Grand Rounds | Clinician's Corner

Polyarteritis Nodosa

John H. Stone, MD, MPH
JAMA. 2002;288(13):1632-1639. doi:10.1001/jama.288.13.1632.
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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.

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Figure 1. Cutaneous Manifestations of the Patient's Polyarteritis Nodosa
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A, Ulcerations appear on the patient's right foot; B, digital tissue loss is apparent in several toes of the left foot.
Figure 2. Patient's Skin Biopsy Specimen
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Biopsy of the skin from the edge of an ulcer shows leukocytoclasis and fibrinoid necrosis in a medium-sized muscular artery situated at the junction of the deep dermis and subcutaneous fat (hematoxylin-eosin, original magnification × 400).
Figure 3. Drawings by Kussmaul and Maier
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Reprinted with permission from the Mayo Foundation.5 B, The epicardial arteries appear as "thickened . . . misshapened [sic], nodular, whitish-yellow cords."5
Figure 4. Gastrointestinal Manifestations of Polyarteritis Nodosa in 2 Patients
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A, Whitish, nodular inflammatory infiltrate tracking the course of medium-sized stomach arteries; B, mesenteric angiogram from a different patient demonstrates diffuse involvement of the gastrointestinal tract arteries supplying the small and large bowel. Panel A is courtesy of Grover Hutchins, MD, Department of Pathology, The Johns Hopkins University School of Medicine.
Figure 5. Jejunal Specimen Obtained at Laparotomy From a Patient Who Died of Complications of Mesenteric Ischemia Related to Polyarteritis Nodosa
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The internal elastic lamina of the artery has been focally disrupted by the inflammatory process (arrows). An area of fibrinoid necrosis is indicated as well (X) (elastin stain, original magnification × 400). The larger vessel, a vein, is not involved (note its intact internal elastic lamina). Reprinted with permission from Excerpta Medica Inc.29



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