Single-center study shows reasonable outcomes for patients with CGD who receive medical therapy alone

In a recent article, published in October 2015, in the peer-reviewed journal, Immunology Report, researchers reviewed 27 patients at Ann & Robert H. Lurie Children’s Hospital of Chicago from March 1985 to November 2013 to test for the types and rates of infections in patients with chronic granulomatous disease (CGD).

Out of 23 males and four females, 19 of whom had X-linked CGD and eight were autosomal recessive, researchers found the following:

• The average age of infection was 3.0 years; 2.1 years for X-linked and 5.3 years for autosomal recessive.
• The most frequent infectious agents were Staphylococcus aureus, Serratia, Klebsiella, Aspergillis, and Burkholderia.
• The most common serious infections were pneumonia, abscess, and lymphadenitis.
• Thirteen patients had granulomatous complications.
• Five patients were below the 5th percentile for height, and 4 were below the 5th percentile for weight.
• 24 patients were compliant and maintained on interferon (ACTIMMUNE), trimethoprimsulfamethoxaole (Bactrim), and an azole (usually itraconazole).
• The serious infection rate was .62 per year.
• 23 patients are alive.
• One patient was lost in follow-up; a second patient left the care of the hospital; one died of intractable pneumonia, sepsis, and cerebral edema in 1992; and one patient had a stem-cell transplant and is alive at the time of this writing.

The researchers concluded from this large, single-center U.S. experience that reasonable outcomes for CGD can be expected with medical therapy alone, which includes interferon, trimethoprim-sulfamethoxazole, and an azole.