seizure, myelitis, cerebritis, coma in SLE patient
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningcatastrophic_aps
    Multiple thrombotic events in <1 week with multi-organ failure
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningtma_thrombocytopenia_with_microangiopathic_hemolysis
    Schistocytes + low platelets + AKI in SLE patient
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverelupus_nephritis_active_class_III_IV_V
    New proteinuria >0.5 g/g + active urine sediment + AKI
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevererefractory_severe_thrombocytopenia
    Platelets <30K with bleeding or refractory to first-line
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereinfection_mimicking_flare
    Fever + multi-organ symptoms + ELEVATED CRP (rare in pure SLE flare)
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

SLE flare — mild / moderate / severe (2023 EULAR + 2024 ACR/EULAR LN)
axis: sle_flare_severity_basedstep 1 - Mild flare — mucocutaneous, mild arthritis
Selected step "Mild flare — mucocutaneous, mild arthritis" — Mucocutaneous (rash, oral ulcer, alopecia), mild arthritis, fatigue without organ-threatening features
  • hydroxychloroquine
    first line
    antimalarial
    5 mg/kg actual weight (typically 200-400 mg/d) • PO • daily
    triggers: any_SLE_diagnosis_universal
    2023 EULAR strong — universal for all SLE; reduces flares + thrombosis + mortality
    rxcui 5521
  • prednisone_low_dose
    add on
    corticosteroid
    5-15 mg PO daily short course • PO • daily, taper over 2-4 weeks
    triggers: mild_flare
    Short course; minimise cumulative dose (EULAR 2023 Fanouriakis)
    rxcui 8640
  • topical_corticosteroid
    first line
    topical_steroid
    Per location (mid-potency for face, high-potency for trunk/extremities) • topical • BID x 2-4 weeks
    triggers: cutaneous_flare
    First-line topical for cutaneous lupus (EULAR 2023)

outpatient playbook — drug actions (4)

  1. 1. HCQ verify/restart
    5 mg/kg actual weight • PO • daily
    trigger: Any flare
    Universal SLE therapy (EULAR 2023 Fanouriakis)
  2. 2. topical or low-dose oral steroid (mild)
    Topical mid-potency or prednisone 5-15 mg/d • topical/PO • daily
    trigger: Mucocutaneous / mild arthritis
    Short course minimises cumulative steroid (EULAR 2023)
  3. 3. moderate-dose steroid + steroid sparer
    Prednisone 0.5 mg/kg + MMF 1-1.5 g BID OR MTX 15-25 mg weekly OR AZA 2 mg/kg/d • PO/SC • daily/weekly
    trigger: Serositis, moderate cytopenias, severe arthritis
    EULAR 2023 + LN 2024
  4. 4. belimumab or anifrolumab
    Belimumab 200 mg SC weekly; anifrolumab 300 mg IV q4 weeks • SC/IV • weekly/q4 weeks
    trigger: Persistent moderate activity despite standard
    Add-on biologics (BLISS Navarra Lancet 2011; TULIP Morand NEJM 2020)

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: New / worsening malar rash, photosensitive flare; Inflammatory polyarthritis / pleuritic pain; Rising anti-dsDNA / falling C3-C4.

Objective

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

Assessment

**SLE flare (acute)** (rheum.sle-flare.core.v1).
Phenotype framing: Distinguish flare from infection (CRP usually normal in pure flare), drug-induced lupus-like reaction, TMA / TTP / aHUS, MAS, malignancy
Scope: Confirmed SLE patient with acute flare. New diagnosis routes to rheum.sle.core.v1; APS / pure thrombosis to hem.aps.core.v1

No severity triggers fired against current inputs.

Plan

Regimen axis: **SLE flare — mild / moderate / severe (2023 EULAR + 2024 ACR/EULAR LN)** — step "Mild flare — mucocutaneous, mild arthritis".
1. hydroxychloroquine 5 mg/kg actual weight (typically 200-400 mg/d) PO daily (antimalarial, first line) — 2023 EULAR strong — universal for all SLE; reduces flares + thrombosis + mortality
2. prednisone_low_dose 5-15 mg PO daily short course PO daily, taper over 2-4 weeks (corticosteroid, add on) — Short course; minimise cumulative dose (EULAR 2023 Fanouriakis)
3. topical_corticosteroid Per location (mid-potency for face, high-potency for trunk/extremities) topical BID x 2-4 weeks (topical_steroid, first line) — First-line topical for cutaneous lupus (EULAR 2023)

Setting playbook (outpatient) — Manage mild-moderate flare with steroid burst + steroid-sparer escalation, exclude infection mimic, expedite rheum follow-up
4. HCQ verify/restart 5 mg/kg actual weight PO daily — Any flare (Universal SLE therapy (EULAR 2023 Fanouriakis))
5. topical or low-dose oral steroid (mild) Topical mid-potency or prednisone 5-15 mg/d topical/PO daily — Mucocutaneous / mild arthritis (Short course minimises cumulative steroid (EULAR 2023))
6. moderate-dose steroid + steroid sparer Prednisone 0.5 mg/kg + MMF 1-1.5 g BID OR MTX 15-25 mg weekly OR AZA 2 mg/kg/d PO/SC daily/weekly — Serositis, moderate cytopenias, severe arthritis (EULAR 2023 + LN 2024)
7. belimumab or anifrolumab Belimumab 200 mg SC weekly; anifrolumab 300 mg IV q4 weeks SC/IV weekly/q4 weeks — Persistent moderate activity despite standard (Add-on biologics (BLISS Navarra Lancet 2011; TULIP Morand NEJM 2020))

Non-pharmacologic actions:
- Sun protection (SPF 50+, broad-spectrum) (EULAR 2023)
- Vaccinate (pneumococcal, influenza, COVID, zoster recombinant when stable) (EULAR 2023 Fanouriakis)
- Bone protection (calcium, vitamin D, DEXA, bisphosphonate if cumulative steroid) (ACR 2024)
- Cardiovascular risk (BP, lipids, ASCVD) (EULAR 2023)
- Pregnancy planning when stable >=6 months on compatible drugs (EULAR 2023 Fanouriakis)

AVOID / contraindication checks:
- HCQ retinal screen baseline and annual after 5y (AAO 2024; EULAR 2023)
- MMF MTX CYC block in pregnancy (EULAR 2023 Fanouriakis)
- CYC fertility counsel and leuprolide protection (ACR 2024 lupus)
- Rituximab HBV screen pre dose (EULAR 2023)
- Voclosporin block with strong CYP3A inhibitors (AURORA Rovin 2021)
- Azathioprine TPMT test pre prescription (EULAR 2023)
- PJP prophylaxis on prednisone 20 x 4 weeks or CYC (EULAR 2023)

Monitoring

Regimen monitoring:
- CBC BMP UA UPCR weekly during induction (ACR 2024 lupus)
- complement dsDNA q4-6 weeks (EULAR 2023 Fanouriakis)
- LFT q2 weeks for MMF AZA (EULAR 2023)
- CYC cytopenia nadir at 7-14 days (Houssiau Euro-Lupus 2002)
- voclosporin BP creatinine q2 weeks first 12 weeks (AURORA Rovin 2021)
- response at 3-6 months MMF or 6-12 months CYC (ACR 2024 lupus)
- HCQ retina baseline then annual after 5 years (AAO 2024; EULAR 2023)

Setting (outpatient) monitoring:
- CBC, BMP, UA, UPCR q2-4 weeks during titration (ACR 2024 lupus)
- C3/C4, dsDNA q1-3 months (EULAR 2023)
- HCQ retina baseline + annual after 5 years (AAO 2024)
- Clinical response at 3-6 months (ACR 2024 lupus)

Follow-up plan: Lifelong rheumatology continuity; SLE-PASS / SLEDAI-2K trend; minimise glucocorticoids; vaccinations (zoster, pneumococcal, HPV, COVID, influenza); CV risk modification; pregnancy planning when stable ≥6 months
- Close-out criterion: long-term management plan documented

Monitoring phase: CBC, BMP, UA, UPCR weekly during induction; complement / dsDNA q4-6 wk; LFT for MMF / AZA; CYC cytopenia at nadir 7-14 d; voclosporin BP + creatinine + GFR q2 wk first 12 wk; HCQ retina screen baseline + annual after 5 y per AAO 2024; bone protection per dose / duration

Disposition

Current setting: outpatient — Manage mild-moderate flare with steroid burst + steroid-sparer escalation, exclude infection mimic, expedite rheum follow-up

Disposition criteria:
- Continue outpatient unless severe organ-threatening flare (EULAR 2023)

Escalation triggers (move to higher acuity):
- New proteinuria > 0.5 g/g, active sediment then urgent renal biopsy (ACR 2024 lupus nephritis)
- New seizure, psychosis, focal deficit then ED for NPSLE workup (EULAR 2023)
- Alveolar hemorrhage / hypoxia then ED + ICU (EULAR 2023)
- Refractory thrombocytopenia (<30K) then admit (BSR 2018)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Hypoxia + hemoptysis + diffuse pulmonary infiltrates + falling Hb
- [LIFE_THREATENING] Psychosis, seizure, myelitis, cerebritis, coma in SLE patient
- [LIFE_THREATENING] Multiple thrombotic events in <1 week with multi-organ failure

Citations

- 2023 EULAR Recommendations for SLE (Fanouriakis et al, ARD 2024) + 2024 ACR/EULAR Lupus Nephritis + AURORA / AURORA-2 (voclosporin) + TULIP (anifrolumab) + BLISS-LN (belimumab) [PMID:37827694](https://pubmed.ncbi.nlm.nih.gov/37827694/)
- 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/)
- Cited evidence (PMID 38229377) [PMID:38229377](https://pubmed.ncbi.nlm.nih.gov/38229377/)

Last reconciled with current guidelines: 2026-05-22.
References
  • 2023 EULAR Recommendations for SLE (Fanouriakis et al, ARD 2024) + 2024 ACR/EULAR Lupus Nephritis + AURORA / AURORA-2 (voclosporin) + TULIP (anifrolumab) + BLISS-LN (belimumab) — PMID:37827694
  • Cited evidence (PMID 33971155) — PMID:33971155
  • Cited evidence (PMID 37466424) — PMID:37466424
  • Cited evidence (PMID 32937045) — PMID:32937045
  • Cited evidence (PMID 38229377) — PMID:38229377