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 and 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%