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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Small-airways disease and bronchiolitis obliterans

Respiratory · UK scope · Sources checked 27 Aug 2026 · Clinical review pending

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Escalate

An immunocompromised or transplant recipient with new hypoxaemia, fever, rapid FEV1 decline or respiratory distress needs urgent same-day transplant, haematology and respiratory assessment. Exclude infection, acute rejection, pulmonary embolism, pneumothorax and fluid or cardiac causes before attributing deterioration to chronic bronchiolitis obliterans, and follow the centre-specific emergency pathway.

Synopsis

Recognise small-airway physiology and constrictive bronchiolitis, identify causal contexts including transplantation and inhalation injury, and coordinate evidence-limited specialist treatment without confusing organising pneumonia.

  • Small airways are bronchioles below the resolution of routine imaging; substantial disease can exist before conventional spirometry becomes clearly abnormal.
  • Bronchiolitis obliterans, or constrictive bronchiolitis, is fibrotic narrowing and obliteration of small airways that usually produces progressive, relatively fixed airflow obstruction.
  • Important contexts include lung transplantation, allogeneic haematopoietic stem-cell transplantation, connective-tissue disease, severe inhalational injury, selected infections and drug or immune-mediated injury.
  • After lung transplantation, an obstructive chronic allograft dysfunction phenotype is termed bronchiolitis obliterans syndrome when defined clinically without requiring surgical histology.
  • Symptoms are often insidious exertional breathlessness and dry cough; examination may be normal or reveal wheeze, crackles or inspiratory squeaks.
  • Expiratory HRCT may show mosaic attenuation and air trapping, but these findings are not specific and require inspiratory comparison, adequate expiration and clinical context.
  • Small-airway indices from spirometry can be suggestive but are variable and should not independently diagnose bronchiolitis obliterans.
  • Exclude reversible asthma, COPD, bronchiectasis, infection and central airway disease; a poor bronchodilator response does not by itself establish bronchiolar fibrosis.
  • Treatment is cause-specific and evidence is limited. Transplant immunosuppression changes belong to the transplant centre; there is no universal community corticosteroid regimen.
  • Selected post-lung-transplant patients may receive long-term azithromycin, but ECG, liver, hearing, drug-interaction, NTM and antimicrobial-resistance safeguards are essential.

Key red flags

Post-lung-transplant BOS pattern

A sustained fall from the best post-transplant expiratory function with new obstruction may represent BOS, but transplant specialists must exclude infection, acute rejection, airway stenosis and restrictive allograft dysfunction.

Post-HSCT bronchiolitis obliterans

Progressive dry cough, wheeze or exertional dyspnoea with fixed obstruction in a person with allogeneic transplantation and chronic graft-versus-host features should prompt urgent haematology-respiratory review.

Acute mimic or complication

Fever, purulent sputum, pleuritic pain, rapid hypoxaemia or abrupt functional loss suggests infection, embolism, pneumothorax or acute transplant complication rather than slow bronchiolar fibrosis alone.

Investigation priorities

01
Serial quality-assured spirometry with bronchodilator testingFirst step

Confirm obstruction, trajectory and degree of reversibility.

Management branches

SuspectConfirm a bronchiolar pattern

Progressive breathlessness or cough accompanies unexplained fixed obstruction or expiratory air trapping.

  1. Construct a timeline of transplantation, infection, inhalational exposure, autoimmune disease and medicines, noting onset relative to each potential insult.
  2. Repeat quality-assured spirometry with bronchodilator and obtain volumes, gas transfer and paired inspiratory-expiratory CT where clinically justified.
  3. Exclude asthma, COPD, bronchiectasis, central airway obstruction, infection, vascular disease and organising pneumonia using the pattern rather than one small-airway index.
  4. Refer for specialist multidisciplinary review and reserve invasive tissue sampling for a specific question likely to alter treatment.

Key medicines

Azithromycin for selected post-lung-transplant BOSA BTS-supported specialist regimen is 250 mg orally three times weekly for prevention, or 250 mg on alternate days for a defined treatment trial.This is specialist and often off-label care, not a universal regimen for all constrictive bronchiolitis. Check ECG, QT interactions, liver tests, hearing, gastrointestinal effects, sputum and NTM risk; counsel about antimicrobial resistance.
Inhaled long-acting bronchodilator trialUse one locally selected licensed inhaler at its device-specific adult maintenance dose after observing technique and documenting a baseline endpoint.Response is not guaranteed and non-response should prevent indefinite device accumulation. Check for beta2-agonist cardiac effects, antimuscarinic urinary or ocular effects and duplicate ingredients; it does not treat fibrotic obliteration.
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Sources and review status5 sources · checked 27 Aug 2026 · clinical review pending+
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

  • BTS Long-term macrolide guidelineBronchiolitis obliterans indications, regimens and safety safeguards.
  • NHS England Spirometry commissioning standardsQuality requirements for serial diagnostic physiology.
  • UKHSA Standards for microbiology investigationsRespiratory and mycobacterial specimen investigation framework.
  • BTS Non-tuberculous mycobacterial disease guidelineWhy macrolide monotherapy must be avoided in active NTM disease.
  • NICE NG115 COPD recommendationsDifferential assessment, bronchodilation and pulmonary rehabilitation principles.

Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

Authoring stateRapid draft
Clinical stateAwaiting review
JurisdictionUnited Kingdom