Mortality and morbidity in common variable immunodeficiency.
Common variable immunodeficiency (CVID) is a heterogeneous group of disorders, characterized by hypogammaglobulinemia, defective specific-antibody production resulting in recurrent bacterial infections. Delay in diagnosis and inadequate treatment result in increased irreversible complications and mortality. To determine persistent morbidities, mortality rate and survival in Iranian patients with CVID, hospital records of 72 (39 males and 33 females) diagnosed CVID patients were reviewed. Probabilities of survival after diagnosis of CVID were estimated from Kaplan-Meier life tables. Studied patients were enrolled over a 20-year period (1984-2005). The most commonly observed complication was bronchiectasis (24 cases), followed by splenomegaly, intestinal villous atrophy (11 cases), and failure to thrive (10 cases). Post-diagnosis survival was estimated as 65% for the first 6.5 years, which remains the same until 14 years after diagnosis when the survival curve drops to nearly 45%. The mortality rate among patients who had no regular visits and did not receive periodical IVIG was more remarkable when compared with those who had been followed up timely (p-value = 0.001). The most common cause of death was respiratory failure. Based on our observation, it can be highlighted that all patients with CVID, even under regular immunoglobulin replacement, need close monitoring for early detection of complications and introduction of appropriate management.
Aghamohammadi
Asghar
A
Children's Medical Center, Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran. aghamohammadi@sina.tums.ac.ir
Pouladi
Nima
N
Parvaneh
Nima
N
Yeganeh
Mehdi
M
Movahedi
Masoud
M
Gharagolou
Mohamad
M
Pourpak
Zahra
Z
Rezaei
Nima
N
Salavati
Ali
A
Abdollahzade
Sina
S
Moin
Mostafa
M
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000401:mortality*; D005260:Female; D006801:Humans; D007492:Iran / Q000453:epidemiology; D015993:Life Tables; D008297:Male; D009017:Morbidity; D016016:Proportional Hazards Models; D018709:Statistics, Nonparametric; D011795:Surveys and Questionnaires; D016019:Survival Analysis