Clinical and Demographic Pattern of Chronic Granulomatous Disease (CGD) from a Multicenter Perspective: Malaysia’s Experience Over 26 Years

Abstract Background:A retrospective review of clinical manifestations and demographic pattern of patients diagnosed as chronic granulomatous disease (CGD) from 7 hospitals in Malaysia.Methods: Data from the Malaysia Primary Immunodeficiency Network (MyPIN) with cases of CGD diagnosed from 1991 until 2016 were collated and analysed.Results: Twenty patients were diagnosed as CGD. Males (N=13, 65%) outnumber females (N=7, 35%). The mean age of diagnosis was 3.7 years. There was a positive family history in 40 %. Abscess was the main presenting feature in 16 patients (80%) with one involving the brain (brain abscess). Pneumonia occurred in 10 (50%) with one (1) with complicated bronchiectasis. Catalase-positive bacteria were the most commonly isolated pathogen with Chromobacterium violaceum (C violaceum) predominating, N=5 (25%) with consequent high mortality, N=4 (80%). CGD is commonest amongst the Malays (65%) followed by the Chinese (15.0%), Indians (10.0%) and natives of Borneo (10.0%) reflective of the ethnic composition of the country.Conclusion: This study has shown CGD occurs in the major ethnic groups of Malaysia. To the best of our knowledge, this is the earliest and the largest series of chronic granulomatous disease in South East Asia (SE Asia) which may be reflective of similar clinical pattern in the region. Chromobacterium violaceum infection is associated with a higher mortality in CGD patients in Malaysia. All the CGDs with (C violaceum) infection in this patient series displayed CD4+ (T helper) lymphopenia. We recorded rare clinical manifestation of CGD viz. brain abscess and bronchiectasis.