Author Affiliation: Division of Rheumatology, Johns Hopkins Vasculitis Center, Johns Hopkins University School of Medicine, Baltimore, Md.
Grand Rounds at The Johns Hopkins Hospital Section
Editors: David B. Hellmann, MD, D. William Schlott, MD, Stephen D.
Sisson, MD, The Johns Hopkins Hospital, Baltimore, Md; David S. Cooper, MD,
Contributing Editor, JAMA.
Polyarteritis nodosa (PAN) is regarded rightly as the grandfather of
the vasculitides. In this Grand Rounds, the case of a 30-year-old man with
a 12-year illness is described. The patient presented with daily fevers, tachycardia,
and cutaneous ulcers on his distal extremities. He eventually developed mononeuritis
multiplex. Because of the striking pattern of his fevers, he was diagnosed
for many years as having adult-onset Still disease. Following the addition
of daily cyclophosphamide to his long-standing regimen of prednisone, the
patient's disease entered remission for the first time in more than a decade.
He was ultimately able to discontinue all of his immunosuppressive medications.
The case is discussed in the context of the first patient ever described with
PAN, the classic report of Kussmaul and Maier.
Stone JH. Polyarteritis Nodosa. JAMA. 2002;288(13):1632-1639. doi:10.1001/jama.288.13.1632