All dossiers
rheum.gca.chronic.v1
Giant cell arteritis (GCA / temporal arteritis)
rheumatologyacutechronicadultgeriatricacuteoutpatient
Manifest is a Batch-23 scaffold — atoms / phenotypes / regimen drug list not yet authored. Regimen axis intentionally empty — prednisone / tocilizumab / methotrexate / aspirin require manifest backing. Large-vessel GCA phenotype + steroid taper protocols need dedicated phenotype axes when manifest splits them.
Entry points (6)
- symptomNew-onset headache in patient ≥50 (ACR 2021 classification criterion)new_onset_headache_age_50plus
- symptomJaw claudication (highest LR+ for GCA — ACR 2021)jaw_claudication
- symptomAmaurosis fugax / acute visual loss (ACR 2021 / EULAR 2023 — IV pulse indication)transient_or_persistent_visual_loss
- symptomScalp tenderness or temporal artery abnormality (ACR 2021)scalp_tenderness
- lab_abnormalityMarkedly elevated ESR or CRP in elderly patient (ACR 2021 / BSR 2020)esr_crp_marked_elevation
- problem_listPolymyalgia rheumatica with cranial features (ACR 2021 — GCA coexists in 15-20% of PMR)pmr_with_cranial_features
Required inputs (12)
- agerequireddemographic • used at CONTEXTGCA essentially restricted to age ≥50 (ACR 2021 classification criterion); rare under 60
- visual_symptomsrequiredsymptom • used at RED_FLAGSVisual loss is irreversible — STAT high-dose steroids before biopsy (ACR 2021 / EULAR 2023 strong recommendation)
- jaw_claudication_qualityrequiredsymptom • used at CONTEXTStrongest LR+ for GCA among presenting features (Smetana JAMA 2002 meta-analysis)
- pmr_featuressymptom • used at CONTEXTPMR present in 40-50% of GCA (BSR 2020); informs treatment + prognosis
- esrrequiredlab • used at INITIAL_WORKUPOften >50 mm/h (ACR 2021); trend on therapy
- crprequiredlab • used at INITIAL_WORKUPMore sensitive than ESR (BSR 2020); key trend marker — CRP suppressed by tocilizumab independent of disease (GiACTA Stone 2017)
- cbcrequiredlab • used at INITIAL_WORKUPNormocytic anemia + thrombocytosis common (BSR 2020)
- lftlab • used at INITIAL_WORKUPMild AST/ALK-P elevation common (BSR 2020); baseline for steroid + tocilizumab hepatotoxicity monitoring
- a1clab • used at TREATMENTSteroid-induced diabetes baseline (ACR 2017 GIO guideline)
- temporal_artery_biopsyrequiredimaging • used at INITIAL_WORKUPGold standard — perform within 14 days of starting steroids (ACR 2021); segmental skip lesions → 1-2 cm sample (BSR 2020)
- temporal_artery_us_or_mriimaging • used at INITIAL_WORKUPHalo sign on color Doppler / MRI vessel-wall enhancement; EULAR 2018/2023 fast-track imaging path
- aortic_imagingimaging • used at BRANCHING_WORKUPLarge-vessel GCA (PET-CT / MRA / CTA aorta) — up to 50% have aortic involvement (ACR 2021 / EULAR 2018)
12-phase flow (12)
- 1FRAMEAdult GCA (cranial + large-vessel phenotypes per ACR 2021). PMR-only without cranial / vascular features routed separatelyinputs: ageadvance: scope confirmed
- 2ENTRYRecognise cranial features in age ≥50 (ACR 2021); PMR with new headache; sudden visual symptomsadvance: entry trigger present
- 3CONTEXTRisk factors (PMR coexistence 40-50% — BSR 2020; female, northern European, age ≥50), comorbidities for steroid-related risk, fall risk, osteoporosisinputs: jaw_claudication_quality, pmr_featuresadvance: risk profile captured
- 4RED_FLAGSVisual loss (transient or persistent), CRAO, diplopia, stroke-like symptoms, severe constitutional decline → STAT IV pulse methylprednisolone before biopsy (ACR 2021 / EULAR 2023 strong)inputs: visual_symptomsadvance: red flags addressed; ophthalmology / neuro engaged
- 5INITIAL_WORKUPESR + CRP + CBC + LFT + creatinine immediately; temporal artery US or MRI (EULAR 2018 fast-track); biopsy within 14 days (ACR 2021); do NOT delay steroids waiting for biopsy (ACR 2021 / BSR 2020)inputs: esr, crp, cbc, temporal_artery_biopsyactions: panel.inflammation, panel.cbcadvance: workup ordered + steroids started
- 6BRANCHING_WORKUPAortic + large-vessel imaging if persistent symptoms / atypical biopsy / suspected LV-GCA (EULAR 2018 — PET-CT / MRA / CTA); bone densitometry baseline (ACR 2017 GIO); PMR overlap workupinputs: aortic_imagingadvance: extracranial mapping complete
- 7DIFFERENTIALCRAO non-arteritic AION, migraine, tension headache, sinusitis, infection, malignancy, ANCA vasculitis, Takayasu (younger — ACR 2021), atherosclerotic diseaseadvance: mimics excluded
- 8RISK_STRATIFICATIONCranial-only vs LV-GCA phenotype (ACR 2021); relapse risk (GiACTA Stone 2017 — ~50% relapse on taper); steroid-toxicity risk profileadvance: phenotype + relapse risk assigned
- 9TREATMENTWithout visual symptoms: prednisone 40-60 mg PO daily (ACR 2021 / EULAR 2023) until symptoms + CRP normalize. With visual symptoms / amaurosis fugax: IV methylprednisolone 500-1000 mg × 3 d → high-dose oral (ACR 2021 strong). Tocilizumab 162 mg SC weekly (or q2wk) for steroid-sparing — Class I (GiACTA Stone NEJM 2017; ACR 2021 + EULAR 2023 strong). Methotrexate alternative steroid-sparer when tocilizumab unavailable (EULAR 2023 conditional). Aspirin 81 mg daily (EULAR 2018 — cardiovascular + ophthalmic protection). Calcium + vitamin D + bisphosphonate for steroid-induced osteoporosis (ACR 2017 GIO). PJP prophylaxis if pred ≥20 mg ≥4 weeks. Slow taper over 18-24 months (BSR 2020 / EULAR 2023)inputs: a1c, lftadvance: induction regimen + adjuncts started
- 10DISPOSITIONOutpatient unless visual symptoms, stroke-like features, or unable to tolerate oral steroids (ACR 2021)inputs: visual_symptomsadvance: level of care set
- 11MONITORINGSymptom + ESR/CRP at 2-4 weeks, then monthly during taper (BSR 2020). Watch for tocilizumab-blunted CRP — rely on symptoms + ESR (GiACTA Stone 2017). DEXA at baseline + annually (ACR 2017 GIO). Glucose monitoring weekly during high-dose steroidsinputs: esr, crp, a1cactions: panel.inflammationadvance: response confirmed
- 12FOLLOWUPSlow steroid taper to ≤5 mg by 12 months when feasible (BSR 2020 / EULAR 2023); aortic surveillance imaging q1-2 years if LV-GCA (EULAR 2018); relapse education + return-precaution counselingadvance: long-term management plan documented