Evaluation of patients with Primary Ciliary Dyskinesia in terms of humoral immunodeficiency

<bold>Introduction:</bold> Primary ciliary dyskinesia (PCD) and predominantly antibody deficiencies (PAD) are rare diseases that cause recurrent respiratory tract infections. <bold>Aims and objectives:</bold> To examine the coexistence of PCD and PAD in a group of patients with known PCD in a tertiary care hospital. <bold>Methods:</bold> Sixty-two patients were enrolled on the study. Complete blood count, serum immunoglobulin levels, isohemagglutinin levels, serum IgG subgroups and lymphocyte subgroups were evaluated. PAD was defined as a value more than 2 SDs below the normal mean for age. <bold>Results:</bold> The median age was 13.7 years (interquartile range: 8.9–17.5) with equal male/female ratio. The results of 47 (75.9%) patients were normal. None of these patients have been diagnosed as PAD according to ESID criteria. However, abnormal results in the immunological evaluation of the patients were present in 15 (24.1%) patients; 8 with IgG3 subclass deficiency, 3 with IgM deficiency, 2 with IgG deficiency, 1 with IgA deficiency, and 1 with reversed CD4/CD8 ratio. The distribution of immunological results of patients is shown in Figure 1. Antibiotic prophylaxis (trimethoprim-sulfamethoxazole or azithromycin) was started in 4 of these 15 patients. <fig><object-id>erj;64/suppl_68/OA4693/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig> <bold>Conclusions:</bold> Since one-quarter of the study population had immunologic abnormalities, which is higher than reported in the literature, routine immunologic evaluation of patients with PCD would be beneficial.