Bronchiectasis (non-CF — chronic + exacerbation)
NEW dossier authored design-disease-first 2026-05-16 (pulm.bronchiectasis.core.v1 did not previously exist). Structure copied from pulm.copd.core.v1 (dual-acuity chronic + exacerbation regimen-builder template with 4 setting playbooks) and pulm.pe.core.v1 (Bayesian / differential-as-data). status=PLANNED (not yet registered in _registry.ts — out of scope; dossier-contract.test.ts only exercises ALL_DOSSIERS). PRIMARY GUIDELINE = ERS 2025 CPG for adult bronchiectasis (Chalmers Eur Respir J 2025; PMID 41016738; DOI 10.1183/13993003.01126-2025) — supersedes/extends BTS 2019; both verified via PubMed get_article_metadata 2026-05-16. All 13 evidence.pmids PubMed-verified (title+journal+DOI); wrong-article search hits (e.g., 24176443 antiarrhythmics, 30683558 beach nourishment, 39167464 motivated reasoning, 28889109 exercise PH letter, 39450955 microglia) were identified and CULLED — none retained. §5.5.1 ≥10 effect-size numbers (delivered ~13) PMID-anchored in regimen rationale + severity_triggers + depth.md §3: BAT azithromycin ≥1-exac HR 0.29 / median exac 0 vs 2 (23532241); EMBRACE event-based exac RR 0.38 (22901887); BLESS erythromycin IRR 0.57 + post-BD FEV1 +2.2% (23532242); BSI AUC mortality 0.80 / hospitalisation 0.88 (24328736); FACED 7-point 5-yr-mortality score (24232697); Pseudomonas mortality OR 2.95 / hospitalisation OR 6.57 / +0.97 exac/yr / SGRQ +18.2 (26356317); 7% hypertonic saline FEV1 +15.1% / SGRQ −6.0 / antibiotic courses 2.4 vs 5.4 (22018993); inhaled-antibiotic exac RR 0.72 / eradication RR 4.2 / sputum −2.65 log10 / bronchospasm ~10% (24925920); brensocatib ASPEN rate ratio 0.79-0.81 / time-to-first HR 0.81 / FEV1 −24 vs −62 mL (40267423); EMBARC sputum-colour exac IRR up to 1.91 / mortality HR 1.12 per increment (38609095); BEST diary MCID 4 / mean exac duration 15.3 d (31931782). §5.5.2 differential/Bayesian as DATA: bronchiectasis vs COPD vs asthma vs CF vs NTM vs ABPA vs post-TB vs PCD vs IPF — HRCT morphology (signet-ring/tram-track/lack-of-tapering) + the ERS/BTS minimum etiologic bundle as pivots with test characteristics; wired via severity_triggers (etiologic_bundle_decision, differential_bronchiectasis_vs_mimics, ntm_isolated_macrolide_gate), DIFFERENTIAL/RISK_STRATIFICATION phase logic, workup.bronchiectasis_exac branches_to, and 4 sibling_differentiation blocks. Cross-dossier engine_ids (all REAL, grep-confirmed): pulm.copd.core.v1, pulm.asthma.core.v1, pulm.tuberculosis.v1, pulm.idiopathic_pulmonary_fibrosis.v1, pulm.pcp-pneumonia.core.v1, pulm.cap.core.v1. CF / NTM-pulmonary-disease / ABPA engines DO NOT exist → encoded as differential-as-data only, NEVER referenced in branches_to / sibling_engine_id. Regimen axis (chronic foundation + escalation + exacerbation, ≥6 special-pop branches as DATA): airway-clearance + mucoactive (hypertonic saline 9863, carbocysteine 2023) foundation → Pseudomonas eradication (ciprofloxacin 2551) → inhaled-antibiotic suppression (colistin 2709 / tobramycin 10627) → macrolide maintenance GATED (azithromycin 18631 / erythromycin 4053; eligibility = ≥3 exac/yr + NTM-negative + QTc + audiogram) → brensocatib (no rxcui) → 14-day exacerbation antibiotics (amoxicillin-clavulanate 19711 / doxycycline 3640). Branches: Pseudomonas-colonised, NTM-positive (macrolide MONOTHERAPY CONTRAINDICATED — hard gate), immunodeficiency (Ig replacement — non_pharm), ABPA-driven (prednisolone 8638 + itraconazole 28031 ± omalizumab 302379), pregnancy (ACT + hypertonic saline only), frequent-exacerbator/CTD-IBD. Contraindications encoded in contraindication_rules + RegimenDrug.triggers. RxCUI VALIDATION (RxNav REST 2026-05-16, NONE hand-authored to SCD/SBD): azithromycin 18631 (IN ✓), erythromycin 4053 (IN ✓), amoxicillin/clavulanate 19711 (MIN ✓), carbocysteine 2023 (IN ✓), tobramycin 10627 (IN ✓), colistin 2709 (IN ✓), ciprofloxacin 2551 (IN ✓), doxycycline 3640 (IN ✓), sodium chloride [hypertonic saline] 9863, itraconazole 28031 (IN ✓), prednisolone 8638 (IN ✓), omalizumab 302379 (IN ✓). brensocatib — NO stable RxNorm ingredient CUI at authoring (newly FDA-pathway DPP-1 inhibitor) → rxcui OMITTED + flagged, NOT hand-authored. Ig replacement / treat-CTD-IBD / NTM-gate / pregnancy-branch are non_pharm. SCHEMA-GAP NOTES: (1) _types.ts has no first-class Bayesian-LR / pretest-prevalence / decision-threshold / etiologic-bundle / effect-size / special-population-matrix field — encoded in severity_triggers, phase purpose/advance_when, calculator guideline_basis, regimen rationale/triggers/contraindication_rules, and depth.md tables; (2) NO BSI or FACED calculator id exists in clinical-tools-registry.ts — encoded as severity DATA + RISK_STRATIFICATION phase logic + notes (calc.mmrc/calc.bode used as nearest registry surrogates for the dyspnea/multidimensional axes, NOT as BSI/FACED themselves; no invented unresolved ids); (3) RequiredCalculator.drives enum lacks diagnostic_gate/etiologic_bundle — reuses severity_classification/risk_stratification/disposition; (4) brensocatib lacks a RegimenDrug field for investigational/just-approved status — noted here. NEW-DOSSIER NEXT STEPS (out of scope this session, listed for the audit): (1) author a dedicated prisma/seed/manifests/pulm.bronchiectasis.core.v1.ts manifest (currently repointed to id.sepsis.core.v1.ts per the id.dengue pattern); (2) register in src/lib/dossiers/_registry.ts so dossier-contract.test.ts + dossier:audit exercise it; (3) build a problem-package + _design-brief under src/lib/tier3/...; (4) add a dedicated BSI/FACED calculator to clinical-tools-registry.ts and a bronchiectasis-specific test file; (5) resolve a brensocatib RxNorm CUI once RxNav indexes it; (6) terminology.icd10/snomed/loinc are best-effort author-curated (J47.x bronchiectasis, Q33.4 congenital, B44.81 ABPA, D80.1/D83.9 antibody deficiency, A15.0 pulmonary TB, M05.79 RA-with-lung) — backfill via the terminology pipeline before any PRODUCTION promotion. DEPTH-PASS-2 2026-05-18 (shard-07-cardio-chronic, terminal CL-4; non-CF bronchiectasis deepen): (1) co-located pulm.bronchiectasis.core.v1._design-brief.md + ._research-bundle.md authored per §5.5 items 1+2 mirroring the cardio.htn.core.v1 golden template + pulm.copd.core.v1 same-domain exemplar; design_brief: repointed from the non-existent _briefs/ path to the co-located ._design-brief.md. (2) Live PubMed MCP research (search_articles ×11, get_article_metadata ×4 batches): 20 verified PMIDs with effect sizes + 95% CI, retrieval 2026-05-18; evidence.pmids 13→20 — ADDED BTS-2019-main (30545985, was only the primary-care 31249313), WILLOW phase-2 (32897034 — HR 0.58 [0.35–0.95]), Haworth inhaled-colistin RCT (24625200 — adherent 168 vs 103 d P=0.038), PROMIS-I/II phase-3 (39270696 — PROMIS-I rate ratio 0.61 [0.46–0.82]), RESPIRE design (28495619), Kapur ICS-not-routine Cochrane (29766487 — FEV1 MD −0.09 NS), Cox pulm-rehab Cochrane (24731015). (3) ASPEN effect size made precise/dose-resolved: rate ratio 0.79 (0.68–0.92, 10 mg) / 0.81 (0.69–0.94, 25 mg), FEV1 decline 50/24/62 mL with 25-mg-ONLY LS-mean +38 mL (11–65, P=0.04) — corrected the prior "−24 vs −62 mL" wording. (4) Bayesian seed prisma/seed/ros-and-ddx/pulm.bronchiectasis.core.v1.{differentials,ros,finding-lrs}.ts authored mirroring htn Prisma shapes exactly (ENGINE_ID pulm.bronchiectasis.core.v1): 11 differentials w/ cohort-anchored priors (idiopathic/post-infective/COPD/asthma/ABPA/NTM/post-TB/immunodeficiency/CTD-IBD/CF[scope]/PCD + BSI severity strata MECE), 13 ROS items, 36 LR rows = 25 LR+ / 25 LR− (HRCT signet-ring/tram-track diagnostic pivot, chronic mucopurulent sputum, recurrent exac ≥3/yr, Pa colonisation, BSI severe/mild bands, NTM culture, ↑↑IgE+Aspergillus ABPA), 4 conditional-dependency rules (HRCT supersedes clinical-pretest surrogates; Pa-LR conditional on prior-culture history; Aspergillus-sensitisation conditional on ↑↑total-IgE gate; BSI bands mutually exclusive), T_test≈5% / T_treat≈20% (etiologic bundle) + ≥3-exac/yr suppression threshold header. (5) 2nd regimen axis bronchiectasis_phenotype_matrix ADDED — drug × pathogen/phenotype gating as DATA (Pseudomonas-colonised / frequent-exacerbator≥3-yr / ABPA-overlap / COPD-asthma-overlap-ICS-only-then / NTM-positive-defer-macrolide / immunodeficiency-Ig / pregnancy), mirroring golden-template htn_comorbidity_phenotype 2nd axis; regimen_axes 1→2 (axis_id count ≥2 confirmed). (6) RxCUI re-validation (RxNav REST 2026-05-18 + research:rxnav:validate): all 13 CUIs confirmed (azithromycin 18631/erythromycin 4053/colistin 2709[RxNav rxcui.json?name=colistin resolves to 2709, IN]/tobramycin 10627/ciprofloxacin 2551/sodium-chloride 9863/carbocysteine 2023/itraconazole 28031/prednisolone 8638/omalizumab 302379/amoxicillin-clav 19711[MIN]/doxycycline 3640) — NO bugs; the DrugEffectProfile colistin.ts canonical (2618 colistimethate-sodium prodrug) is validator-NOT_FOUND but the dossier 2709 is the correct ingredient CUI (logged in bundle §4). brensocatib NO RxNorm CUI → rxcui omitted, non_pharm:false, flagged NEEDS_RXNAV_VALIDATION, NOT hand-authored. Counts: 20 verified PMIDs, ~20 effect sizes w/ 95% CI, 25 LR+, 25 LR− (36 LR rows), 11 differentials, 13 ROS, 13 RxCUIs, 2 regimen axes. status: PLANNED kept (registry-registration out of scope per dispatch). 96-fail drug-coverage baseline OUT OF SCOPE per dispatch. DEPTH-PASS-3 2026-05-26 (lane-E): +NMA +USPSTF +Cochrane +ICER stubs +decision thresholds, side-car at pulm.bronchiectasis.core.v1._depth-pass-3.md.
Entry points (5)
- symptomChronic productive cough >8 weeks ± recurrent lower respiratory tract infection (ERS 2025; BTS 2019)chronic_productive_cough
- imagingHRCT showing bronchoarterial ratio >1 / signet-ring, tram-track, lack of tapering, ballooning (ERS 2025 — diagnostic reference standard)hrct_bronchiectasis
- symptomAcute exacerbation: deterioration in ≥3 of cough/sputum volume/sputum purulence/dyspnea/fatigue/haemoptysis ≥48 h (EMBARC/ERS 2025 exacerbation definition)acute_exacerbation
- problem_listExisting bronchiectasis — annual review, severity re-stratification, or post-exacerbation visit (ERS 2025)bronchiectasis_existing
- symptomHaemoptysis (a frequent presenting feature; massive haemoptysis = RED_FLAG)haemoptysis
Required inputs (20)
- agerequireddemographic • used at CONTEXTFACED/BSI age component (cut-off 70 yr); alpha-1 + PCD pretest higher if young (ERS 2025; FACED PMID 24232697)
- hrct_chestrequiredimaging • used at INITIAL_WORKUPHRCT is the diagnostic reference standard (bronchoarterial ratio >1, lack of tapering, signet-ring); also defines radiological extent (lobes) for FACED/BSI (ERS 2025; BSI PMID 24328736)
- spirometryrequiredimaging • used at INITIAL_WORKUPFEV1 % predicted — BSI (<30%) and FACED (<50%) components + monitoring trend (ERS 2025; FACED PMID 24232697; BSI PMID 24328736)
- sputum_culturerequiredlab • used at INITIAL_WORKUPRoutine bacterial culture identifies Pseudomonas / other pathogenic organisms — prognosis multiplier + directs eradication/suppression and exacerbation antibiotics (Finch PMID 26356317; ERS 2025)
- sputum_mycobacterial_culturerequiredlab • used at INITIAL_WORKUPNTM culture — MANDATORY before macrolide maintenance (macrolide monotherapy in unrecognised NTM drives resistance); part of the etiologic bundle (ERS 2025)
- immunoglobulinsrequiredlab • used at INITIAL_WORKUPSerum IgG/IgA/IgM ± specific antibody — immunodeficiency is a treatable cause (Ig replacement); ERS/BTS minimum etiologic bundle (ERS 2025; BTS 2019)
- total_ige_aspergillusrequiredlab • used at INITIAL_WORKUPTotal IgE + Aspergillus-specific IgE/IgG — ABPA screen (changes regimen to steroid ± antifungal); etiologic bundle (ERS 2025)
- blood_eosinophilslab • used at INITIAL_WORKUPEosinophilia supports ABPA / eosinophilic-bronchiectasis phenotype; informs ICS/biologic consideration (ERS 2025 — emerging eosinophilic endotype)
- alpha1_antitrypsinlab • used at INITIAL_WORKUPAAT deficiency screen — part of the etiologic bundle (ERS 2025; BTS 2019)
- exacerbations_12morequiredhistory • used at CONTEXTDrives macrolide-maintenance / inhaled-antibiotic / brensocatib eligibility (≥3 exac/yr) + BSI/FACED prognosis (ERS 2025; BAT PMID 23532241; ASPEN PMID 40267423)
- prior_pseudomonasrequiredhistory • used at CONTEXTPrior Pseudomonas isolation → first-isolation eradication window vs established chronic infection → suppression; antipseudomonal exacerbation cover (Finch PMID 26356317; ERS 2025)
- mrc_dyspnearequiredhistory • used at CONTEXTMRC dyspnea ≥II is a FACED component and ≥4 a BSI component (FACED PMID 24232697; BSI PMID 24328736)
- bmidemographic • used at CONTEXTLow BMI is an independent BSI mortality predictor (BSI PMID 24328736)
- prior_hospitalisationhistory • used at CONTEXTPrior hospital admission is a strong BSI predictor of future hospitalisation/mortality (BSI PMID 24328736)
- current_medsrequiredmedication • used at CONTEXTCurrent airway-clearance/mucoactive/macrolide/inhaled-antibiotic regimen for stepwise titration; flag QTc-prolonging co-medication before macrolide (ERS 2025)
- qtc_or_ototoxicity_riskhistory • used at CONTEXTBaseline QTc + audiogram gate macrolide maintenance (azithromycin QT/ototoxicity; BAT PMID 23532241)
- pregnancyhistory • used at CONTEXTPregnancy branch — avoid chronic azithromycin/quinolone/itraconazole; ACT + hypertonic saline remain safe (ERS 2025 special populations)
- connective_tissue_or_ibdhistory • used at CONTEXTRA / IBD / other CTD as an etiology — treating the underlying disease is part of management (ERS 2025 etiologic bundle)
- haemoptysis_volumesymptom • used at RED_FLAGSMassive haemoptysis → bronchial artery embolisation / IR — RED_FLAG escalation (ERS 2025)
- spo2vital • used at CONTEXTSevere-exacerbation respiratory-failure assessment + advanced-disease oxygen need (ERS 2025)
12-phase flow (12)
- 1FRAMEConfirm chronic structural disease on HRCT (bronchoarterial ratio >1, lack of tapering) in an adult; scope = non-CF; flag whether an exacerbation overlay is active now (ERS 2025)inputs: hrct_chestadvance: HRCT-confirmed non-CF bronchiectasis established
- 2ENTRYTriggered by chronic productive cough >8 wk + recurrent LRTI, incidental HRCT bronchiectasis, or an acute exacerbation in known disease (ERS 2025; BTS 2019)inputs: ageadvance: Entry trigger identified
- 3CONTEXTExacerbations/12mo, sputum microbiology history (esp. Pseudomonas/NTM), prior etiologic workup, comorbidity (COPD/asthma/RA/IBD/immunodeficiency), MRC dyspnea, BMI, prior hospitalisation, QTc/ototoxicity + pregnancy status before any macrolide (ERS 2025)inputs: exacerbations_12mo, prior_pseudomonas, mrc_dyspnea, bmi, prior_hospitalisation, current_meds, qtc_or_ototoxicity_risk, pregnancy, connective_tissue_or_ibd, spo2advance: Exacerbation burden + microbiology + comorbidity + macrolide-safety sweep complete
- 4RED_FLAGSMassive haemoptysis → bronchial artery embolisation / IR + airway protection; severe exacerbation with respiratory failure; first Pseudomonas isolation (eradication window); decompensated cor pulmonale (ERS 2025)inputs: haemoptysis_volume, spo2actions: workup.bronchiectasis_exacadvance: No emergent escalation needed OR haemoptysis/respiratory-failure pathway initiated
- 5INITIAL_WORKUPHRCT (reference standard) + spirometry + sputum bacterial AND mycobacterial culture + the ERS/BTS minimum etiologic bundle (immunoglobulins, total IgE + Aspergillus serology, alpha-1 antitrypsin, eosinophils) — because ~30-40% have a treatable/modifying cause (ERS 2025; BTS 2019)inputs: hrct_chest, spirometry, sputum_culture, sputum_mycobacterial_culture, immunoglobulins, total_ige_aspergillus, blood_eosinophils, alpha1_antitrypsinactions: workup.bronchiectasis_exacadvance: Structural confirmation + microbiology + etiologic bundle complete
- 6BRANCHING_WORKUPBy finding: PCD testing (nasal NO / ciliary studies) if young + situs/infertility/neonatal respiratory distress; CTD/IBD serology if articular/GI features; bronchoscopy if focal disease, suspected obstruction, or atypical/persistent organisms; CFTR consideration if young + suggestive (CF differential-as-data — no CF engine) (ERS 2025)inputs: connective_tissue_or_ibdadvance: Secondary etiologic branches resolved or excluded
- 7DIFFERENTIAL§5.5.2 differential as data — bronchiectasis vs COPD (smoking + fixed FEV1/FVC <0.70, emphysema not signet-ring → pulm.copd.core.v1) vs asthma (reversible, atopy, eosinophilic → pulm.asthma.core.v1) vs CF (young, sweat chloride/CFTR, upper-lobe + steatorrhoea — DATA only, no engine) vs NTM (mycobacterial culture positive, tree-in-bud + nodules — DATA + macrolide-monotherapy CONTRAINDICATED) vs ABPA (central bronchiectasis + ↑↑IgE + Aspergillus IgE/IgG + eosinophilia — DATA, treat as regimen branch) vs post-TB (upper-lobe fibrocavitary, endemic/prior TB → pulm.tuberculosis.v1) vs PCD (situs/infertility, nasal NO low — DATA) vs IPF/ILD (traction bronchiectasis on fibrotic UIP background → pulm.idiopathic_pulmonary_fibrosis.v1); each discriminator carries its test characteristic (ERS 2025)inputs: hrct_chest, sputum_mycobacterial_culture, total_ige_aspergillus, immunoglobulinsadvance: Aetiology assigned (or routed) + NTM/CF/ABPA explicitly addressed
- 8RISK_STRATIFICATIONBSI (Chalmers AJRCCM 2014 PMID 24328736 — AUC mortality 0.80, hospitalisation 0.88: age, BMI, FEV1, prior hosp, ≥3 exac, MRC dyspnea, Pseudomonas/other-organism colonisation, ≥3 lobes) and FACED (Martinez-Garcia Eur Respir J 2014 PMID 24232697 — FEV1/Age/Colonisation/Extension/Dyspnea, 5-yr mortality) → low/intermediate/high → suppressive-therapy intensity + follow-up cadence; Pseudomonas colonisation = independent ~3× mortality multiplier (Finch PMID 26356317)inputs: age, spirometry, exacerbations_12mo, prior_pseudomonas, mrc_dyspnea, bmi, prior_hospitalisation, hrct_chestadvance: BSI + FACED tier assigned + Pseudomonas-prognosis flagged
- 9TREATMENTERS 2025: airway clearance technique + mucoactive (nebulised hypertonic saline; carbocisteine) FOUNDATION for all + pulmonary rehab if exercise-limited + vaccines; chronic infection → first-isolation eradication then inhaled-antibiotic suppression; ≥3 exac/yr → azithromycin maintenance (GATED: NTM-negative + QTc + audiogram first); etiology-specific (Ig replacement for immunodeficiency, prednisolone ± itraconazole for ABPA, treat CTD/IBD); exacerbation → 14-day sputum-directed antibiotics; refractory frequent exacerbator → brensocatib (ASPEN PMID 40267423)inputs: sputum_culture, sputum_mycobacterial_culture, exacerbations_12mo, prior_pseudomonas, total_ige_aspergillus, immunoglobulinsadvance: ERS-aligned foundation + phenotype-conditional escalation + exacerbation plan documented
- 10DISPOSITIONOutpatient for most chronic management; ED/ward for severe exacerbation, respiratory failure, or significant haemoptysis; ICU for respiratory failure or massive haemoptysis pending embolisation (ERS 2025)inputs: spo2, haemoptysis_volumeadvance: Disposition set
- 11MONITORINGSerial sputum microbiology (Pseudomonas/NTM emergence), annual spirometry, exacerbation count / BEST symptom diary (Artaraz PMID 31931782), sputum-colour trend as a severity/risk marker (EMBARC Aliberti PMID 38609095), macrolide safety (QTc + audiogram + annual NTM culture), Ig trough if on replacement (ERS 2025)advance: Microbiology + spirometry + exacerbation + drug-safety monitoring scheduled
- 12FOLLOWUPVaccination (influenza, pneumococcal), pulmonary rehabilitation, airway-clearance technique reinforcement + adherence, severity re-stratification (BSI/FACED) at annual review, lung-transplant referral in advanced disease, advance care planning (ERS 2025; BTS 2019)advance: Prevention + rehab + re-stratification + advanced-disease planning complete