PubMed1991
Chronic relapsing polychondritis is a rare connective tissue disease of presumed autoimmunologic pathogenesis. It may involve multiple organ systems. The most characteristic signs are: relapsing inflammation of the cartilage of the outer ear, non-erosive polyarthritis, chondritis of the nasal cartilage, inflammation of different ocular tissues, inflammation of tracheal and bronchial cartilages and lesions of the inner ear. The wide range of ocular tissue involvement is discussed on the basis of two new cases with emphasis on scleritis, episcleritis, keratitis and chorioretinal involvement. Ocular disease complications that have not been published before are the massive development of subretinal stands following multiple intra- and subretinal infiltrates in the posterior pole connecting areas of chorioretinal scars. The importance of high-dose, long-term steroid therapy is stressed. In addition to steroids, immunosuppressive agents such as azathioprine and cyclophosphamide are sometimes mandatory to cope with severe multi-organ disease. Another option in very severe relapses may be plasma separation to improve the condition rapidly until drug therapy can be effective.