Chronic granulomatous disease in Japan: Incidence and natural history

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Abstract
Purpose: To analyze the incidence and causes of fatality of patients with chronic granulomatous disease (CGD) in Japan and to provide an opportunity for comparison with reports from other countries. Methods: The Study Group of Phagocytic Disorders in Japan conducted a questionnaire survey on CGD patients in Japan, results of which formed the basis of the study. Results: Clinical details of 221 patients were analyzed: 194 male and 27 female (ratio: 7.2/1), 152 living, 51 dead and 18 unknown. The prevalence of CGD was estimated to be 1/287,709 live births. The fatality rate was 23.1%. The mean age of the surviving patients increased from 8 years 4 months in 1985 to 16 years 0 months in 1998. Although the mean age of death was advanced by 4 years 11 months during the same period of time, the fatality rate has remained practically unchanged during the past 13 years. The number of living adult patients has tended to increase (32.1%). At the time of study, approximately 90% of patients had been placed on sulfamethoxazole-trimethoprim prophylactically or therapeutically, either singly or in combination with other modalities, including interferon-γ, antifungal agents and various antibiotics. Pulmonary infections were responsible for 58.3% of fatalities. Conclusions: With early diagnosis and prompt institution of appropriate therapy, the mean age of CGD patients in Japan has been increasing, but the fatality rate has remained practically unchanged during the last 13 years, mostly due to the fungal infections. Key wordsAspergillus infection, chronic granulomatous disease, questionnaire survey.

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