The AHA syndrome: arthritis, hives and angioedema

Nine patients who have intermittently exhibited the concurrent triad of arthritis or arthralgia (A), hives or urticaria (H) and angioedema (A), in the absence of associated infection or connective-tissue disease, are reported. The ratio of women to men is 4:1, with no apparent age specificity. The duration of the disease has been up to 16 years, with an average of seven acute episodes per year, lasting up to 14 days. Upper-airway angioedema has been severe in four patients. Routine laboratory studies were normal, as were studies of complement levels, and both humoral and cellular immunity. Two samples of synovial fluid from one patient contained a marked preponderance of Ia-positive macrophages. The absence of associated infection and connective-tissue disease suggests this recurrent triad represents a distinct entity, which is designated the AHA syndrome.