Polyarteritis nodosa

Last updated: December 8, 2022

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Polyarteritis nodosa (PAN) is a systemic vasculitis of medium-sized vessels that most commonly affects the skin, peripheral nerves, muscles, joints, gastrointestinal tract, and kidneys, but usually spares the lungs. Most cases are idiopathic, but PAN is associated with certain viral infections, most commonly hepatitis B virus (HBV) infection. Patients typically present with constitutional symptoms (e.g., fever, weight loss, muscle and joint pain); additional symptoms vary based on the organ of involvement (e.g., acute kidney injury, myocardial infarction, rash). ANCAs and cryoglobulins are typically negative on laboratory studies. The diagnosis is confirmed via biopsy or visceral angiography of the affected organs. Management typically involves immunosuppressive agents (e.g., glucocorticoids). For patients with HBV-associated PAN, antiviral therapy and, in selected cases, plasmapheresis, are also indicated.

Epidemiological data refers to the US, unless otherwise specified.

In PAN, the Pulmonary Artery is Not involved, PANmural inflammation of the arterial wall is present, and PAN may be associated with hePAtitis B.

PAN should be considered in adults < 65 years of age presenting with stroke or myocardial infarction.

General principles [2][4][5]

Laboratory studies [2][5]

Imaging studies [6][7]

Biopsy of affected tissue [2]

Most guideline recommendations are based on low-level evidence or expert opinion. Use shared decision-making. [2][5][7]

General principles

Plasmapheresis may be considered for patients with life-threatening PAN refractory to pharmacotherapy and those with HBV-associated PAN. [7]

Pharmacotherapy [7]

Some patients achieve remission with glucocorticoid monotherapy, however, the addition of glucocorticoid-sparing agents reduces the dose of glucocorticoids required, reducing the potential adverse effects. [7]

HBV-associated PAN [2][5][7]

The addition of plasmapheresis increases the rate of remission induction in patients with HBV-associated PAN. [5][7]

Supportive care

  1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis & Rheumatism. 2012; 65 (1): p.1-11. doi: 10.1002/art.37715 . | Open in Read by QxMD
  2. Hernández-Rodríguez J, Alba MA, Prieto-González S, Cid MC. Diagnosis and classification of polyarteritis nodosa. J Autoimmun. 2014; 48-49 : p.84-89. doi: 10.1016/j.jaut.2014.01.029 . | Open in Read by QxMD
  3. Ellen C. Ebert, Klaus D. Hagspiel, Michael Nagar, Naomi Schlesinger. Gastrointestinal Involvement in Polyarteritis Nodosa. Clinical Gastroenterology and Hepatology. 2008 .
  4. Guggenberger KV, Bley TA. Imaging in Vasculitis. Curr Rheumatol Rep. 2020; 22 (8). doi: 10.1007/s11926-020-00915-6 . | Open in Read by QxMD
  5. Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2008; 68 (3): p.310-317. doi: 10.1136/ard.2008.088096 . | Open in Read by QxMD
  6. Hughes LB, Bridges SL. Polyarteritis nodosa and microscopic polyangiitis: Etiologic and diagnostic considerations. Curr Rheumatol Rep. 2002; 4 (1): p.75-82. doi: 10.1007/s11926-002-0027-8 . | Open in Read by QxMD
  7. Chung SA, Gorelik M, Langford CA, et al. American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa. Arthritis Care Res. 2021; 73 (8): p.1061-1070. doi: 10.1002/acr.24633 . | Open in Read by QxMD
  8. Chung SA, Langford CA, Maz M, et al. American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis. Arthritis Care Res. 2021; 73 (8): p.1088-1105. doi: 10.1002/acr.24634 . | Open in Read by QxMD

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