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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.

Cryptogenic organising pneumonia

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

RapidFull
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Escalate

Organising pneumonia can occasionally produce rapidly progressive hypoxaemic respiratory failure. Admit urgently when oxygenation, respiratory effort or haemodynamics are compromised, and investigate severe infection, pulmonary embolism, oedema, diffuse alveolar haemorrhage and acute eosinophilic pneumonia. Do not give outpatient corticosteroids for an undifferentiated acute bilateral infiltrative illness; obtain early respiratory and critical-care input and treat time-critical alternatives.

Synopsis

Recognise organising pneumonia as a clinicoradiological syndrome, exclude secondary causes and infection, and use specialist corticosteroid treatment with relapse-aware monitoring.

  • Organising pneumonia is a tissue-repair pattern with intra-alveolar granulation tissue; cryptogenic organising pneumonia is diagnosed only when no cause is found.
  • Secondary triggers include infection, connective-tissue disease, medicines, radiotherapy, malignancy, inflammatory bowel disease and transplantation.
  • Patients often have weeks of dry cough, breathlessness, fever, malaise and weight loss that fail to resolve with routine antibiotics.
  • HRCT commonly shows patchy peripheral or peribronchovascular consolidation, ground glass and migrating opacities, but no single sign is pathognomonic.
  • The reverse-halo sign can support the pattern but also occurs in fungal infection, infarction, granulomatous disease and malignancy.
  • A brisk corticosteroid response is typical but is not retrospective proof; lymphoma, eosinophilic disease and infection can transiently improve or fluctuate.
  • Relapse during taper or after stopping treatment is common and usually prompts diagnostic and exposure review before simply increasing steroids.
  • Biopsy is unnecessary in every typical case but becomes important when imaging, clinical behaviour or treatment response is atypical.
  • Cryptogenic disease has no ongoing causal treatment beyond controlling the inflammatory process; secondary organising pneumonia requires the trigger addressed.
  • Pulmonary function, oxygenation and imaging trajectory should guide treatment duration rather than pursuing complete radiographic clearing at any toxicity cost.

Key red flags

Severe diffuse presentation

Rapidly spreading bilateral opacity, profound desaturation, tachypnoea or exhaustion can resemble ARDS and requires emergency respiratory and critical-care assessment.

Malignancy warning

A persistent focal mass-like consolidation, haemoptysis, unilateral nodes or weight loss out of proportion to inflammation should enter an urgent lung-cancer pathway.

Atypical non-response

Failure to improve after an adequate supervised corticosteroid trial should prompt urgent reconsideration of infection, lymphoma, adenocarcinoma, eosinophilic pneumonia, vasculitis or an incorrect pathological label.

Investigation priorities

01
Chest radiograph and HRCTFirst step

Define extent, distribution and features requiring another pathway.

Management branches

Diagnostic work-upMove beyond non-resolving pneumonia

Subacute multifocal consolidation persists despite appropriate initial antimicrobial care.

  1. Reassess physiological stability and obtain HRCT, looking for organising distribution while identifying pleural, nodal or mass-like features that require a cancer or infection pathway.
  2. Review all medicines, radiotherapy, systemic inflammatory symptoms, immune status and microbiology; do not use the word cryptogenic until secondary causes have been addressed.
  3. Discuss at respiratory or ILD MDT whether clinicoradiological confidence is sufficient or lavage and tissue would change the decision.
  4. Document the working diagnosis and planned response measures, including when non-response will trigger biopsy or a renewed malignancy and infection search.

Key medicines

PrednisoloneThere is no single current UK regimen for COP; specialist practice uses a daily induction tailored to physiological severity, followed by a slow response-guided taper over months under the local ILD protocol.Establish reasonable diagnostic confidence and assess infection first. Monitor glucose, blood pressure, mood, weight, bone and eye health; relapse is common during taper, while lack of response should challenge the diagnosis rather than trigger endless dose escalation.
Long-term macrolide in selected casesNo routine COP dose should be copied across services; if an ILD specialist uses azithromycin or clarithromycin off label, follow the BTS macrolide and local antimicrobial protocol exactly.Exclude non-tuberculous mycobacterial infection where relevant and review ECG QTc, liver function, hearing, interactions and antimicrobial resistance. This is not a substitute for investigating a focal lesion or treating severe hypoxaemia.
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Sources and review status4 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 interstitial lung disease guidelineArchived UK reference for organising pneumonia; use current MDT and local protocols for modern treatment decisions.
  • BTS long-term macrolide guidelineSpecialist safety framework; evidence in organising pneumonia remains limited.
  • NICE suspected cancer referral guidanceUrgent referral framework for suspicious persistent pulmonary findings.
  • Prednisolone summary of product characteristicsCorticosteroid contraindications, interactions and adverse-effect profile.

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

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom