Diagnostic Considerations

The diagnosis of polyarteritis nodosa (PAN) can be difficult because of the spectrum of clinical manifestations and the rarity of the disease. Mimics of vasculitis, including processes that lead to a loss of vascular integrity and blood-vessel occlusion alternate forms of vasculitis, and syndromes associated with vasculitis should be considered. Segmental arterial mediolysis is one example of a noninflammatory vasculopathy that can be misdiagnosed as a vasculitic process.^[51]Drugs such as minocycline have been reported to cause cutaneous and systemic PAN-type manifestations.^[52]Vitamin K antagonists have also been reported to cause cutaneous necrotic leg ulcers with leukocytoclastic vasculitis and microthrombosis on biopsy.^[53]

Microscopic polyangiitis (MPA; formerly called microscopic polyarteritis) and granulomatosis with polyangiitis (GPA; the disease formerly known as Wegener granulomatosis [WG]) are ANCA-associated systemic vasculitides that have some features similar to those of classic PAN, with the additional involvement of renal glomeruli and pulmonary capillaries.

A clear distinction should be made between limited disease versus systemic disease and idiopathic PAN versus hepatitis B–related PAN, because differences exist in the prognosis and treatment.

Finally, some patients with chronic HCV infection can present with vasculitis that is clinically consistent with PAN, not cryoglobulinemic vasculitis.

The entity of HCV-associated PAN may have a more severe presentation compared with HCV-associated cryoglobulinemia. In a cohort of 161 patients with HCV-related vasculitis, 31 patients were diagnosed with PAN. These patients had more frequent fevers, weight loss, GI tract involvement, severe acute sensory-motor multifocal mononeuropathy, kidney and liver microaneurysms, and increased C-reactive protein level than patients with HCV-associated mixed cryoglobulinemia.^[19]

Differential Diagnoses

Antiphospholipid Syndrome
Atrial Myxoma
Cholesterol Embolism
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
Cryoglobulinemia
Genetics of Ehlers-Danlos Syndrome
Goodpasture Syndrome
Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)
IgA Vasculitis (Henoch-Schonlein Purpura)
Infective Endocarditis
Leukocytoclastic (small vessel) vasculitis
Microscopic Polyangiitis
Segmental arterial mediolysis

Workup

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Media Gallery

Nonspecific, firm, tender subcutaneous nodules without livedo reticularis and/or systemic involvement may be the first sign of polyarteritis nodosa (PAN).
Tender, hyperpigmented, firm subcutaneous nodules with a background of livedo reticularis common in cutaneous polyarteritis nodosa (PAN).
Tender erythematous nodules with central "punched out" ulcerations common in cutaneous polyarteritis nodosa (PAN).
Polyarteritis nodosa (PAN) is characterized by fibrinoid necrosis of the arterial wall with a leukocytic infiltrate. In this slide, a large, pale occlusion of a muscular artery can be seen. Within this collagenous tissue is a leukocytic infiltrate, which is the hallmark of PAN. Courtesy of Urbana Atlas of Pathology.
Livedo reticularis in polyarteritis nodosa (PAN).

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Author

Dana Jacobs-Kosmin, MD, FACP Attending Physician, Department of Medicine, Division of Rheumatology, Einstein Medical Center; Clinical Assistant Professor of Medicine, Jefferson Medical College of Thomas Jefferson University

Dana Jacobs-Kosmin, MD, FACP is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

J Mark Jackson, MD Clinical Professor of Medicine/Dermatology, Division of Dermatology, University of Louisville School of Medicine

J Mark Jackson, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Kentucky Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Acknowledgements

Mahendra Agraharkar, MD, MBBS, FACP, FASN, Clinical Associate Professor of Medicine, Baylor College of Medicine; President and CEO, Space City Associates of Nephrology

Mahendra Agraharkar, MD, MBBS, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Nephrology, and National Kidney Foundation

Disclosure: South Shore DaVita Dialysis Center Ownership interest

Bruce A Baethge, MD, Faculty, Texas A&M Medical School

Bruce A Baethge, MD is a member of the following medical societies: Alpha Omega Alpha; American College of Physicians; American College of Rheumatology and Arthritis Foundation