Diagnostic evaluation of relapsing polychondritis
- Clement J Michet, MD
Clement J Michet, MD
- Associate Professor of Medicine
- Mayo Clinic
Relapsing polychondritis (RPC) is a systemic inflammatory/degenerative disease process that may potentially compromise the structural and functional integrity of cartilage, organs of special sense, and the cardiovascular, renal, and nervous systems (table 1). (See "Clinical manifestations of relapsing polychondritis".)
The diagnostic evaluation of RPC is reviewed here. The pathogenesis, pathology, clinical manifestations, and treatment of RPC are discussed separately. (See "Etiology and pathogenesis of relapsing polychondritis" and "Pathology of relapsing polychondritis" and "Clinical manifestations of relapsing polychondritis" and "Treatment of relapsing polychondritis".)
The diagnosis of relapsing polychondritis (RPC) is established by the combination of clinical findings, supportive laboratory data, imaging procedures, and biopsy of an involved cartilaginous site (see 'Diagnostic criteria' below). There is no blood test that is specific for RPC.
A spectrum of histologic findings may be present in involved organs. The specific findings depend, in part, on the timing of the biopsy. Histopathologic findings upon biopsy of the auricular cartilage during active disease are pathognomonic, while an auricle with advanced disease and deformity reveals fibrosis. The pathologic features of auricular chondritis and lesions of the aorta, tracheobronchial tree, synovium, kidneys, and eye are presented separately. (See "Pathology of relapsing polychondritis".)
Diagnostic criteria — Either of two sets of empirically derived and related diagnostic criteria may be used to diagnose RPC:
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- Diagnostic criteria
- Diagnostic evaluation
- - History and physical examination
- - Laboratory testing
- - Tissue biopsy
- - Other testing/specialized examinations
- DIFFERENTIAL DIAGNOSIS
- Auricular inflammation
- Saddle nose deformity
- Narrowing of the airway
- Ocular inflammation
- Granulomatosis with polyangiitis (Wegener’s)
- Systemic inflammatory polyarthritis
- Aortitis and aortic aneurysms
- Chest wall pain
- POST-DIAGNOSTIC EVALUATION
- Overview of post-diagnostic testing
- - All patients
- - Selected patients
- Evaluation for pulmonary involvement
- - Pulmonary function testing
- - Imaging
- Chest radiography
- Computed tomography
- Magnetic resonance imaging
- Other imaging techniques
- - Bronchoscopy
- Evaluation for cardiac involvement
- SUMMARY AND RECOMMENDATIONS