What is the prognosis of chronic granulomatous disease (CGD)?

Updated: Jul 29, 2019
  • Author: Roman J Nowicki, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Answer

The long-term survival of patients who develop symptoms after the end of the first year of life is significantly better than that of patients whose illness starts in infancy. Survival rates are variable but improving; approximately 50% of patients survive to age 30-40 years. Infections are less common in adults than in children, but the propensity for severe life-threatening bacterial infections persists throughout life.

Fungal infections remain a major determinant of survival in chronic granulomatous disease (CGD). Morbidity secondary to infection or granulomatous complications remains significant for many patients, particularly those with the X-linked form. X-linked patients generally have more severe disease, and this is generally in those with lower residual superoxide production. Survival in CGD has increased over the years, but infections are still major causes of morbidity and mortality. [18] Currently, the annual mortality rate is 1.5% per year for persons with autosomal recessive CGD and 5% for those with X-linked CGD.

Since the advent of prophylactic antibiotics, antifungals, and interferon-gamma (INF-gamma), the prognosis for patients with CGD has improved. Patients living to their 30s and 40s is now common.

Patients with CGD and modest residual production of reactive oxygen intermediates (ROIs) have significantly less severe illness and a greater likelihood of long-term survival than patients with little residual ROI production. The production of residual ROI is predicted by the specific NADPH oxidase mutation, regardless of the specific gene affected, and is a predictor of survival in patients with CGD. [19]


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