Gholamreza Azizi1,2,3,4, Marzieh Tavakol1,5, Hosein Rafiemanesh6,7, Fatemeh Kiaee3, Reza Yazdani3, Amin Heydari3, Kosar Abouhamzeh3, Pardis Anvari3, Sara Mohammadikhajehdehi3, Laleh Sharifia3, Yasser Bagheri8,9, Hamed Mohammadi10, Hassan Abolhassani3,4,11, Asghar Aghamohammadi3,4. 1. a Non-Communicable Diseases Research Center , Alborz University of Medical Sciences , Karaj , Iran. 2. b Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital , Alborz University of Medical Sciences , Karaj , Iran. 3. c Research Center for Immunodeficiencies, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran. 4. d Primary Immunodeficiency Diseases Network (PIDNet) , Universal Scientific Education and Research Network (USERN) , Tehran , Iran. 5. e Department of Allergy and Clinical Immunology, Shahid Bahonar Hospital , Alborz University of Medical Sciences , Karaj , Iran. 6. f Students' Research Committee, School of Public Health , Shahid Beheshti University of Medical Sciences , Tehran , Iran. 7. g Department of Epidemiology, School of Public Health , Shahid Beheshti University of Medical Sciences , Tehran , Iran. 8. h Student Research Committee , Golstan University of Medical Sciences , Gorgan , Iran. 9. i Department of Allergy and Clinical Immunology , Iran University of Medical Sciences , Tehran , Iran. 10. j Department of Immunology, School of Medicine , Tabriz University of Medical Sciences , Tabriz , Iran. 11. k Division of Clinical Immunology, Department of Laboratory Medicine , Karolinska Institute at Karolinska University Hospital Huddinge , Stockholm , Sweden.
Abstract
OBJECTIVES: The aim of this study was to evaluate the frequency of autoimmunity in primary antibody deficiency (PAD). METHODS: A total of 471 patients with PADs enrolled in this retrospective cohort study. For all patients' demographic information, clinical records and laboratory data were collected to investigate autoimmune complications. RESULTS: Autoimmune disorders as the first presentation of immunodeficiency were recorded in 11 patients (2.5%). History of autoimmunity was recorded in 125 patients during the course of the disease (26.5%). The frequency of autoimmunity in common variable immune deficiency (32.0%) was higher than other forms of PADs. The most common autoimmune manifestations were reported to be autoimmune gastrointestinal disease and autoimmune cytopenias. Among patients with autoimmunity, 87 patients (69.6%) had a history of one autoimmune disorder, while 38 patients (30.4%) had a history of multiple autoimmunities. The immune thrombocytopenic purpura and autoimmune hemolytic anemia were the most two concomitant autoimmune disorders in 16 (42.1%) of 38 patients with multiple autoimmunities. Comparing the frequency of Tregs in PAD patients with autoimmunity showed that, patients with multiple autoimmunities had lower Tregs than those with single autoimmunity (p = 0.017). CONCLUSION: It is important that non-immunologist physicians be alert of the associated autoimmunity with PADs in order to reduce the diagnostic delay and establish timely immunoglobulin replacement therapy in these patients.
OBJECTIVES: The aim of this study was to evaluate the frequency of autoimmunity in primary antibody deficiency (PAD). METHODS: A total of 471 patients with PADs enrolled in this retrospective cohort study. For all patients' demographic information, clinical records and laboratory data were collected to investigate autoimmune complications. RESULTS:Autoimmune disorders as the first presentation of immunodeficiency were recorded in 11 patients (2.5%). History of autoimmunity was recorded in 125 patients during the course of the disease (26.5%). The frequency of autoimmunity in common variable immune deficiency (32.0%) was higher than other forms of PADs. The most common autoimmune manifestations were reported to be autoimmune gastrointestinal disease and autoimmune cytopenias. Among patients with autoimmunity, 87 patients (69.6%) had a history of one autoimmune disorder, while 38 patients (30.4%) had a history of multiple autoimmunities. The immune thrombocytopenic purpura and autoimmune hemolytic anemia were the most two concomitant autoimmune disorders in 16 (42.1%) of 38 patients with multiple autoimmunities. Comparing the frequency of Tregs in PAD patients with autoimmunity showed that, patients with multiple autoimmunities had lower Tregs than those with single autoimmunity (p = 0.017). CONCLUSION: It is important that non-immunologist physicians be alert of the associated autoimmunity with PADs in order to reduce the diagnostic delay and establish timely immunoglobulin replacement therapy in these patients.
Authors: Ida Judyta Malesza; Michał Malesza; Iwona Krela-Kaźmierczak; Aleksandra Zielińska; Eliana B Souto; Agnieszka Dobrowolska; Piotr Eder Journal: Int J Mol Sci Date: 2020-07-23 Impact factor: 5.923
Authors: Pasquale Comberiati; Giorgio Costagliola; Niccolò Carli; Annalisa Legitimo; Sofia D'Elios; Rita Consolini; Diego G Peroni Journal: Front Immunol Date: 2019-07-17 Impact factor: 7.561