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.
2 min synopsisUK scopeSources checked 27 Aug 2026Clinical 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.
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.
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.
Document lobule sparing and at least one additional cartilage, ocular, hearing, joint or cardiovascular feature and apply criteria as support rather than proof.
Exclude bacterial perichondritis, GPA and focal trauma or tumour and obtain FBC indices, urine and organ-directed autoimmune or infection tests.
Airway first-line escalationStabilise before sedating or scoping
Stridor, progressive hoarseness, central wheeze or positional respiratory compromise is present.
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.
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.
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.