Association of HLA-DR Alleles With Pulmonary Cystic Fibrosis

Abstract Background: Cystic fibrosis (CF) is the most common lethal autosomal recessive disease in white Caucasians. It affects many organs including the lung, pancreas, and liver. Whilst CF is a monogenic disease, several studies revealed a complex relationship between genotype and clinical phenotype of diseases. Objective of study: We examined the expression of HLA class II alleles among Iranian CF patients in relation to disease-related bacterial infection. Materials and methods: This study was conducted on 50 CF patients (27 males, 23 females aged 15.5±6.5 years) hospitalized in the Masih Daneshvari hospital in Tehran, Iran and 50 healthy age- and gender-matched control subjects. 5ml whole blood was harvested and after isolation of genomic DNA, HLA-DRB1 subtypes were determined by Single Specific Primer Polymerase Chain Reaction (SSP-PCR) methods. Results: HLA-DRB1*10 was less frequent than HLA-DRB1*04 and HLA-DRB1*11 but none reached significance. 16 CF patients with high serum IgE levels (430.25 ± 219.7 IU/ml) and 27 CF patients positive for Pseudomonas aeruginosa colonization were most closely associated with the HLA-DRB1*04 allele. 31 CF patients had candida Albicans colonization which was most closely associated with HLA-DRB1*11. 3 CF patients had allergic bronchopulmonary aspergillosis (ABPA) and two were diabetic. Discussion: The DR4 and DR11 serotypes that recognize the HLA-DRB1*04 and HLA-DRB1*11 gene products respectively, are not significantly enriched in the Iranian CF population. Further research should be conducted on DR4 and DR11 in CF patients to understand their possible role in infection and IgE expression.Trial registration: The study was approved by Ethical committee of Masih Daneshvari Hospital IR.SBMU.NRITLD.1395.234