Three or more clinical signs must be present:

1. Recurrent chondritis both auricles
2. Non-erosive inflammatory polyarthritis
3. Nasal chondritis
4. Ocular inflammation
5. Respiratory tract chondritis
6. 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.