eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Polyarteritis Nodosa: Follow-up

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

Follow-up

Further Outpatient Care

  • PAN is a chronic disease with diverse neurologic features, which are multifocal and fluctuating. Patients should be educated about the future complications and the risks associated with long-term use of immunosuppressants. Patients are monitored carefully for the following symptoms:
    • Headache
    • Uncontrolled hypertension
    • Dementia
    • Psychosis
    • Encephalopathy
    • Stroke
    • Peripheral neuropathy

Complications

  • Stroke
  • Encephalopathy
  • Myelopathy
  • Heart failure
  • Myocardial infarction
  • Pericarditis
  • Renal failure
  • GI bleeding
  • Bowel infarction
  • Peripheral neuropathy

Prognosis

  • PAN carries a high mortality rate when untreated. Nearly one half of patients die within the first 3 months of onset. Corticosteroid treatment improves the 5-year survival rate to 50-60%. When it is combined with other immunosuppressants, the 5-year survival rate may increase to greater than 80%.

Patient Education

  • The benefits of medical treatments should be discussed clearly with the patient. Many patients will attempt to discontinue their medications after initial symptomatic improvement due to the potential side effects. Patients should understand that PAN is a progressive systemic disease, and that further complications and involvement of other organ systems are quite common. They should be monitored closely for many years to come with appropriate immunosuppressive therapy.

Miscellaneous

Medicolegal Pitfalls

  • Many neurologic and systemic disorders can present with headache. Pathological headaches are often difficult to differentiate from benign headaches solely on clinical grounds. Persisting severe headaches, with or without neurologic deficit, warrant complete neurologic evaluation including brain-imaging studies.
  • Even though stroke is a late complication in many cases, it certainly can occur early. PAN and other vasculitic diseases should be considered in many patients with stroke with multiple foci or combination of hemorrhage and infarction.
  • Evidence of peripheral neuropathy should be sought carefully with history and electromyography (EMG), since it is a common complication of PAN (as many as 60% of patients). Mononeuritis multiplex is the most common form of PAN neuropathy, but other forms can be present.
 


More on Polyarteritis Nodosa

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

References

  1. Alarcon-Segovia D. Polyarteritis nodosa, Churg-Strauss syndrome, and other eosinophilic syndromes. Curr Opin Rheumatol. Feb 1990;2(1):50-4. [Medline].

  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].

  11. Lhote F, Cohen P, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis and Churg-Strauss syndrome. Lupus. 1998;7(4):238-58. [Medline].

  12. Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. Aug 1990;33(8):1088-93. [Medline].

  13. Macovei M, Popa C, Alexianu ME. Particular neurological aspects in vascular autoimmune diseases. II. Cryoglobulinemic vasculitis and polyarteritis nodosa. Neurol Psychiatr (Bucur). Oct-Dec 1989;27(4):249-60. [Medline].

  14. Prescott JE, Johnson JE, Dice WH. Polyarteritis nodosa presenting as seizures. Ann Emerg Med. Oct 1983;12(10):642-4. [Medline].

  15. Travers RL, Allison DJ, Brettle RP, Hughes GR. Polyarteritis nodosa: a clinical and angiographic analysis of 17 cases. Semin Arthritis Rheum. Feb 1979;8(3):184-99. [Medline].

  16. Trepo CG, Zucherman AJ, Bird RC, Prince AM. The role of circulating hepatitis B antigen/antibody immune complexes in the pathogenesis of vascular and hepatic manifestations in polyarteritis nodosa. J Clin Pathol. Nov 1974;27(11):863-8. [Medline].

  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.

  18. Walker GL. Neurological features of polyarteritis nodosa. Clin Exp Neurol. 1978;15:237-47. [Medline].

Further Reading

Keywords

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

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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.