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rheum.gpa.core.v1

Granulomatosis with polyangiitis (GPA)

rheumatologyacutechronicadultacuteinpatientoutpatient
Start encounter →Print handoutPRODUCTION

Manifest pointer is a PLACEHOLDER — prisma/seed/manifests/rheum.gca.chronic.v1.ts (GCA scaffold) reused; no GPA-specific manifest, atoms, or problem-package folder on disk yet (see design brief Open gaps). regimen_axes carry NO rxcui — placeholder manifest; RxNav validation deferred to PRODUCTION (scripts/research/rxnav-validate.ts). BVAS / VDI / EUVAS severity grouping referenced clinically but not exposed as registry calculators; calc.news2 + calc.qsofa used as available deterioration/sepsis-discriminator surfaces. workup.rpgn is the integrated entry workup; workup.acute_weakness / workup.joint_pain are whitelisted branching workups. Bayesian likelihood ratios for PR3/MPO-ANCA + biopsy not yet authored — deferred.

Entry points (6)

  • symptom
    Refractory sinusitis / bloody nasal crusting / septal perforation / saddle-nose / new hearing loss
    chronic_sinusitis_nasal_crusting
  • symptom
    Hemoptysis / hypoxia + active urinary sediment (pulmonary-renal syndrome)
    pulmonary_renal_syndrome
  • lab_abnormality
    Positive c-ANCA / anti-PR3 (or MPO) with multisystem disease
    positive_anca_pr3
  • lab_abnormality
    Rising creatinine + dysmorphic RBCs / RBC casts (RPGN pattern)
    rising_creatinine_active_sediment
  • imaging
    Pulmonary nodules / cavities or diffuse alveolar opacities on CT
    lung_nodules_cavities
  • symptom
    Acute foot/wrist drop — mononeuritis multiplex
    mononeuritis_multiplex

Required inputs (12)

  • ent_disease_historyrequired
    history • used at ENTRY
    Destructive upper-airway disease (sinusitis, septal perforation, saddle-nose, subglottic stenosis) is the GPA-defining axis vs MPA/EGPA (2022 ACR/EULAR)
  • organ_systems_involvedrequired
    symptom • used at RISK_STRATIFICATION
    Drives EUVAS severity grouping (limited vs systemic vs organ/life-threatening) and BVAS (2021 ACR/VF; KDIGO 2024)
  • anca_pr3_mpo_elisarequired
    lab • used at INITIAL_WORKUP
    PR3-ANCA vs MPO-ANCA — PR3 more relapsing, ENT-destructive, rituximab-responsive; MPO sways toward MPA-overlap phenotype (RAVE; KDIGO 2024)
  • creatininerequired
    lab • used at INITIAL_WORKUP
    Cr trend defines RPGN; dialysis dependence drives PEXIVAS PLEX consideration + drug dosing (KDIGO 2024)
  • urinalysis_with_microrequired
    lab • used at INITIAL_WORKUP
    Dysmorphic RBCs / RBC casts / proteinuria = pauci-immune necrotizing crescentic GN (KDIGO 2024)
  • cbc_with_diffrequired
    lab • used at INITIAL_WORKUP
    Anemia (alveolar hemorrhage / GN), leukocytosis, eosinophilia (EGPA pivot); CYC/RTX cytopenia baseline
  • esr_crprequired
    lab • used at INITIAL_WORKUP
    Disease-activity surrogate; CRP elevation prompts infection-vs-flare discrimination on immunosuppression
  • ct_chestrequired
    imaging • used at INITIAL_WORKUP
    Nodules / cavities / ground-glass alveolar hemorrhage define lung involvement and severity (2021 ACR/VF)
  • airway_assessment
    imaging • used at RED_FLAGS
    Subglottic / tracheobronchial stenosis is organ-threatening and may need urgent airway evaluation (2021 ACR/VF)
  • infection_screen_hbv_tbrequired
    history • used at CONTEXT
    HBV/HCV/TB/strongyloides screen pre-rituximab/cyclophosphamide; live-vaccine timing (2021 ACR/VF)
  • fertility_statusrequired
    demographic • used at CONTEXT
    Cyclophosphamide gonadotoxicity — fertility preservation + GnRH-agonist protection; rituximab preferred if fertility priority (2021 ACR/VF)
  • current_immunosuppression
    medication • used at CONTEXT
    Prior CYC cumulative dose (malignancy/bladder risk), prior RTX, glucocorticoid burden inform induction vs relapse strategy (2021 ACR/VF)

12-phase flow (12)

  1. 1FRAME
    Necrotizing granulomatous small-vessel vasculitis spanning ENT + lung + pauci-immune crescentic GN; typically c-ANCA/anti-PR3. New diagnosis vs known-relapsing GPA both handled; pure renal-limited routes to neph engine, MPA/EGPA via §5.5.2 pivots
    inputs: ent_disease_history
    advance: AAV scope confirmed; GPA phenotype framed
  2. 2ENTRY
    Recognize triggering pattern: destructive ENT disease, pulmonary-renal syndrome, positive ANCA, RPGN, lung nodules/cavities, or mononeuritis multiplex
    inputs: ent_disease_history, organ_systems_involved
    advance: triggering presentation captured
  3. 3CONTEXT
    Capture prior immunosuppression + cumulative CYC dose, ANCA serotype if known, infection screen (HBV/HCV/TB/strongyloides), vaccination status, fertility plans, comorbidities affecting drug choice
    inputs: infection_screen_hbv_tb, fertility_status, current_immunosuppression
    advance: immunosuppression history + infection/fertility context captured
  4. 4RED_FLAGS
    Organ/life-threatening disease: diffuse alveolar hemorrhage (hypoxia + falling Hb), rapidly progressive GN / dialysis-dependent AKI, subglottic/airway compromise (stridor), CNS or cardiac vasculitis, mononeuritis multiplex → urgent immunosuppression + ICU/airway team
    inputs: creatinine, airway_assessment
    actions: calc.news2, calc.qsofa
    advance: organ-threatening features screened; ICU/airway escalation triggered
  5. 5INITIAL_WORKUP
    ANCA (PR3/MPO ELISA + IF), UA with micro + UPCR, creatinine/eGFR trend, CBC, ESR/CRP, CMP, coags, CXR + CT chest, hypoxia/ABG if DAH; anti-GBM (co-positivity), hepatitis/HIV serologies; ENT exam/laryngoscopy
    inputs: anca_pr3_mpo_elisa, creatinine, urinalysis_with_micro, cbc_with_diff, esr_crp, ct_chest
    actions: workup.rpgn, panel.renal, panel.ua, panel.cbc, panel.inflammation, panel.cmp
    advance: ANCA + renal + pulmonary + inflammatory workup sent
  6. 6BRANCHING_WORKUP
    Tissue confirmation by least-invasive accessible site — renal biopsy (pauci-immune necrotizing crescentic GN), lung/ENT biopsy (necrotizing granulomatous inflammation + vasculitis); bronchoscopy/BAL for serial-bloodier returns in DAH; nerve/EMG for mononeuritis; echo for cardiac
    inputs: organ_systems_involved
    actions: workup.acute_weakness, panel.cardiac, panel.coag
    advance: biopsy and organ-specific workup booked/completed
  7. 7DIFFERENTIAL
    Within AAV: GPA vs MPA (no granuloma/ENT destruction, MPO/p-ANCA, less relapse) vs EGPA (asthma + eosinophilia, ANCA often negative). Non-AAV mimics: anti-GBM/Goodpasture, cryoglobulinemic vasculitis, IgA vasculitis, SLE, infective endocarditis, cocaine/levamisole-induced midline destruction, IgG4-RD, lymphomatoid granulomatosis, NTM/TB, malignancy
    inputs: anca_pr3_mpo_elisa
    advance: AAV subtype assigned; mimics excluded/co-managed
  8. 8RISK_STRATIFICATION
    EUVAS severity grouping — limited/non-organ-threatening vs systemic vs severe (organ/life-threatening: RPGN, DAH, airway, CNS, cardiac, mononeuritis); 2022 ACR/EULAR classification criteria; BVAS for activity, VDI for damage
    inputs: organ_systems_involved, creatinine
    actions: calc.news2
    advance: EUVAS severity group + BVAS/VDI set; ICU vs ward vs outpatient decided
  9. 9TREATMENT
    Severity-stratified. Organ/life-threatening: high-dose glucocorticoid with reduced-dose PEXIVAS taper (or pulse methylprednisolone 0.25–1 g/d ×1–3 for DAH/RPGN) + rituximab (preferred — relapsing/PR3/fertility) OR cyclophosphamide; avacopan as steroid-sparing C5aR antagonist; plasma exchange SELECTIVELY (severe AKI/dialysis-dependent GN or DAH per PEXIVAS — not routine). Non-severe: glucocorticoid + RTX or methotrexate/MMF. Adjuncts: PJP prophylaxis, bone protection, vaccination (non-live, timed pre-RTX), fertility preservation pre-CYC. Maintenance: rituximab (preferred) or azathioprine/methotrexate, prolonged ≥18–48 mo. Subglottic stenosis: local intralesional steroid/dilation/surgery
    inputs: organ_systems_involved, creatinine, fertility_status
    advance: induction regimen + PEXIVAS steroid taper + prophylaxis + maintenance plan documented
  10. 10DISPOSITION
    Organ/life-threatening (DAH, RPGN/dialysis, airway, CNS/cardiac) → admit, frequently ICU; systemic → admit for induction + biopsy; limited/non-organ-threatening → expedited rheumatology + nephrology outpatient induction
    advance: level of care + subspecialty consults set
  11. 11MONITORING
    Induction: CBC + creatinine + UA weekly→biweekly; CYC nadir CBC at 7–14 d + microscopic hematuria (bladder toxicity); RTX — CD19/CD20 B-cells + IgG, infusion reactions, HBV reactivation; ANCA + clinical surveillance (PR3 rise/ENT recurrence anticipates relapse); infection surveillance on immunosuppression; serial spirometry/laryngoscopy for airway disease
    inputs: creatinine, cbc_with_diff
    actions: panel.renal, panel.cbc, panel.ua
    advance: remission achieved (BVAS 0 / off-glucocorticoid target) — transition to maintenance
  12. 12FOLLOWUP
    Prolonged maintenance (RTX redosing schedule or AZA/MTX) ≥18–48 mo with relapse surveillance; manage accrued damage (VDI) — CKD, hearing loss, subglottic stenosis, nasal deformity; cardiovascular + venous-thromboembolism + infection + malignancy (CYC bladder) long-term risk; vaccination catch-up; bone health; fertility/pregnancy planning when stable
    advance: long-term maintenance + damage-surveillance + relapse plan documented