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.Sargun Singh Walia M.B.B.S.[3]

Overview

PAN if left untreated, the disease is fatal in most cases. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. Common complications of PAN include Stroke,Kidney failure, heart attack,Intestinal necrosis and perforation. Prognosis is generally good if the treatment is started.Therapy results in remissions or cures in 90% of cases. 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.

Natural History

• PAN if left 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

• Common complications of PAN include:
  • Stroke
  • Kidney failure
  • Heart attack
  • Intestinal necrosis
  • perforation

Prognosis

• Prognosis is generally good if the treatment is started.
• 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%