Keywords
Humoral immunodeficiencies
Bruton agammaglobulinemia
Common variable immunodeficiency
Abstract
Background: Primary immunodeficiencies (PID) are characterized by alteration of the components of the immune system. Humoral deficiencies represent 50%. The most common are selective IgA deficiency, Bruton agammaglobulinemia, and common variable immunodeficiency (CVID).
Objective: To describe the epidemiological and clinical characteristics of adults with humoral PID, cared for in a Primary Humoral Immunodeficiencies Clinic.
Methods: A descriptive cross-sectional study that included a year of analysis, including 35 patients with humoral PID, 31 with CVID, and 4 with Bruton agammaglobulinemia. Data were analyzed with descriptive statistics.
Results: Of 35 patients studied, 31 had CVID (88.5%) and 4 (11.5%) Bruton agammaglobulinemia; 21 were men and 14 women. The age at onset of symptoms was 22.7 years, and the delay in diagnosis was 7.2 years. 11.4% of CVID patients died during the study; 4 had malignancies, 22.8% autoimmune diseases, and 48.5% gastrointestinal disorders. Patients with Bruton agammaglobulinemia presented no comorbidities.
Conclusions: Unlike reports in the literature, in the study group, CVID was the most common cause of humoral PID, predominantly in men; the most common gastrointestinal disorder was intestinal functional disorder.
References
Bonilla FA, Khan DA, Ballas ZK, Chinen J, Frank MM, Hsu JT, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015;136:1186-1205. doi: 10.1016/j.jaci.2015.04.049.
McCusker C, Warrington R. Primary Immunodeficiency. Allergy Asthma Clin Immunol. 2011;7 Suppl 1: S11. doi: 10.1186/1710-1492-7-S1-S11.
Bousfiha A, Jeddane L, Al-Herz W, Ailal F, Casanova JL, Chatila T, et al. The 2015 IUIS Phenotypic classification for primary immunodeficiencies. J Clin Immunol. 2015;35:727-738.
Coria-Ramírez E, Espinosa-Padilla S, Espinosa-Rosales F, Vargas-Camaño ME, Blancas-Galicia L. Panorama epidemiológico de las inmunodeficiencias primarias en México. Rev Alergia Mex. 2010;57(5):159-163.
Giorgetti O, Paolini M, Oleastro M, Fernández-Romero DS. Agammaglobulinemia ligada al cromosoma X en adultos. Evolución clínica. Medicina. 2016;76:65-70.
Agarwal S, Mayer L. Diagnosis and treatment of gastrointestinal disorders in patients with primary immunodeficiency. Clin Gastroenterol Hepatol. 2013;11(9):1050-1063. doi: 10.1016/j.cgh.2013.02.024
Cunningham-Rundles C. Autoimmunity in primary immune deficiency: taking lessons from our patients. Clin Exp Immunol. 2011;164(Suppl2):6-11. doi: 10.1111/j.1365-2249.2011.04388.x
Coraglia A, Galassi N, Fernández-Romero DS, Juri MC, Felippo M, Malbrán A, et al. Common variable Immunodeficiency and circulating TFH. J Immunol Res. 2016;2016:4951587. doi: 10.1155/2016/4951587
Tam J, Routes M. Common variable immunodeficiency. Am J Rhinol Allergy. 2013;27:260-265. doi: 10.2500/ajra.2013.27.3899
Ameratunga R, Brewerton M, Slade C, Jordan A, Gillis D, Steele R, et al. Comparison of diagnostic criteria for common variable immunodeficiency disorder. Front Immunol. 2014;5:415. doi: 10.3389/ fimmu.2014.00415
Bonilla F, Barian I, Chapel H, Costa-Carvalho BT, Cunningham-Rundles C, de la Morena MT, et al. International Consensus Document (ICON): common variable Immunodeficiency disorders. J Allergy Clin Immunol Pract. 2016:4(1):38-59. doi: 10.1016/j.jaip.2015.07.025
Galindo-Pacheco LV, Amaya-Mejía AS, O’Farrill-Romanillos PM, Del Rivero-Hernández LG, Segura- Méndez NH. Calidad de vida en adultos con Inmunodeficiencia común variable y bronquiectasias. Rev Alergia Mex. 2013;60:123-128.
Uzzan M, Ko HM, Mehandru S, Cunningham-Rundles C. Gastrointestinal disorders asocciated with common variable immunodeficiency (CVID) and chronic granulomatous disease (CGD). Curr Gastroenterol Rep. 2016;18(4):17. doi: 10.1007/s11894-016-0491-3
Rodríguez-Negrete EV, Mayoral-Zavala A, Rodríguez-Mireles KA, Díaz de León-Salazar OE, et al. Prevalencia de alteraciones gastrointestinales en adultos con inmunodeficiencia común variable en el Hospital de Especialidades Dr. Bernardo Sepúlveda. Rev Alergia Mex. 2015;62:1-7.
Pituch-Noworolska A, Siedlar M, Kowalczyk D, Szaflarska A, Błaut-Szlósarczyk A, Zwonarz K. Thrombocytopenia in common variable immunodeficiency patients: clinical course, management, and effect of immunoglobulins. Centr Eur J Immunol. 2015;40(1):83-90. doi: 10.5114/ceji.2015.5083
O’Farrill-Romanillos PM, Campos-Romero FH, Mendoza-Reyna LD, Amaya-Mejía AS, Galindo-Pacheco LV, González-Virla B, et al. Enfermedades hematológicas autoinmunes en adultos con inmunodeficiencia común variable. Rev Alergia Mex. 2012;59(4):187-191.
López-Rocha E, Rodríguez-Mireles K, Segura-Méndez NH, Yamazaki-Nakashimada MA. Prevalencia de cáncer en pacientes adultos con inmunodeficiencia común variable. Rev Alergia Mex. 2015;62:22- 27.
Jolles S. The variable in common variable immunodeficiency: a disease of complex phenotypes. J Allergy Clin Immunol Pract. 2013;1:545-556. doi: 10.1016/j.jaip.2013.09.015
Cunningham-Rundles C, Maglione P. Common variable immunodeficiency. J Allergy Clin Immunol. 2012;129(5):1426-1428.
Resnick E, Moshier E, Godbold J, Cunningham-Rundles C. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 2012;119(7):1650-1657. doi: 10.1182/blood-2011-09-377945
Rodríguez-Mireles KA, Galguera-Sauceda A, Gaspar-López A, López-Rocha EG, Campos-Romero FH, Del Rivero-Hernández LG, et al. Efectos adversos de la aplicación ambulatoria de inmunoglobulina intravenosa en adultos con inmunodeficiencia común variable. Rev Alergia Mex. 2014;61:131-140.
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Copyright (c) 2016 Revista Alergia México