Immunological and Clinical Phenotyping in Primary Antibody Deficiencies: a Growing Disease Spectrum
| Autor: | Florence Ida Hsu, Daniel Liauw, Eun Jae Chung, Sabrina Z. Siddiqui, Christina Price, Juhyeon Lee, Ryan P. Steele, Junghee J. Shin, Insoo Kang |
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| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
Adult Male Primary Immunodeficiency Diseases Immunology Population Autoimmunity Malignancy medicine.disease_cause Infections Article Pneumococcal Vaccines 03 medical and health sciences Young Adult 0302 clinical medicine Immune system medicine Immunology and Allergy Humans education education.field_of_study Bronchiectasis business.industry Common variable immunodeficiency Interstitial lung disease Middle Aged medicine.disease body regions Killer Cells Natural 030104 developmental biology Common Variable Immunodeficiency Hematologic Neoplasms Immunoglobulin G IgG deficiency Female business 030215 immunology |
| Zdroj: | J Clin Immunol |
| ISSN: | 1573-2592 |
| Popis: | PURPOSE: Although Common Variable Immunodeficiency (CVID) is considered the most prevalent symptomatic primary antibody deficiency (PAD), there is a population with symptomatic PADs that do not meet criteria for CVID. We analyzed clinical and immunological profiles of patients with different PADs to better understand the differences and similarities between CVID and other PADs. METHODS: We extracted clinical and laboratory data of patients with PADs from electronic medical records. Patients were categorized into CVID, IgG subclass 2 deficiency (IgG2D), IgG deficiency (IgGD) and selective antibody deficiency (sAbD) based on basal immunoglobulin levels and pneumococcal vaccine responses. We compared clinical and immunological characteristics in these groups. RESULTS: All patients, regardless of PAD types, showed similar frequencies of infections, bronchiectasis, and interstitial lung disease (ILD). Hematopoietic malignancies were more frequently found in the CVID than in the IgG2D, IgGD and sAbD groups, while the latter groups trended towards an increased frequency of connective tissue diseases (CTD). Low counts of natural killer (NK) cells were associated with malignancy, autoimmunity, and ILD in CVID but not in other PAD groups. CONCLUSIONS: Higher frequency of hematopoietic malignancy in CVID than in the other PADs and association of lower NK cell counts with non-infectious complications in CVID suggest a relationship between immune alterations and the development of non-infectious manifestations in PADs. |
| Databáze: | OpenAIRE |
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