Arthritis, hives, and angioedema may sometimes be a part of the clinical presentation of infectious or connective tissue diseases.
However this symptomatological triad may occur in the absence of any other associated disease and for these cases the diagnosis of AHA syndrome has been proposed.
The joint and skin manifestations are generally in close temporary correlation even if incomplete attacks may occur and relapses may take place with varying frequency (from two episodes/month to one every six months).
The arthritis is commonly asymetric and involves the metacarpophalangeal and proximal interphalangeal joints of the hands, wrists, elbows, shoulders, ankles and knees.
Hives is often generalized and may histologically present with leukocytoclastic vasculitis (urticaria vasculitica) or a modest perivascular mononuclear infiltration.
Steroid treatment is probably the most effective though other drugs have been used with good results in individual cases.