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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.

Relapsing polychondritis

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

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Critical laryngotracheobronchial disease

Stridor, rapidly progressive hoarseness, fixed or monophonic wheeze, respiratory distress, cyanosis or positional collapse may reflect subglottic stenosis, cartilage oedema or tracheobronchomalacia and can worsen during sedation or instrumentation.

Action: Admit with senior anaesthetic, ENT, respiratory and critical-care involvement, keep spontaneous ventilation until an airway plan is agreed, obtain urgent dynamic airway imaging or endoscopy only when safe, give specialist high-dose glucocorticoid for active inflammation, and prepare rigid bronchoscopy, tracheostomy or stenting for mechanical failure.

Synopsis

Recognise recurrent cartilage inflammation, detect dynamic laryngotracheal and cardiovascular disease before collapse, distinguish infection, vasculitis and VEXAS, and match immune and airway treatment to organ threat despite a limited evidence base.

  • Relapsing polychondritis is recurrent immune inflammation of cartilage and proteoglycan-rich structures; the classic hot tender pinna spares the non-cartilaginous lobule.
  • Ask about hoarseness, cough, stridor, positional or exertional breathlessness and wheeze that does not behave like asthma at every encounter because airway disease determines mortality.
  • Nasal chondritis causes bridge pain before saddle-nose collapse; audiovestibular, ocular, non-erosive joint, skin, aortic and valvular disease complete the multisystem pattern.
  • Diagnosis is clinical and no blood test confirms it. McAdam, Damiani or Michet criteria organise evidence but do not replace exclusion of infection, GPA and VEXAS.
  • First-line airway assessment combines inspiratory and expiratory CT, flow-volume loops and ENT laryngoscopy; bronchoscopy requires an expert plan because sedation and instrumentation can precipitate collapse.
  • Biopsy is not routine in a classic presentation. Sample an active accessible cartilage edge when malignancy, infection, VEXAS or another diagnosis remains materially possible.
  • Mild auricular, nasal or joint disease may use an NSAID, colchicine or dapsone, but systemic prednisolone is usual first-line treatment for important inflammation.
  • Airway, ocular, audiovestibular, cardiac or vascular threat requires high-dose glucocorticoid plus early steroid-sparing therapy and the relevant surgical or device team.
  • Methotrexate is a common steroid-sparing choice; azathioprine, mycophenolate, cyclophosphamide and biologics are off-label and selected from organ severity and associated disease.
  • Screen older men with macrocytosis, cytopenia, thrombosis or neutrophilic dermatosis for VEXAS because treating it as ordinary cartilage-limited disease misses marrow ownership.

Key red flags

Stridor, new voice change, refractory monophonic wheeze, inability to lie flat or exertional desaturation requires same-day upper and central airway assessment.

Sudden visual loss, severe ocular pain, proptosis or corneal change may indicate scleritis, retinal vasculitis, keratitis or orbital inflammation and needs urgent ophthalmology.

Syncope, pulse deficit, new murmur, chest or back pain, heart failure or unequal pressures raises aortitis, aneurysm, dissection, coronary ostial or valvular disease.

Fever with a hot swollen pinna after piercing, surgery or trauma requires infection assessment; immunosuppression for presumed relapse can accelerate Pseudomonas perichondritis.

Macrocytic anaemia, thrombocytopenia, neutrophilic skin disease, venous thrombosis or steroid-dependent chondritis in an older man raises VEXAS and marrow failure.

Haematuria, rapidly falling eGFR, pulmonary haemorrhage or destructive ENT disease is atypical for isolated relapsing polychondritis and requires vasculitis or associated-disease assessment.

Laryngotracheobronchial disease

Hoarseness, stridor, fixed wheeze, exertional or positional dyspnoea and recurrent lower-respiratory infection can indicate stenosis or dynamic collapse.

Investigation priorities

01
First-line dynamic inspiratory and expiratory CTFirst stepFirst line

Define subglottic and central-airway thickening, fixed stenosis, calcification and expiratory collapse.

Management branches

First diagnostic sequenceProve the pattern and test dangerous alternatives

Recurrent ear, nose, airway or costochondral inflammation raises relapsing polychondritis.

  1. Document lobule sparing and at least one additional cartilage, ocular, hearing, joint or cardiovascular feature and apply criteria as support rather than proof.
  2. Exclude bacterial perichondritis, GPA and focal trauma or tumour and obtain FBC indices, urine and organ-directed autoimmune or infection tests.
  3. In an older man with macrocytosis, cytopenia, thrombosis or neutrophilic skin disease, refer for marrow and somatic UBA1 assessment before final classification.
Airway first-line escalationStabilise before sedating or scoping

Stridor, progressive hoarseness, central wheeze or positional respiratory compromise is present.

  1. Admit, monitor oxygenation and ventilation and involve anaesthesia, ENT, respiratory, interventional bronchoscopy and critical care before airway manipulation.
  2. Obtain dynamic CT and nasendoscopy if stable, preserving spontaneous ventilation; perform bronchoscopy only with a planned rigid, surgical and tracheostomy rescue.
  3. Give specialist glucocorticoid for active inflammation and use positive pressure, dilation, tracheostomy, stent or reconstruction according to fixed versus dynamic failure.

Key medicines

PrednisoloneFor important systemic disease, give 0.5–1 mg/kg orally each morning, then taper against organ response; minor disease may require a lower, shorter course and airway or vascular disease often needs early steroid-sparing therapy.Exclude infection, monitor glucose, pressure, mood, bone, eye and adrenal risks and avoid allowing partial symptom response to postpone airway or vascular procedures.
Methylprednisolone intravenous pulseFor selected life-, sight-, hearing- or airway-threatening inflammation, give 500–1000 mg intravenously once daily for three days, followed by oral taper and steroid-sparing treatment.Coordinate cultures, airway stability, cardiac rhythm, glucose, mental state and infection prophylaxis; this off-label syndrome regimen requires multidisciplinary supervision.
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Sources and review status7 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.

  • ERN ReCONNET relapsing polychondritis practice reviewClinical criteria, organ assessment, airway danger and treatment evidence gaps.
  • Relapsing polychondritis comprehensive reviewClinical manifestations, differential diagnosis, pathology and severity-based care.
  • Original description of VEXAS syndromeUBA1 somatic disease, chondritis, cytopenia, thrombosis and marrow phenotype.
  • NHS England National Genomic Test DirectoryCurrent NHS genomic testing and specialist laboratory route.
  • Prednisolone UK product informationGlucocorticoid dosing principles and safety.
  • Methotrexate UK product informationWeekly dosing, interactions and organ monitoring.
  • Dapsone UK product informationDose, G6PD, haemolysis, methaemoglobinaemia and blood monitoring.

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

Authoring stateRapid draft
Ocular and audiovestibular disease

Scleritis, uveitis, keratitis, sudden hearing loss, tinnitus or vertigo is function threatening and needs organ-specialist treatment alongside systemic control.

Cardiovascular involvement

A new regurgitant murmur, pulse asymmetry, aneurysmal pain, conduction change or heart failure raises aortitis, valvulitis and coronary or arterial disease.

Clinical stateAwaiting review
JurisdictionUnited Kingdom