Thirty Years of Followup in 3 Patients with Familial Polyarteritis Nodosa due to Adenosine Deaminase 2 Deficiency

Three sisters of Northern European descent provided an opportunity to examine the longterm course and possible familial aspects of a rare disease, polyarteritis nodosa (PAN). Approval and consent was obtained from each patient. The sisters have been cared for in the Johns Hopkins Vasculitis Center for the past 3 decades. At age 7, the first sister developed rash, fever, lethargy, and a deviated right eye and was treated empirically for...