Laryngeal and tracheal disease can cause hoarseness or life-threatening up per respiratory obstruction. Ocular manifestations are common and include conjunctivitis, episcleritis, iridocyclitis, proptosis, and rarely other problems such as optic neuritis. Antigens in the eye that are cross-reactive with cartilage proteoglycans and their link protein have been identified. Cardiac involvement , especially of the aortic root with aortic insufficiency, is seen in up to one fourth of cases. There may also be aortic aneurysms.
Arthritis is reported in about three fourths of cases.
This is generally nondestructive. Fever, rashes, oral or genital ulcers, and neurologic and renal disease can occur. There are no diagnostic laboratory tests, although the erythrocyte sedimentation rate is often elevated. There may be anemia and leukocytosis. Roentgenograms can detect advanced tracheal narrowing.