Miscellaneous vasculitides

A. Relapsing polychondritis is a rare disease characterized by inflammation of cartilage, mainly of the ears, nose, larynx, and joints.

1. Clinical presentation. The most common clinical manifestation is a destructive auricular chondritis with sparing of the ear lobule. Articular symptoms are second in frequency and are usually self-limited, with a nonerosive oligoarticular or polyarticular peripheral arthritis. Inflammatory eye disease may also occur. Leukocytoclastic vasculitis causing skin lesions and aneurysms of the thoracic-abdominal aorta and cerebral artery can occur. In 30% of cases, there is an association with rheumatoid arthritis, Sjogren's syndrome, WG, microscopic polyangiitis, and malignancies (including carcinoma of the lung, breast, and colon and myeloproliferative disorders). There are no specific laboratory tests for relapsing polychondritis, but biopsy of an affected area is diagnostic, showing loss of basophilic staining of the cartilage matrix accompanied by perichondral inflammation at the interface of cartilage and soft tissue and fibrocyte and capillary endothelial cell proliferation.

2. Treatment. Although NSAIDs can control mild episodes of inflammation, corticosteroids are the mainstay of treatment, initially in dosages of 0.75 to 1 mg/kg daily. Immunosuppressive drugs are used as steroid-sparing agents in patients requiring long-term steroid therapy.

B. Behcet's syndrome. First described by Hulusi Behget of Istanbul, this syndrome is a systemic vasculitis involving both arteries and veins.

1. The clinical presentation is characterized by recurrent oral and genital aphthous ulceration, chronic relapsing uveitis, and a variety of skin manifestations, including the pathergy reaction (nonspecific hyperreactivity of the skin), erythema nodosum, and superficial thrombophlebitis. Uveitis can lead to blindness in up to 20% of patients with eye disease. Male and young patients have a more severe course than do female and older patients. Behget's syndrome has been associated with HLA-B51 and is mainly observed in countries around the Mediterranean and in the Far East. The natural history of Behget's syndrome is one of exacerbations and remissions. Disease manifestations as a rule usually become less severe with time.

2. Treatment. Mild oral and genital ulcers can be treated with local corticosteroids. Thalidomide has also been used successfully in treating mucocutaneous lesions. Colchicine, which has traditionally been used for every aspect of Behget's syndrome, seems to be effective mainly for the treatment of mucocutaneous lesions in female patients. Immunosuppression is the mainstay of treatment for eye disease; azathioprine has been shown to maintain visual acuity and prevent new eye disease, and cyclosporin A is used for disease flares. The role of heparin or anticoagulants for thrombophlebitis is still being debated. Anti-platelet drugs are preferred for milder cases.

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