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Updated: May 27, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Relapsing polychondritis: tracheobronchial involvement and differential diagnoses.

Louis Grandière¹, Hugues Vicaire¹, Gabriel Pop²

¹Pulmonology Department, Reference Center for Rare Pulmonary Diseases, Hôpital Avicenne, Assistance Publique - Hôpitaux de Paris - Hôpitaux Universitaires de Paris Seine-Saint-Denis, Bobigny, France.

Journal of Thoracic Disease

|February 20, 2025

Summary

Relapsing polychondritis (RP) can affect the airways, requiring a multimodal diagnostic approach. Early diagnosis and targeted therapies, including TNF-α inhibitors, improve outcomes for this rare autoimmune disease.

Keywords:

Relapsing polychondritis (RP)subglottic stenosis trachea tracheal stenosis tracheomalacia

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Published on: September 20, 2024

Area of Science:

Rheumatology
Pulmonology
Immunology

Background:

Relapsing polychondritis (RP) is a rare systemic autoimmune disease targeting cartilage.
Tracheobronchial involvement in RP is severe, significantly impacting prognosis.
Differential diagnosis is challenging due to overlapping inflammatory conditions.

Purpose of the Study:

To review diagnostic and therapeutic strategies for tracheobronchial involvement in RP.
To highlight differential diagnoses of inflammatory origin.
To discuss advancements in managing RP-related airway complications.

Main Methods:

Multimodal assessment including dynamic thoracic imaging and flexible bronchoscopy.
Pulmonary function tests, emphasizing pulse oscillometry for obstructive ventilatory impairment.
Nuclear imaging for detecting metabolic activity in affected cartilages.

Main Results:

Tracheobronchial lesions require comprehensive evaluation for accurate diagnosis.
Pulse oscillometry aids in diagnosing and monitoring ventilatory impairment.
Nuclear imaging assists in sharpening the diagnosis by identifying active inflammation.

Conclusions:

Improved diagnostic tools and interventional bronchoscopy enhance management.
Immunosuppressants, particularly TNF-α inhibitors, offer effective treatment options.
A combination of therapies and supportive care improves the prognosis for RP patients with airway involvement.