RELAPSING POLYCHONDRITIS

This uncommon disease is characterized by recurrent inflammation and destruction of cartilaginous and other connective tissue structures. Frequently involved cartilages are the pinnae of the ears, nasal cartilages, and tracheal rings.

Polychondritis occurs nearly equally in both sexes and at any age, but with a peak of onset between the ages of 40 and 60. The pathologic lesion seen by light microscopy consists of loss of matrix staining, predominantly superficial infiltration with polymorphonuclear neutrophils or lymphocytes, and eventual destruction of normal structures followed by fibrosis. Electron microscopy in addition shows alterations of superficial chondrocytes, matrix, and elastic fibers. The cause of polychondritis is unknown, but the location of lesions and frequency of associated systemic diseases suggest the importance of systemic factors. Antibodies to type II collagen and the presence of cell-mediated immunity to proteoglycan and type II collagen are evidences of immunologic aberrations

Inflammation of the cartilaginous structures of the ears is the most common initial finding. There may be acute onset of pain and tenderness with erythema and swelling of one or both helice's. The lobe is spared. Inner and middle ear involvement can occur, causing hearing loss or vertigo. Nasal cartilage involvement can produce a saddle nose. 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. Cine CT scans and pulmonary function tests can detect more subtle airway obstruction.

Relapsing polychondritis is associated with other diseases in one third or more of cases. These include rheumatoid arthritis, systemic lupus erythematosus, Sj6gren's syndrome, thyroid disease, ulcerative colitis, vasculitis of various types, cryoglobulinernia, diabetes mellitus, biliary cirrhosis, malignancies, sinusitis, and mastoiclitis.

In mild cases nonsteroidal anti-inflammatory agents can be used for symptomatic treatment, although adrenocorticosteroids in the range of 30 to 60 mg prednisone per day are generally needed for acute inflammatory episodes and severe respiratory involvement. There is no evidence that steroids alter the long-term course of the disease. Immunosuppressives and recently cyclosporin A have been used with apparent benefit. Dapsone has been used with variable results in several series.

The course is unpredictable, with about 55 per cent of subjects surviving for 10 years. Infection and systemic vasculitis caused more deaths than did airway obstruction in a recent series. Remissions do occur. Aortic valve disease has required surgery.