Polyarteritis nodosa natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Haritha Machavarapu, M.B.B.S.

Natural History

• Untreated, the disease is fatal in most cases.
• The most serious associated conditions generally involve the kidneys and gastrointestinal tract.
• Without treatment, the outlook is poor.

Complications

• Complications include the following:
  • Stroke
  • Kidney failure
  • Heart attack
  • Intestinal necrosis
  • perforation

Prognosis

• Therapy results in remissions or cures in 90% of cases.
• Current treatments using steroids and other drugs that suppress the immune system (such as cyclophosphamide) can improve symptoms and the chance of long-term survival.
• Guillevin and coworkers have described five prognostic factors that predict high probability of mortality and are considered indications for another immunosuppressive drug in addition to prednisone.

1. Proteinuria >1g/day
2. Azotemia
3. Cardiomyopathy
4. Gastrointestinal involvement
5. Central nervous system disease With none of these factors, 5-year mortality is 12%. With 2 or more 5-year mortality is 46%