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pulm.abpa.v1

Allergic Bronchopulmonary Aspergillosis (ABPA)

pulmonologychronicadultoutpatientinpatient
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NEW dossier (design-disease-first, 2026-05-16): pulm.abpa.v1 did not exist; pulm.asthma.core.v1 + the bronchiectasis differential referenced ABPA as a look-alike with no terminal engine — this dossier is that terminal engine and cross-links back. Closest sibling pulm.asthma.core.v1 (structure/shape copied); Bayesian exemplar pulm.pe.core.v1 (the ABPA diagnosis IS a Bayesian criteria-bundle). Design brief authored FIRST: _briefs/pulm.abpa.v1.md. §5.5.1 ≥10 effect-size numbers, all PMID-anchored + PubMed-verified 2026-05-16 (13 PMIDs): Agarwal medium-vs-high-dose steroid RCT — exac 1yr 50% vs 40.9% (p=0.59), GC-dependent 2yr 14.6% vs 11.4% (p=0.88), medium-dose cumulative dose/AEs lower (PMID 26585431); Stevens itraconazole RCT response 46% vs 19% placebo P=0.04 (PMID 10717010); Wark Cochrane sputum eos ↓35% vs 19% p<0.01, exac 0.4 vs 1.3/pt p<0.03, IgE↓≥25% Peto OR 3.30 95%CI 1.30–8.15 (PMID 15266440); Agarwal prednisolone vs pred+itraconazole 1yr exac 33% vs 20.6% p=0.054, IgE↓~47.6%/45.5% (PMID 34503983); Ram nebulised amphotericin B exac 8.3% vs 66.7% at 1yr p=0.016 (PMID 26666774); ABPA prevalence ≈2.0M India (PMID 36589484). evidence.last_reconciled 2026-05-16. §5.5.2 differential/Bayesian as DATA (priority — ABPA dx IS a Bayesian criteria-bundle): ISHAM obligatory+supportive criteria + Patterson/ISHAM staging (0–VI) + serologic-ABPA/ABPA-CB/ABPA-CB-ORF encoded across 7 severity_triggers (isham_criteria_bundle, abpa_staging_bayesian, total_ige_response_action_chain, azole_hepatotoxicity_or_subtherapeutic_tdm, massive_hemoptysis_from_bronchiectasis, azole_in_pregnancy_contraindicated, differential_abpa_vs_mimics), DIFFERENTIAL/BRANCHING_WORKUP phase logic with IgE/IgG/eosinophil/HRCT test-characteristic pivots, calculator guideline_basis, and 3 sibling_differentiation blocks. Total-IgE threshold → action chain (25–35% fall = response/taper; ≥50% rise = exacerbation/escalate). Cross-dossier engine_ids (4, ALL verified on disk via grep src/lib/dossiers/): pulm.asthma.core.v1, id.invasive-aspergillosis.core.v1, pulm.idiopathic_pulmonary_fibrosis.v1, pulm.cap.core.v1 — wired via workups[].branches_to + sibling_differentiation + severity_trigger routes. pulm.bronchiectasis.core.v1 does NOT exist on disk → "other bronchiectasis" encoded as differential-as-data (NOT a branches_to id) per scope. Registry ids reused (ALL resolve in clinical-tools-registry by id — confirmed by grep): workups workup.bronchiectasis_exac (registry entry explicitly references ABPA/CF/immunoglobulin workup — the natural ABPA branching workup), workup.hemoptysis, workup.chronic_cough, workup.ild_acute_exac; calculators calc.act, calc.ckd_epi_2021; panels panel.cbc, panel.lft. No invented/unresolved ids. Regimen axis abpa_stage_directed (chronic): Step1 OCS induction (medium-dose prednisolone — Agarwal PMID 26585431; TAPER encoded in rationale/monitoring, NOT a taper_plan field — no such field in _types.ts), Step2 azole steroid-sparing (itraconazole/voriconazole + MANDATORY TDM, Stevens PMID 10717010 / Wark PMID 15266440), Step3 biologic steroid-sparing (omalizumab/mepolizumab/dupilumab, Eraso PMID 33050821) + nebulised amphotericin B adjunct (Ram PMID 26666774). ≥4 special-pop branches delivered (6): CF-ABPA (distinct — Ashkenazi PMID 29950869), pregnancy (azole TERATOGENIC — contraindicated, steroid/omalizumab), azole-resistance/hepatotoxicity, hepatic-impairment, end-stage-fibrotic, plus glucocorticoid-dependent stage logic. Azole+pregnancy and azole CYP3A4 (statin/DOAC/corticosteroid potentiation) interactions encoded in contraindication_rules. RxCUI: all RxNav REST-verified 2026-05-16 (https://rxnav.nlm.nih.gov/REST/rxcui/{cui}/properties.json, TTY=IN): prednisolone 8638, itraconazole 28031, voriconazole 121243, omalizumab 302379, mepolizumab 1720597, dupilumab 1876376, amphotericin B 732 (base ingredient — nebulised product SCD/SBD flagged NEEDS_RXNAV_VALIDATION, NOT hand-authored). No hand-authored CUIs. SCHEMA-GAP NOTES: (1) _types.ts has NO first-class Bayesian-criteria / staging / decision-threshold / TDM / special-population-matrix / effect-size field — encoded in severity_triggers, phase purpose/advance_when, calculator guideline_basis, regimen rationale/triggers/contraindication_rules, and _briefs/pulm.abpa.v1.depth.md tables; (2) no taper_plan field — steroid taper encoded in RegimenDrug.rationale + axis monitoring; (3) RequiredCalculator.drives enum lacks diagnostic_gate — calc.act reuses risk_stratification; (4) manifest re-points to prisma/seed/manifests/id.sepsis.core.v1.ts (real on-disk shared manifest, same pattern as id.dengue.v1) — ABPA-specific manifest/package/atoms OOS; package undefined, atoms []. STATUS PLANNED per scope (engine_id reserved). Not added to _registry.ts (OOS) so the dossier-contract describe.each does not execute against it. Terminology: ICD-10 B44.81 (ABPA) + B44.9/J45.50/J47.9; SNOMED/LOINC best-effort (backfilled later via terminology pipeline). Companion depth payload _briefs/pulm.abpa.v1.depth.md (pulm.copd.core.v1.depth.md format) + research bundle _research-bundles/pulm.abpa.v1.md (PubMed PMID/DOI/URL + criteria-vs-dossier comparison, retrieval 2026-05-16).

Entry points (6)

  • problem_list
    Poorly-controlled or steroid-dependent asthma — screen for ABPA (ISHAM-ABPA; Agarwal Clin Exp Allergy 2013 PMID 23889240)
    poorly_controlled_asthma
  • problem_list
    Cystic fibrosis with clinical/spirometric deterioration — CF-ABPA screen (ISHAM-ABPA; CF-ABPA distinct entity, Ashkenazi PMID 29950869)
    cystic_fibrosis
  • symptom
    Expectoration of brownish/black mucus plugs (characteristic of ABPA mucoid impaction; Agarwal review PMID 38154470)
    brownish_mucus_plugs
  • imaging
    Fleeting/transient pulmonary infiltrates or central bronchiectasis on imaging (ISHAM supportive radiology PMID 23889240)
    fleeting_infiltrates
  • lab_abnormality
    Unexplained peripheral blood eosinophilia >500/µL in an asthmatic (ISHAM supportive criterion PMID 23889240)
    unexplained_eosinophilia
  • lab_abnormality
    Markedly elevated total serum IgE (>500, classically >1000 IU/mL) in asthma/CF (ISHAM obligatory criterion PMID 23889240)
    high_total_ige

Required inputs (16)

  • agerequired
    demographic • used at CONTEXT
    Adult ABPA pathway; biologic eligibility and azole TDM targets are age-aware
  • asthma_or_cf_statusrequired
    history • used at FRAME
    Predisposing condition is OBLIGATORY for ABPA — asthma vs CF changes thresholds and the CF-ABPA branch (ISHAM-ABPA PMID 23889240; Ashkenazi PMID 29950869)
  • prior_abpa_stage
    history • used at CONTEXT
    ISHAM/Patterson stage (remission vs exacerbation vs glucocorticoid-dependent vs end-stage) sets the prior for treatment selection (Agarwal PMID 23889240)
  • steroid_historyrequired
    history • used at CONTEXT
    Eosinophil count must be interpreted steroid-naïve; prior OCS courses define glucocorticoid-dependent stage (ISHAM PMID 23889240)
  • pregnancy_statusrequired
    history • used at CONTEXT
    Azoles are TERATOGENIC and contraindicated in pregnancy — drives steroid/omalizumab-only branch (azole label; ISHAM management PMID 23889240)
  • current_medsrequired
    medication • used at CONTEXT
    Azole CYP3A4 interactions (statins, DOACs, inhaled/systemic corticosteroid potentiation → iatrogenic Cushing/adrenal suppression) (Wark Cochrane PMID 15266440)
  • total_igerequired
    lab • used at INITIAL_WORKUP
    OBLIGATORY diagnostic gate (>500, classically >1000 IU/mL) AND the principal treatment-response biomarker — 25–35% fall = response, ≥50% rise over baseline = exacerbation (Agarwal PMID 34503983 ~47.6% decline at response; PMID 23889240)
  • asp_specific_igerequired
    lab • used at INITIAL_WORKUP
    OBLIGATORY — A. fumigatus-specific IgE (or immediate-type skin test) elevation (ISHAM obligatory criterion PMID 23889240)
  • asp_specific_igg
    lab • used at INITIAL_WORKUP
    SUPPORTIVE — A. fumigatus-specific IgG / precipitins (ISHAM supportive criterion PMID 23889240)
  • absolute_eosinophils
    lab • used at INITIAL_WORKUP
    SUPPORTIVE — peripheral eosinophilia >500/µL (steroid-naïve) (ISHAM supportive criterion PMID 23889240)
  • lft
    lab • used at TREATMENT
    Baseline + serial liver enzymes before/on azole — itraconazole/voriconazole hepatotoxicity (Wark Cochrane PMID 15266440)
  • azole_trough_level
    lab • used at MONITORING
    Mandatory therapeutic drug monitoring — itraconazole/voriconazole have erratic absorption + narrow therapeutic window; subtherapeutic troughs = treatment failure (Stevens NEJM 2000 PMID 10717010)
  • hrct_chestrequired
    imaging • used at INITIAL_WORKUP
    SUPPORTIVE radiology + staging — fleeting infiltrates, central bronchiectasis, high-attenuation mucus (HAM), mucoid impaction; defines serologic-ABPA vs ABPA-CB vs ABPA-CB-ORF (ISHAM PMID 23889240; Agarwal PMID 38154470)
  • spirometry
    imaging • used at RISK_STRATIFICATION
    Lung-function decline tracks ABPA activity and bronchiectasis progression; secondary endpoint in ABPA RCTs (Agarwal PMID 26585431)
  • sputum_culture
    lab • used at BRANCHING_WORKUP
    A. fumigatus sputum growth (supportive, non-specific); distinguishes airway colonisation; Pseudomonas in established bronchiectasis (Agarwal PMID 23889240)
  • anca
    lab • used at DIFFERENTIAL
    Differential — ANCA + multisystem + eosinophilia >1500 favours EGPA over ABPA (differential-as-data)

12-phase flow (12)

  1. 1FRAME
    Confirm the ABPA scope: a predisposing condition (asthma OR CF) is OBLIGATORY — without asthma/CF this is not ABPA (consider chronic/invasive pulmonary aspergillosis, EGPA, eosinophilic pneumonia instead) (ISHAM-ABPA, Agarwal PMID 23889240)
    inputs: asthma_or_cf_status
    advance: Predisposing asthma or CF confirmed; otherwise route to the appropriate non-ABPA engine
  2. 2ENTRY
    Trigger from poorly-controlled/steroid-dependent asthma, CF deterioration, brownish mucus plugs, fleeting infiltrates, unexplained eosinophilia, or markedly elevated total IgE (Agarwal review PMID 38154470)
    inputs: age
    advance: ABPA suspicion trigger captured
  3. 3CONTEXT
    Prior ABPA stage, steroid history (eosinophils must be steroid-naïve), pregnancy (azole TERATOGENIC — contraindicated), current meds (azole CYP3A4 interactions: statins/DOACs/corticosteroid potentiation), hepatic status (Wark Cochrane PMID 15266440; ISHAM PMID 23889240)
    inputs: prior_abpa_stage, steroid_history, pregnancy_status, current_meds
    actions: calc.act
    advance: Stage + steroid history + azole-safety context (pregnancy/hepatic/interactions) documented
  4. 4RED_FLAGS
    Massive hemoptysis from ABPA bronchiectasis (>200 mL/24 h); severe ABPA exacerbation with respiratory compromise; azole hepatotoxicity (transaminitis/jaundice) → stop azole (Wark Cochrane PMID 15266440)
    inputs: lft
    actions: workup.hemoptysis
    advance: No life-threatening hemoptysis/respiratory failure/hepatotoxicity OR escalated to inpatient pathway
  5. 5INITIAL_WORKUP
    ISHAM criteria battery — total IgE (obligatory gate >500, classically >1000 IU/mL) + A. fumigatus-specific IgE/skin test (obligatory) + A. fumigatus IgG/precipitins (supportive) + absolute eosinophils >500 steroid-naïve (supportive) + HRCT chest (fleeting infiltrates / central bronchiectasis / high-attenuation mucus — supportive radiology) (Agarwal PMID 23889240; PMID 38154470)
    inputs: total_ige, asp_specific_ige, asp_specific_igg, absolute_eosinophils, hrct_chest
    actions: panel.cbc
    advance: ISHAM criteria battery complete enough to apply the diagnostic bundle
  6. 6BRANCHING_WORKUP
    Apply the ISHAM Bayesian bundle: predisposing + 2 obligatory + ≥2 supportive = ABPA. Substage by HRCT — serologic-ABPA (no bronchiectasis) vs ABPA-CB (central bronchiectasis) vs ABPA-CB-ORF (other radiologic findings/fibrosis). If cavitation/aspergilloma/weight-loss → branch to chronic/invasive pulmonary aspergillosis (Agarwal PMID 23889240; PMID 38154470)
    inputs: hrct_chest, sputum_culture
    actions: workup.bronchiectasis_exac
    advance: ABPA confirmed + substaged, OR routed to invasive/chronic pulmonary aspergillosis or another differential
  7. 7DIFFERENTIAL
    §5.5.2 differential-as-data — ABPA vs severe eosinophilic asthma (no Asp-sIgE/IgG, IgE not markedly ↑, no central bronchiectasis) vs chronic eosinophilic pneumonia (peripheral photographic-negative infiltrates, no Asp serology) vs EGPA (ANCA, eos >1500, multisystem/neuropathy) vs CF (sweat chloride/CFTR — CF-ABPA is a distinct branch) vs invasive/chronic pulmonary aspergillosis (immunocompromise/cavitation/aspergilloma) vs other bronchiectasis (no Asp serology, no IgE gate) — IgE/IgG/eosinophil/HRCT pivots with test characteristics (Agarwal PMID 23889240)
    inputs: anca, asp_specific_igg, absolute_eosinophils
    advance: Competing diagnoses excluded by discriminator; ABPA vs ABPM spectrum assigned
  8. 8RISK_STRATIFICATION
    ISHAM/Patterson staging — Stage 0 asymptomatic, I acute, II response, III exacerbation, IV remission, V glucocorticoid-dependent, VI end-stage fibrotic; serologic-ABPA vs ABPA-CB vs ABPA-CB-ORF (Agarwal PMID 23889240). Lung-function (FEV1) and total-IgE level set treatment intensity
    inputs: spirometry, total_ige
    actions: calc.act
    advance: ABPA stage + radiologic subtype + lung-function tier assigned
  9. 9TREATMENT
    Oral corticosteroid induction (medium-dose prednisolone — Agarwal RCT PMID 26585431: medium ≈ high efficacy, safer; taper over months) + azole steroid-sparing (itraconazole/voriconazole + MANDATORY TDM + CYP3A4 interaction map — Stevens NEJM 2000 PMID 10717010; Wark Cochrane PMID 15266440; combination trend Agarwal PMID 34503983) + biologic steroid-sparing for GC-dependent (omalizumab/mepolizumab/dupilumab — Eraso PMID 33050821) + nebulised amphotericin B for recurrent exacerbations (Ram PMID 26666774) + bronchiectasis airway clearance
    inputs: pregnancy_status, lft, total_ige
    advance: Stage-directed regimen (steroid ± azole ± biologic) + taper plan + airway clearance documented
  10. 10DISPOSITION
    Outpatient management for stable/responding ABPA; admit for massive hemoptysis, severe exacerbation with respiratory compromise, or azole hepatotoxicity; refer to pulmonology/severe-asthma or CF clinic for glucocorticoid-dependent / end-stage disease (Agarwal PMID 38154470)
    advance: Disposition + specialist referral set
  11. 11MONITORING
    Total IgE trend (baseline then q6–8 wk: 25–35% fall = response, ≥50% rise over the patient baseline = serologic exacerbation — do NOT chase IgE to normal — Agarwal PMID 34503983 ~47.6% decline at response); azole trough TDM + LFTs on therapy; serial HRCT for bronchiectasis progression; eosinophils + spirometry (Wark Cochrane PMID 15266440; Agarwal PMID 23889240)
    inputs: total_ige, azole_trough_level, lft
    actions: panel.lft
    advance: IgE trend + azole TDM/LFT + radiologic surveillance plan scheduled
  12. 12FOLLOWUP
    Relapse surveillance (clinical + total-IgE + radiology); complete the corticosteroid taper; long-term bronchiectasis care (airway clearance, exacerbation plan, anti-pseudomonal if colonised); biologic step-down only in sustained remission; vaccination; environmental Aspergillus exposure counselling (Agarwal PMID 38154470; PMID 23889240)
    actions: workup.chronic_cough
    advance: Relapse plan + taper completion + long-term bronchiectasis care documented