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pulm.bronchiectasis.core.v1PRODUCTION
pulm.bronchiectasis.core.v1

Bronchiectasis (non-CF — chronic + exacerbation)

pulmonologychronicacuteadult
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Care setting:

Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Confirm 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)

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Advance rule
Set
Advance when

HRCT-confirmed non-CF bronchiectasis established

Patient inputs (20)

FACED/BSI age component (cut-off 70 yr); alpha-1 + PCD pretest higher if young (ERS 2025; FACED PMID 24232697)

Drives macrolide-maintenance / inhaled-antibiotic / brensocatib eligibility (≥3 exac/yr) + BSI/FACED prognosis (ERS 2025; BAT PMID 23532241; ASPEN PMID 40267423)

Prior Pseudomonas isolation → first-isolation eradication window vs established chronic infection → suppression; antipseudomonal exacerbation cover (Finch PMID 26356317; ERS 2025)

MRC dyspnea ≥II is a FACED component and ≥4 a BSI component (FACED PMID 24232697; BSI PMID 24328736)

Current airway-clearance/mucoactive/macrolide/inhaled-antibiotic regimen for stepwise titration; flag QTc-prolonging co-medication before macrolide (ERS 2025)

HRCT 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)

FEV1 % predicted — BSI (<30%) and FACED (<50%) components + monitoring trend (ERS 2025; FACED PMID 24232697; BSI PMID 24328736)

Routine bacterial culture identifies Pseudomonas / other pathogenic organisms — prognosis multiplier + directs eradication/suppression and exacerbation antibiotics (Finch PMID 26356317; ERS 2025)

NTM culture — MANDATORY before macrolide maintenance (macrolide monotherapy in unrecognised NTM drives resistance); part of the etiologic bundle (ERS 2025)

Serum IgG/IgA/IgM ± specific antibody — immunodeficiency is a treatable cause (Ig replacement); ERS/BTS minimum etiologic bundle (ERS 2025; BTS 2019)

Total IgE + Aspergillus-specific IgE/IgG — ABPA screen (changes regimen to steroid ± antifungal); etiologic bundle (ERS 2025)

Low BMI is an independent BSI mortality predictor (BSI PMID 24328736)

Prior hospital admission is a strong BSI predictor of future hospitalisation/mortality (BSI PMID 24328736)

Baseline QTc + audiogram gate macrolide maintenance (azithromycin QT/ototoxicity; BAT PMID 23532241)

Pregnancy branch — avoid chronic azithromycin/quinolone/itraconazole; ACT + hypertonic saline remain safe (ERS 2025 special populations)

RA / IBD / other CTD as an etiology — treating the underlying disease is part of management (ERS 2025 etiologic bundle)

Severe-exacerbation respiratory-failure assessment + advanced-disease oxygen need (ERS 2025)

Eosinophilia supports ABPA / eosinophilic-bronchiectasis phenotype; informs ICS/biologic consideration (ERS 2025 — emerging eosinophilic endotype)

AAT deficiency screen — part of the etiologic bundle (ERS 2025; BTS 2019)

Massive haemoptysis → bronchial artery embolisation / IR — RED_FLAG escalation (ERS 2025)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (7)

7 need judgement
  • informationallife_threateningmassive_haemoptysis
    Massive haemoptysis (large-volume / haemodynamic or airway compromise) in known bronchiectasis (ERS 2025)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverentm_isolated_macrolide_gate
    Non-tuberculous mycobacteria isolated on mycobacterial culture in a patient on or being considered for macrolide maintenance (ERS 2025)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresevere_exacerbation_respiratory_failure
    Severe exacerbation with respiratory failure / haemodynamic compromise (ERS 2025)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatefirst_pseudomonas_isolation
    First isolation of Pseudomonas aeruginosa on sputum culture (not yet chronic) (ERS 2025; Finch PMID 26356317)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatefrequent_exacerbator_high_BSI_FACED
    ≥3 exacerbations/yr OR high BSI/FACED tier — eligible for suppressive escalation (inhaled antibiotic / macrolide / brensocatib) (ERS 2025; BSI PMID 24328736; FACED PMID 24232697)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateetiologic_bundle_decision
    New bronchiectasis diagnosis — the ERS/BTS minimum etiologic bundle drives a treatable-cause decision chain: immunoglobulins↓ → Ig replacement; total IgE↑↑ + Aspergillus IgE/IgG+ + eosinophilia → ABPA (steroid ± antifungal); mycobacterial culture+ → NTM (macrolide-monotherapy CONTRAINDICATED); AAT↓ → alpha-1; PCD/CTD/IBD features → targeted testing (ERS 2025; BTS 2019)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatedifferential_bronchiectasis_vs_mimics
    Bronchiectasis vs mimics — §5.5.2 pivots: smoking + fixed post-BD FEV1/FVC <0.70 + emphysema (not signet-ring) → COPD (pulm.copd.core.v1); reversible airflow + atopy + eosinophilia → asthma (pulm.asthma.core.v1); upper-lobe fibrocavitary + endemic/prior TB → post-TB (pulm.tuberculosis.v1); traction bronchiectasis on a fibrotic UIP background → IPF/ILD (pulm.idiopathic_pulmonary_fibrosis.v1); recurrent infection in antibody deficiency → PCP/immunodeficiency (pulm.pcp-pneumonia.core.v1); young + sweat-chloride/CFTR + steatorrhoea → CF (DATA only — no CF engine); mycobacterial culture+ → NTM (DATA only — no NTM engine); central bronchiectasis + ↑↑IgE → ABPA (regimen branch — no ABPA engine) (ERS 2025)
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

Run this disease's risk and dosing calculators inline.

RISK_STRATIFICATIONoptionalDrives severity classification
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Recommended regimen

ERS 2025 — airway-clearance foundation + phenotype escalation + exacerbation
axis: bronchiectasis_chronic_plus_exacerbationstep 1 - Foundation — all patients
Selected step "Foundation — all patients" — Any confirmed non-CF bronchiectasis (ERS 2025 strong recommendation for airway clearance for most patients)
  • airway clearance technique (ACBT / ELTGOL / oscillatory PEP)
    first line
    airway_clearance
    Daily, ≥1-2 sessions/day, taught by respiratory physiotherapy • n/a • daily
    ERS 2025 STRONG recommendation — airway clearance for most patients with bronchiectasis (sputum retention drives the vicious vortex); ELTGOL has the best comparative trial evidence
  • nebulised hypertonic saline (6-7% sodium chloride)
    first line
    mucoactive_osmotic
    4 mL of 6-7% nebulised (pre-treat with bronchodilator + tolerance test for bronchospasm) • nebulised • BID
    Kellett Respir Med 2011 (PMID 22018993) — 7% HS improved FEV1 +15.1% and SGRQ −6.0 vs isotonic, with fewer antibiotic courses (2.4 vs 5.4/pt-yr); ERS 2025 conditional mucoactive recommendation
    rxcui 9863
  • carbocysteine
    add on
    mucoactive_mucolytic
    750 mg • PO • TID (then taper to 750 mg BID)
    triggers: mucus_hypersecretion, hypertonic_saline_not_tolerated
    Oral mucolytic alternative/adjunct where nebulised hypertonic saline is not tolerated (ERS 2025 conditional mucoactive)
    rxcui 2023

outpatient playbook — drug actions (6)

  1. 1. airway clearance technique + nebulised hypertonic saline
    ACT ≥1-2×/day; 6-7% saline 4 mL nebulised BID (tolerance-tested) • inhaled/physio • daily / BID
    trigger: All confirmed bronchiectasis
    ERS 2025 foundation; Kellett PMID 22018993
  2. 2. Pseudomonas first-isolation eradication (ciprofloxacin ± inhaled antibiotic)
    Cipro 500-750 mg PO BID × 14 d ± inhaled colistin/tobramycin 1-3 mo • PO/inhaled • 14 d course + suppression
    trigger: First Pseudomonas isolation
    ERS 2025; Finch PMID 26356317
  3. 3. inhaled-antibiotic suppression (colistin or tobramycin)
    Colistin 1-2 MIU neb BID OR tobramycin 300 mg neb BID (28-on/28-off) • inhaled • long-term
    trigger: Chronic Pseudomonas + high exacerbation risk
    ERS 2025 strong rec; Brodt PMID 24925920 exac RR 0.72
  4. 4. azithromycin maintenance (GATED)
    250 mg PO 3×/week • PO • 3×/week
    trigger: ≥3 exacerbations/yr AND NTM-negative AND acceptable QTc
    BAT PMID 23532241 HR 0.29; EMBRACE PMID 22901887 RR 0.38
  5. 5. brensocatib (refractory frequent exacerbator)
    10 mg (or 25 mg) PO once daily • PO • once daily
    trigger: Persistent exacerbations despite optimised therapy
    ASPEN PMID 40267423 rate ratio 0.79
  6. 6. etiology-specific (Ig replacement / ABPA steroid ± antifungal / treat CTD-IBD)
    Per branch (IVIG ~0.4-0.6 g/kg q3-4 wk; prednisolone 0.5 mg/kg/d taper ± itraconazole 200 mg BID) • IV/SC/PO • per branch
    trigger: Etiologic bundle identifies treatable cause
    ERS 2025 etiologic bundle — disease-modifying

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Chronic productive cough >8 weeks ± recurrent lower respiratory tract infection (ERS 2025; BTS 2019); HRCT showing bronchoarterial ratio >1 / signet-ring, tram-track, lack of tapering, ballooning (ERS 2025 — diagnostic reference standard); Acute exacerbation: deterioration in ≥3 of cough/sputum volume/sputum purulence/dyspnea/fatigue/haemoptysis ≥48 h (EMBARC/ERS 2025 exacerbation definition).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Bronchiectasis (non-CF — chronic + exacerbation)** (pulm.bronchiectasis.core.v1).
Phenotype framing: §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)
Scope: Confirm 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)

No severity triggers fired against current inputs.

Plan

Regimen axis: **ERS 2025 — airway-clearance foundation + phenotype escalation + exacerbation** — step "Foundation — all patients".
1. airway clearance technique (ACBT / ELTGOL / oscillatory PEP) Daily, ≥1-2 sessions/day, taught by respiratory physiotherapy n/a daily (airway_clearance, first line) — ERS 2025 STRONG recommendation — airway clearance for most patients with bronchiectasis (sputum retention drives the vicious vortex); ELTGOL has the best comparative trial evidence
2. nebulised hypertonic saline (6-7% sodium chloride) 4 mL of 6-7% nebulised (pre-treat with bronchodilator + tolerance test for bronchospasm) nebulised BID (mucoactive_osmotic, first line) — Kellett Respir Med 2011 (PMID 22018993) — 7% HS improved FEV1 +15.1% and SGRQ −6.0 vs isotonic, with fewer antibiotic courses (2.4 vs 5.4/pt-yr); ERS 2025 conditional mucoactive recommendation
3. carbocysteine 750 mg PO TID (then taper to 750 mg BID) (mucoactive_mucolytic, add on) — Oral mucolytic alternative/adjunct where nebulised hypertonic saline is not tolerated (ERS 2025 conditional mucoactive)

Setting playbook (outpatient) — Confirm diagnosis + complete the ERS/BTS etiologic bundle, stratify with BSI/FACED, and maintain the airway-clearance + mucoactive foundation with phenotype-conditional escalation to reduce exacerbations and slow decline (ERS 2025)
4. airway clearance technique + nebulised hypertonic saline ACT ≥1-2×/day; 6-7% saline 4 mL nebulised BID (tolerance-tested) inhaled/physio daily / BID — All confirmed bronchiectasis (ERS 2025 foundation; Kellett PMID 22018993)
5. Pseudomonas first-isolation eradication (ciprofloxacin ± inhaled antibiotic) Cipro 500-750 mg PO BID × 14 d ± inhaled colistin/tobramycin 1-3 mo PO/inhaled 14 d course + suppression — First Pseudomonas isolation (ERS 2025; Finch PMID 26356317)
6. inhaled-antibiotic suppression (colistin or tobramycin) Colistin 1-2 MIU neb BID OR tobramycin 300 mg neb BID (28-on/28-off) inhaled long-term — Chronic Pseudomonas + high exacerbation risk (ERS 2025 strong rec; Brodt PMID 24925920 exac RR 0.72)
7. azithromycin maintenance (GATED) 250 mg PO 3×/week PO 3×/week — ≥3 exacerbations/yr AND NTM-negative AND acceptable QTc (BAT PMID 23532241 HR 0.29; EMBRACE PMID 22901887 RR 0.38)
8. brensocatib (refractory frequent exacerbator) 10 mg (or 25 mg) PO once daily PO once daily — Persistent exacerbations despite optimised therapy (ASPEN PMID 40267423 rate ratio 0.79)
9. etiology-specific (Ig replacement / ABPA steroid ± antifungal / treat CTD-IBD) Per branch (IVIG ~0.4-0.6 g/kg q3-4 wk; prednisolone 0.5 mg/kg/d taper ± itraconazole 200 mg BID) IV/SC/PO per branch — Etiologic bundle identifies treatable cause (ERS 2025 etiologic bundle — disease-modifying)

Non-pharmacologic actions:
- Pulmonary rehabilitation if exercise capacity impaired (ERS 2025 strong recommendation)
- Airway-clearance technique training + reinforcement by respiratory physiotherapy (ERS 2025)
- Vaccination — influenza + pneumococcal (ERS 2025)
- Smoking cessation if applicable (general)
- Self-management / written exacerbation action plan (BTS 2019)

AVOID / contraindication checks:
- Macrolide_monotherapy_CONTRAINDICATED_if_NTM_isolated_exclude_NTM_before_and_during_maintenance (ERS 2025; BAT PMID 23532241 macrolide resistance 88% vs 26%)
- Baseline_QTc_and_audiogram_before_long_term_macrolide_and_periodically (ERS 2025; BAT PMID 23532241)
- ICS_NOT_routine_in_bronchiectasis_reserve_for_concomitant_asthma_or_ABPA (ERS 2025 — suggest not to routinely use inhaled corticosteroids)
- Long_term_oral_non_macrolide_antibiotics_NOT_recommended_routinely (ERS 2025 conditional against)
- Inhaled_antibiotic_first_dose_supervised_bronchospasm_risk_pretreat_bronchodilator (Brodt PMID 24925920 — bronchospasm ~10%)
- Exacerbation_antibiotic_course_is_14_days_in_bronchiectasis_not_5_days_like_COPD (ERS 2025)
- Attempt_Pseudomonas_eradication_at_first_isolation_before_chronic_infection_established (ERS 2025; Finch PMID 26356317 — Pseudomonas mortality OR 2.95)
- Pregnancy_avoid_chronic_azithromycin_fluoroquinolone_itraconazole_continue_ACT_and_hypertonic_saline (ERS 2025 special populations)
- Itraconazole_teratogenic_avoid_in_pregnancy_monitor_LFTs_and_drug_levels (ERS 2025)
- Brensocatib_monitor_hyperkeratosis_and_dental_periodontal_effects (ASPEN PMID 40267423)

Monitoring

Regimen monitoring:
- serial sputum culture bacterial and mycobacterial (ERS 2025 — Pseudomonas/NTM emergence; NTM gate for ongoing macrolide)
- annual spirometry FEV1 trend (ERS 2025; BSI/FACED FEV1 component)
- exacerbation count or BEST symptom diary (Artaraz Respir Res 2020 PMID 31931782 — MCID 4 points)
- sputum colour trend as severity and risk marker (EMBARC Aliberti Eur Respir J 2024 PMID 38609095 — purulent sputum exacerbation IRR up to 1.91, mortality HR 1.12/increment)
- QTc and audiogram periodically on long term macrolide (ERS 2025; BAT PMID 23532241)
- IgG trough if on immunoglobulin replacement (ERS 2025 antibody-deficiency branch)
- BSI and FACED re stratification at annual review (Chalmers PMID 24328736; Martinez-Garcia PMID 24232697)
- LFTs and itraconazole levels if on antifungal for ABPA (ERS 2025)

Setting (outpatient) monitoring:
- Sputum bacterial + mycobacterial culture serially (Pseudomonas/NTM)
- Annual spirometry (ERS 2025)
- Exacerbation diary / BEST score (Artaraz PMID 31931782)
- QTc + audiogram periodically on macrolide (BAT PMID 23532241)
- BSI/FACED re-stratification at annual review

Follow-up plan: Vaccination (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)
- Close-out criterion: Prevention + rehab + re-stratification + advanced-disease planning complete

Monitoring phase: Serial 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)

Disposition

Current setting: outpatient — Confirm diagnosis + complete the ERS/BTS etiologic bundle, stratify with BSI/FACED, and maintain the airway-clearance + mucoactive foundation with phenotype-conditional escalation to reduce exacerbations and slow decline (ERS 2025)

Disposition criteria:
- Continue foundation + current escalation tier if stable (ERS 2025)
- Step up escalation tier if exacerbation frequency worsens (ERS 2025)
- Refer for surgical resection if focal disease + refractory; transplant if advanced (ERS 2025)

Escalation triggers (move to higher acuity):
- Acute severe exacerbation or respiratory failure → ED (ERS 2025)
- Significant or massive haemoptysis → ED + interventional radiology (ERS 2025)
- Progressive decline despite maximal therapy → transplant assessment / surgical resection review (ERS 2025)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Massive haemoptysis (large-volume / haemodynamic or airway compromise) in known bronchiectasis (ERS 2025)
- [SEVERE] Non-tuberculous mycobacteria isolated on mycobacterial culture in a patient on or being considered for macrolide maintenance (ERS 2025)
- [SEVERE] Severe exacerbation with respiratory failure / haemodynamic compromise (ERS 2025)

Citations

- ERS 2025 Clinical Practice Guideline for the management of adult bronchiectasis (Chalmers, Eur Respir J 2025; PMID 41016738; DOI 10.1183/13993003.01126-2025) + BTS 2019 Bronchiectasis Guideline (Hill, Thorax 2019; PMID 30545985; DOI 10.1136/thoraxjnl-2018-212463) [PMID:41016738](https://pubmed.ncbi.nlm.nih.gov/41016738/)
- Cited evidence (PMID 30545985) [PMID:30545985](https://pubmed.ncbi.nlm.nih.gov/30545985/)
- Cited evidence (PMID 31249313) [PMID:31249313](https://pubmed.ncbi.nlm.nih.gov/31249313/)
- Cited evidence (PMID 32897034) [PMID:32897034](https://pubmed.ncbi.nlm.nih.gov/32897034/)
- Cited evidence (PMID 24625200) [PMID:24625200](https://pubmed.ncbi.nlm.nih.gov/24625200/)

Last reconciled with current guidelines: 2026-05-26.
References
  • ERS 2025 Clinical Practice Guideline for the management of adult bronchiectasis (Chalmers, Eur Respir J 2025; PMID 41016738; DOI 10.1183/13993003.01126-2025) + BTS 2019 Bronchiectasis Guideline (Hill, Thorax 2019; PMID 30545985; DOI 10.1136/thoraxjnl-2018-212463) — PMID:41016738
  • Cited evidence (PMID 30545985) — PMID:30545985
  • Cited evidence (PMID 31249313) — PMID:31249313
  • Cited evidence (PMID 32897034) — PMID:32897034
  • Cited evidence (PMID 24625200) — PMID:24625200