Three or more clinical signs must be present:
- Recurrent chondritis both auricles
- Non-erosive inflammatory polyarthritis
- Nasal chondritis
- Ocular inflammation
- Respiratory tract chondritis
- Cochlear and, or, vestibular dysfunction
OR 1 or more signs with histologic confirmation
OR Chondritis in 2 or more separate sites AND response to steroids or immunosuppression
Differential diagnosis for these individual symptoms and signs includes most of the autoimmune disorders and other infectious and non-infectious granulomatous disorders including Wegener's granulomatosis, polyarteritis nodosa (PAN), Takayasu's arteritis, giant cell arteritis (GCA), rheumatoid arthritis, Reiter's syndrome, rheumatic fever, polymorphic reticulosis, syphilis, tuberculosis, histoplasmosis, leprosy, sarcoidosis, and malignancy.