Сhronic granulomatous disease

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Bridges–Good syndrome
Сhronic granulomatous
disease

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CGD affects
about 1 in 200,000 people in the United State, with about 20 new cases
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diagnosed each year.
Classically, patients with chronic granulomatous disease will have recurrent bouts of
infection due to the decreased capacity of their immune system to fight off disease-causing
organisms. The recurrent infections they acquire are specific and are, in decreasing order of
frequency:
pneumonia
abscesses of the skin, tissues, and organs
septic arthritis
osteomyelitis
bacteremia/fungemia
superficial skin infections such as cellulitis or impetigo
Most people with CGD are diagnosed in childhood, usually, before age 5.

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Treatment
Managementzof chronic granulomatous disease revolves around two goals: 1) diagnose the disease
early so that antibiotic prophylaxis can be given to keep an infection from occurring, and 2) educate the
patient about his or her condition so that prompt treatment can be given if an infection occurs.
Antibiotics
Physicians often prescribe the antibiotic trimethoprim-sulfamethoxazole to prevent bacterial infections.
This drug also has the benefit of sparing the normal bacteria of the digestive tract. Fungal infection is
commonly prevented with itraconazole, although a newer drug of the same type
called voriconazole may be more effective. The use of this drug for this purpose is still under scientific
investigation.
Immunomodulation
Interferon, in the form of interferon gamma-1b (Actimmune) is approved by the Food and Drug
Administration for the prevention of infection in CGD. It has been shown to reduce infections in CGD
patients by 70% and to decrease their severity. Although its exact mechanism is still not entirely
understood, it has the ability to give CGD patients more immune function and therefore, greater ability
to fight off infections. This therapy has been the standard treatment for CGD for several years.[30]
Hematopoietic stem cell transplantation (HSCT)
Hematopoietic stem cell transplantation from a matched donor is curative although not without
significant risk.