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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

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Relapsing polychondritis with large airway involvement.

Christiaan Yu¹, Simon A Joosten¹

¹Monash Lung and Sleep Monash Health Clayton Victoria Australia.

Respirology Case Reports

|November 20, 2019

Summary

Relapsing polychondritis can affect major airways, causing shortness of breath. This case highlights the need for airway assessment and positive airway pressure support alongside standard immunosuppression for effective treatment.

Keywords:

Non‐invasive ventilation relapsing polychondritis tracheomalacia

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Area of Science:

Rheumatology
Pulmonology
Autoimmune Diseases

Background:

Relapsing polychondritis (RP) is a rare autoimmune disorder causing progressive cartilage inflammation.
RP presents diversely, affecting multiple organs.
Large airway involvement in RP is uncommon but can cause significant respiratory distress.

Observation:

A patient with diagnosed relapsing polychondritis presented with shortness of breath.
CT imaging revealed thickened tracheal and bronchial cartilage, indicative of tracheobronchomalacia.
Evidence of repeated cartilaginous destruction was observed.

Findings:

The case highlights significant large airway compromise in relapsing polychondritis.
Tracheobronchomalacia and cartilaginous destruction were key CT findings.
Effective treatment involved high-dose systemic glucocorticoids and continuous positive airway pressure.

Implications:

Early identification of airway involvement is critical for patients with relapsing polychondritis.
Continuous positive airway pressure provides essential support for major airway compromise.
Integrated management combining immunosuppression and airway support optimizes outcomes.