eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Polyarteritis Nodosa: Differential Diagnoses & Workup

Author: Steve Chung, MD, Residency Director; Director, Clinical Epilepsy Research, Department of Neurology, Assistant Professor of Clinical Neurology, Barrow Neurological Institute
Contributor Information and Disclosures

Updated: Sep 28, 2006

Differential Diagnoses

Amyloid Angiopathy
Cerebral Aneurysms
Dermatomyositis/Polymyositis
Systemic Lupus Erythematosus
Vasculitic Neuropathy

Other Problems to Be Considered

Wegener granulomatosis (WG) involves both pulmonary and renal systems. WG is associated almost exclusively with the cytoplasmic type of antineutrophil cytoplasmic antibody (c-ANCA) but not with the perinuclear type of antineutrophil cytoplasmic antibody (p-ANCA). In PAN, both c- and p-ANCA can be found, although p-ANCA more commonly is associated with the condition.

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 implicated pathogenetic mechanisms, their treatment, and prognosis.

Workup

Laboratory Studies

  • Antineutrophil cytoplasmic antibody (ANCA) test: No diagnostic serologic tests are available for PAN. Positive p-ANCA titers often are found; however, they are not diagnostic.
  • Other tests
    • Serum leukocytes usually are elevated, with neutrophil predominance.
    • Erythrocyte sedimentation rate is almost always elevated.
    • Hypergammaglobulinemia is found in 30% of patients with PAN.

Imaging Studies

  • Arteriogram reveals microaneurysms in the small and medium-sized arteries of the kidneys and abdominal viscera.
  • New MRI techniques, including susceptibility for blood, diffusion, and perfusion-weighted images, make it a very powerful tool to differentiate hemorrhage from potentially reversible ischemia.

Other Tests

  • EEG shows generalized slow wave activity during periods of encephalopathy or toxic delirium.
  • Cerebrospinal fluid findings often are normal.

Procedures

  • Biopsy of small arteries from the abdominal viscera in conjunction with arteriography facilitates identification of the vasculitis.

Histologic Findings

Microscopic polyangiitis is seen with PAN but not exclusively, for it also is observed in WG.

More on Polyarteritis Nodosa

Overview: Polyarteritis Nodosa
Differential Diagnoses & Workup: Polyarteritis Nodosa
Treatment & Medication: Polyarteritis Nodosa
Follow-up: Polyarteritis Nodosa
Multimedia: Polyarteritis Nodosa
References
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References

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  2. Boehm I, Bauer R. Low-dose methotrexate controls a severe form of polyarteritis nodosa. Arch Dermatol. Feb 2000;136(2):167-9. [Medline].

  3. Chudacek Z. Angiographic diagnosis of polyarteritis nodosa of the liver, kidney and mesentery. Br J Radiol. Nov 1967;40(479):864-5. [Medline].

  4. Cohen P, Guillevin L, Baril L, et al. Persistence of antineutrophil cytoplasmic antibodies (ANCA) in asymptomatic patients with systemic polyarteritis nodosa or Churg-Strauss syndrome: follow-up of 53 patients. Clin Exp Rheumatol. Mar-Apr 1995;13(2):193-8. [Medline].

  5. Colmegna I, Maldonado-Cocco JA. Polyarteritis nodosa revisited. Curr Rheumatol Rep. Aug 2005;7(4):288-96. [Medline].

  6. Fujioka M, Bender T, Young LW, Girdany BR. Polyarteritis nodosa in children: radiological aspects and diagnostic correlation. Radiology. Aug 1980;136(2):359-64. [Medline].

  7. Guillevin L, Lhote F, Jarrousse B, et al. Polyarteritis nodosa related to hepatitis B virus. A retrospective study of 66 patients. Ann Med Interne (Paris). 1992;143 Suppl 1:63-74. [Medline].

  8. Guillevin L, Lhote F, Sauvaget F, et al. Treatment of polyarteritis nodosa related to hepatitis B virus with interferon-alpha and plasma exchanges. Ann Rheum Dis. May 1994;53(5):334-7. [Medline].

  9. Guillevin L, Lhote F, Gherardi R. Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: clinical aspects, neurologic manifestations, and treatment. Neurol Clin. Nov 1997;15(4):865-86. [Medline].

  10. Hekali P, Kajander H, Pajari R, et al. Diagnostic significance of angiographically observed visceral aneurysms with regard to polyarteritis nodosa. Acta Radiol. Mar 1991;32(2):143-8. [Medline].

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  17. Von Kummer R, Brandt T, Muller-Kuypers M. Thrombolytic therapy of basilar artery occlusion: preconditions for recanalization and good clinical outcome. In: Yamaguchi T, Mori E, Minematsu K, del Zoppo G, eds. Thrombolytic Therapy in Acute Ischemic Stroke III. Tokyo, Japan: Springer-Verlag; 1995:. 343-8.

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Further Reading

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Keywords

PAN, microscopic polyangiitis, systemic necrotizing vasculitis, arteritis nodosa, Kussmaul disease, Kussmaul's disease, periarteritis nodosa, microscopic polyarteritis, polyarteritis nodosa

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Contributor Information and Disclosures

Author

Steve Chung, MD, Residency Director; Director, Clinical Epilepsy Research, Department of Neurology, Assistant Professor of Clinical Neurology, Barrow Neurological Institute
Steve Chung, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Medical Association, California Medical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Medical Editor

Christopher Luzzio, MD, Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison
Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Howard S Kirshner, MD, Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center
Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association
Disclosure: Boehringer Ingelheim Honoraria Speaking and teaching; BMS/Sanofi Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Novartis Consulting fee Review panel membership

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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