Relapsing Polychondritis Diagnosis and Management

Definition and Pathophysiology

Relapsing polychondritis involves perichondral inflammation with cartilaginous fragmentation and destruction that is eventually replaced by fibrosis 1
The disease demonstrates an equal male/female ratio with a mean age at diagnosis of 51 years 1

The most frequent symptoms include swelling and erythema of the ears (88%) and arthralgias (81%) 1
A relapsing course is seen in 86% of patients, while continuous symptoms occur in 14% 1
Airway involvement represents one of the most serious manifestations and is responsible for many pulmonary complications 1
The respiratory system is affected in >50% of patients, with pulmonary symptoms being the presenting features in up to 25% of cases 1
Respiratory cartilage involvement can include the external nares, nasal septal turbinates, eustachian tubes, epiglottis, larynx, thyroid, cricoid, arytenoid, trachea, and bronchi 1
Persistent or chronic cough is often one of the first and important symptoms signifying respiratory involvement 1
Other pulmonary symptoms include wheezing, stridor, dyspnea, hoarseness or aphonia, and tenderness over the laryngotracheal cartilage 1

Patients with suspected relapsing polychondritis should undergo pulmonary function tests, including inspiratory and expiratory flow-volume curves to identify clinically subtle airway involvement 1
Chest radiographs are usually unhelpful in diagnosis 1
CT scanning may reveal:
- Thickening and stenosis of the tracheobronchial wall 2
- Collapse and calcification of cartilage 1
- Bronchiectasis (in late stages of disease) 1
Bronchoscopy is not always required but may show inflammation, collapse, and stenosis of the larynx, trachea, and/or bronchi 1

Therapy for relapsing polychondritis typically includes:
- Salicylates and nonsteroidal anti-inflammatory drugs for milder cases 1
- Cytotoxic agents and immunosuppressants for more severe disease 1
Patients with acute cases generally exhibit a good response to therapy 1
Respiratory involvement usually requires long-term use of systemic corticosteroids and other immunosuppressants 1

Auricular chondritis presents as painful swelling, warmth, and redness in a portion of the auricle that characteristically spares the earlobe, according to the American Academy of Pediatrics 3, 4