GROUP OF PATHOGENS
CGD may become apparent at any time from infancy to late adulthood; however, most affected individuals are diagnosed before age 5 years.1 Approximately 1 out of every 200,000 people in the United States has CGD.2
Prevention of life-threatening infections is vital for patients with CGD1-6
Life-threatening infections can be difficult to manage and may require lengthy hospitalizations.
Recovery from severe infections in CGD is often complicated by3:
- Increased scar formation
- Wound dehiscence
- Stricture development
The exaggerated inflammatory response of CGD probably contributes to:
- Dysregulated cytokine production4,5
- Decreased apoptosis6
Life span remains well below that of the general population3,7-10
Although survival has increased, infections are still major causes
of mortality for patients with CGD.7
Left untreated, CGD can be fatal3,8
For comparison, the survival curve of the general US
population has been included.
Kaplan-Meier survival curves for patients with X-linked and autosomal recessive CGD3
1. Leiding JW, Holland SM. Chronic granulomatous disease. In: Pagon RA, Adam MP, Ardinger HH, et al, eds. GeneReviews. Seattle, WA: University of Washington, Seattle; 1993-2018. 2. Winkelstein JA, Marino MC, Johnston RB, et al. Chronic granulomatous disease: report on a national registry of 368 patients. Medicine (Baltimore). 2000;79(3):155-169. 3. Kuhns DB, Alvord WG, Heller T, et al. Residual NADPH oxidase and survival in chronic granulomatous disease. N Engl J Med. 2010;363(27):2600-2610. 4. Lekstrom-Himes JA, Kuhns DB, Alvord WG, Gallin JI. Inhibition of human neutrophil IL-8 production by hydrogen peroxide and dysregulation in chronic granulomatous disease. J Immunol. 2005;174(1):411-417. 5. van de Veerdonk FL, Smeekens SP, Joosten LA, et al. Reactive oxygen species–independent activation of the IL-1β inflammasome in cells from patients with chronic granulomatous disease. Proc Natl Acad Sci U S A. 2010;107(7):3030-3033. 6. Kasahara Y, Iwai K, Yachie A, et al. Involvement of reactive oxygen intermediates in spontaneous and CD95 (Fas/APO-1)-mediated apoptosis of neutrophils. Blood. 1997;89(5):1748-1753. 7. Marciano BE, Spalding C, Fitzgerald A, et al. Common severe infections in chronic granulomatous disease. Clin Infect Dis. 2015;60(8):1176-1183. 8. Martire B, Rondelli R, Soresina A, et al. Clinical features, long-term follow-up and outcome of a large cohort of patients with chronic granulomatous disease: an Italian multicenter study. Clin Immunol. 2008;126(2):155-164. 9. Jones LBKR, McGrogan P, Flood TJ, et al. Special article: Chronic granulomatous disease in the United Kingdom and Ireland: a comprehensive national patient-based registry. Clin Exp Immunol. 2008;152(2):211-218. 10. Chronic granulomatous disorder: A guide for medical professionals. CGD Society website. https://cgdsociety.org/wp-content/uploads/2019/05/CGDS-Medical-Guide.pdf. Accessed September 16, 2019.