Familial Granulomatous Arteritis with Polyarthritis of Juvenile Onset

Granulomatous arteritis, which occurs through cell-mediated immune mechanisms, can be seen in several disease states. This chronic process is not regarded as an inherited phenomenon. We report on a family with granulomatous arteritis, associated with juvenile-onset polyarthritis, that appears to be inherited in a dominant fashion with variable penetrance. The affected members of the family share the clinical triad of fever, hypertension, and polyarthritis of juvenile onset, along with the pathological feature of noncaseating granulomas in vascular and extravascular distribution. The clinical and pathological findings and the genetic assessment of this family form the basis of this report. Case Reports . . .