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Relapsing polychondritis

Case contributed by Dr Necip Gungor Atikeler
Diagnosis almost certain

Presentation

Refractory non-productive cough and dyspnea. Airway obstruction findings in respiratory function tests.

Patient Data

Age: 55 years
Gender: Male

Chest

ct

Long segment anterior and lateral tracheal wall thickening with sparing of the posterior tracheal wall.

Case Discussion

Relapsing polychondritis is a rare rheumatic disease characterized by recurrent inflammation of cartilaginous structures, with airway involvement being a major cause of morbidity and mortality. Relapsing polychondritis affects cartilage in multiple organs, such as the ear, nose, larynx, trachea, bronchi, and joints.

Radiologic manifestations include long segment tracheobronchial strictures, mural thickening, and calcification. Posterior membranous wall of trachea is typically spared like tracheobronchopathia osteochondroplastica. In relapsing polychondritis, tracheal wall thickening is more likely smooth and hyperdense. But in tracheobronchopathia osteochondroplastica, thickening is irregular and nodular. 

The mainstay of treatment remains medical management with corticosteroids. Invasive techniques include placement of endobronchial polymeric silicone stents to help maintain airway patency.

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