SELECTIVE IGA DEFICIENCY WITH RECURRENT VASCULITIS OF THE CENTRAL-NERVOUS-SYSTEM

We describe an unusual case of selective IgA deficiency complicated by recurrent vasculitis of the central nervous system (CNS). The patient suffered from two episodes of CNS vasculitis, one of which was locate d in the cerebrum and the other in the cerebellum. The vasculitic proc ess resulted in brain tumor-like lesions shown by computed tomography. There was no evidence of associated connective tissue diseases. Vascu litis in other organs or tissues was not noted. This is the first deta iled description in the English literature of pathologically proven CN S vasculitis in a patient with selective IgA deficiency. Our report de monstrates that isolated CNS angiitis can be a rare clinical feature o f selective IgA deficiency.