All dossiers
rheum.sle-flare.core.v1
SLE flare (acute)
rheumatologyacuteadultacuteinpatientoutpatient
Manifest is a Batch-23 scaffold — atoms / phenotypes / regimen drug list not yet authored; full atoms live on rheum.sle.core.v1 and need to be partitioned for flare-specific workflow. Regimen axis intentionally empty — HCQ / MMF / CYC / voclosporin / belimumab / anifrolumab / rituximab / pulse methylpred require manifest backing. Pregnancy phenotype + NPSLE require dedicated phenotype splits when manifest is authored.
Entry points (6)
- symptomNew / worsening malar rash, photosensitive flaremalar_rash_or_photosensitivity_active
- symptomInflammatory polyarthritis / pleuritic painjoint_pain_serositis
- lab_abnormalityRising anti-dsDNA / falling C3-C4rising_ds_dna_or_falling_complement
- lab_abnormalityNew proteinuria UPCR > 0.5 (lupus nephritis)new_proteinuria
- symptomActive hemolytic anemia / thrombocytopenia / leukopeniacytopenias_active
- symptomNew seizure / psychosis / cerebritis / myelitiscns_features_active
Required inputs (12)
- sle_diagnosis_confirmedrequiredhistory • used at ENTRYDifferentiates flare from new-onset SLE; informs flare type (EULAR 2023 Fanouriakis)
- current_immunosuppressionrequiredhistory • used at CONTEXTHCQ adherence, MMF / AZA / belimumab / anifrolumab on board (EULAR 2023 Fanouriakis)
- organ_systems_involvedrequiredsymptom • used at RISK_STRATIFICATIONDrives mild / moderate / severe classification + therapy intensity (EULAR 2023; ACR 2024)
- cbc_with_diffrequiredlab • used at INITIAL_WORKUPCytopenias define hematologic flare; rule out infection mimic (EULAR 2023)
- creatininerequiredlab • used at INITIAL_WORKUPLupus nephritis monitoring; drug dosing (ACR 2024 lupus)
- urinalysis_with_microrequiredlab • used at INITIAL_WORKUPActive sediment (RBC casts, WBC, dysmorphic RBCs) = lupus nephritis flare (ACR 2024 lupus)
- upcrrequiredlab • used at INITIAL_WORKUPUPCR >0.5 induction therapy decision; >3 severe (ACR 2024 lupus nephritis)
- complement_c3_c4requiredlab • used at INITIAL_WORKUPFalling C3/C4 supports active disease (EULAR 2023 Fanouriakis)
- anti_dsdnarequiredlab • used at INITIAL_WORKUPRising titer correlates with renal flare (EULAR 2023)
- esr_crprequiredlab • used at RED_FLAGSCRP elevation in SLE is unusual without infection / serositis — important infection / mimics flag (EULAR 2023)
- anti_phospholipid_panellab • used at BRANCHING_WORKUPThrombotic phenotype risk; affects anticoagulation and pregnancy management (EULAR 2023; ACR 2024)
- pregnancy_statusrequireddemographic • used at CONTEXTMMF / CYC / methotrexate teratogenic; flare may herald pre-eclampsia (EULAR 2023 Fanouriakis)
12-phase flow (12)
- 1FRAMEConfirmed SLE patient with acute flare. New diagnosis routes to rheum.sle.core.v1; APS / pure thrombosis to hem.aps.core.v1inputs: sle_diagnosis_confirmedadvance: flare scope confirmed
- 2ENTRYRecognise organ-specific or constitutional flare featuresinputs: organ_systems_involvedadvance: flare features documented
- 3CONTEXTCurrent ISD, HCQ adherence, recent infections, vaccinations, pregnancy / lactation, comorbiditiesinputs: current_immunosuppression, pregnancy_statusadvance: baseline ISD and adherence captured
- 4RED_FLAGSSevere flare — rapidly progressive GN, alveolar hemorrhage, NPSLE psychosis / seizure / myelitis, refractory thrombocytopenia, catastrophic APS → ICU + pulse methylpredinputs: cbc_with_diff, creatinine, esr_crpadvance: red flags acted on; infection ruled out
- 5INITIAL_WORKUPCBC + diff, creatinine, UA + UPCR, C3/C4, anti-dsDNA, ESR/CRP, LFT, AP panel, infection screen (cultures, CMV/HSV PCR if immunosuppressed)inputs: cbc_with_diff, creatinine, urinalysis_with_micro, upcr, complement_c3_c4, anti_dsdna, esr_crpactions: panel.cbc, panel.renal, panel.ua, panel.inflammationadvance: workup sent + infection screen complete
- 6BRANCHING_WORKUPRenal biopsy if proteinuria >0.5 / active sediment / unexplained AKI; brain MRI / LP for NPSLE; echo for myocarditis / pericardial effusion; CT chest for alveolar hemorrhage; APS panel if thrombosisinputs: anti_phospholipid_panelactions: workup.aki, workup.rpgn, workup.apsadvance: organ-specific workup booked / completed
- 7DIFFERENTIALDistinguish flare from infection (CRP usually normal in pure flare), drug-induced lupus-like reaction, TMA / TTP / aHUS, MAS, malignancyinputs: esr_crpactions: tma_differentialadvance: mimics excluded or co-managed
- 8RISK_STRATIFICATIONMild (mucocutaneous / mild arthritis), moderate (serositis, mild cytopenias), severe (renal class III/IV/V, NPSLE, AHA, severe thrombocytopenia, lupus pneumonitis, alveolar hemorrhage)inputs: organ_systems_involvedadvance: severity tier set
- 9TREATMENTHCQ for ALL (5 mg/kg actual weight, 2023 EULAR). Mild: topical steroids + intra-articular + short-course low-dose prednisone. Moderate: prednisone 0.5 mg/kg/d + add MMF / AZA / methotrexate / belimumab / anifrolumab. Severe: pulse methylprednisolone 250-1000 mg × 3 d → prednisone 0.5-1 mg/kg/d + MMF (preferred) or Euro-Lupus CYC (low-dose IV) for class III/IV ± voclosporin add-on (AURORA-LN). Belimumab BLISS-LN for renal flare add-on. Anifrolumab TULIP for moderate-severe with skin / joint dominance. Rituximab refractory cytopenias. PJP prophylaxis if pred ≥20 mg ≥4 weeks or CYC. Avoid MMF / CYC / MTX in pregnancy — switch to AZA / HCQ-only / tacrolimusinputs: creatinine, pregnancy_statusadvance: induction regimen + steroid taper plan documented
- 10DISPOSITIONSevere flare → admit (often ICU for NPSLE / alveolar hemorrhage / refractory cytopenias); moderate → expedited rheum f/u; mild → outpatientadvance: level of care set
- 11MONITORINGCBC, 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 / durationinputs: cbc_with_diff, creatinine, upcractions: panel.renal, panel.cbcadvance: response by 3-6 months (MMF) / 6-12 months (CYC)
- 12FOLLOWUPLifelong rheumatology continuity; SLE-PASS / SLEDAI-2K trend; minimise glucocorticoids; vaccinations (zoster, pneumococcal, HPV, COVID, influenza); CV risk modification; pregnancy planning when stable ≥6 monthsadvance: long-term management plan documented