Clinical and immunological features of common variable immunodeficiency in Mexican patients
Por:
Ramírez-Vargas N., Arablin-Oropeza S.E., Mojica-Martínez D., Yamazaki-Nakashimada M.A., De la Luz García-Cruz M., Terán-Juárez L.M., Cortés-Grimaldo R.M., Torres-Lozano C., Madrigal-Beas I., Ortega-Cisneros M., Vargas-Camaño M.E., Staines-Boone T., Pietropaolo-Cienfuegos D., Berrón-Ruiz L., Espinosa-Rosales F.J., Guevara-Cruz M., Blancas-Galicia L.
Publicada:
1 ene 2014
Categoría:
Immunology and Allergy
Resumen:
Background: Common variable immunodeficiency (CVID) is characterised by hypogammaglobulinaemia and a broad clinical spectrum, mainly showing recurrent bacterial infections accompanied sometimes by increased susceptibility to chronic lung disease, autoimmunity, and neoplastic diseases. Objectives: To evaluate the clinical and immunological characteristics of patients with CVID in Mexico. Methods: This is a retrospective analysis of 43 patients with CVID from the Immunology Division of seven different reference centres in Mexico. Patients were diagnosed according to the diagnostic criteria of the European Society for Immunodeficiency Diseases. We collected demographics, clinical and immunological data from each patient and a statistical analysis was performed. Results: There were 23 (53.5%) male and 20 (46.5%) female patients. Median age at onset of disease was 13.7 years, and median age at diagnosis was 19 years. Average delay in diagnosis was 12.5 years. The median total serum levels of IgG, IgM, and IgA at diagnosis were 175, 18, and 17.8. mg/dL, respectively. The mean percentage of CD19+ B cells was 8.15%. Sinusitis (83%), pneumonia (83%), gastrointestinal infection (70%), and acute otitis media (49%) were the most common manifestations. Bronchiectasis was present in 51% of the patients, 44% manifested non-infectious chronic diarrhoea, and 70% experienced weight loss. Autoimmunity was present in 23% of the patients; haemolytic anaemia and autoimmune thrombocytopenic purpura were the most common presentations. Allergy was present in 30.2% of patients, with allergic rhinitis and asthma being the most frequent types. Two patients developed malignancy. All the patients received Intravenous immunoglobulin (IVIG) as a fundamental part of the treatment at a mean dose of 408. mg/kg. Conclusion: This is the first cohort of CVID reported in Mexico We found that infection diseases were the most frequent presentations at onset. Moreover, patients had an average diagnosis delay of twelve years and thus a major prevalence of bronchiectasis. We suggest performing an extended analysis of patients with CVID patients in other Latin American countries. © 2012 SEICAP.
Filiaciones:
Ramírez-Vargas N.:
Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, MX City, Mexico
Arablin-Oropeza S.E.:
Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, MX City, Mexico
Mojica-Martínez D.:
Centro Médico Nacional, La Raza, IMSS, MX City, Mexico
Yamazaki-Nakashimada M.A.:
Servicio de Inmunología, Instituto Nacional de Pediatría, MX City, Mexico
De la Luz García-Cruz M.:
Departamento de Alergia, Instituto Nacional de Enfermedades Respiratorias, MX City, Mexico
Terán-Juárez L.M.:
Departamento de Alergia, Instituto Nacional de Enfermedades Respiratorias, MX City, Mexico
Cortés-Grimaldo R.M.:
Clínica 180 del IMSS, Guadalajara, Mexico
Torres-Lozano C.:
Hospital de Especialidades del Centro Médico Nacional de Occidente, IMSS, Guadalajara, Mexico
Madrigal-Beas I.:
Hospital de Especialidades del Centro Médico Nacional de Occidente, IMSS, Guadalajara, Mexico
Ortega-Cisneros M.:
Hospital de Especialidades del Centro Médico Nacional de Occidente, IMSS, Guadalajara, Mexico
Vargas-Camaño M.E.:
Centro Médico Nacional 20 de Noviembre, ISSSTE, MX City, Mexico
Staines-Boone T.:
Unidad Médica de Alta Especialidad #25 IMSS, Monterrey N.L., Mexico
Pietropaolo-Cienfuegos D.:
Servicio de Alergia e Inmunología, Hospital Infantil de Mexico, MX City, Mexico
Berrón-Ruiz L.:
Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, MX City, Mexico
Espinosa-Rosales F.J.:
Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, MX City, Mexico
Dirección de investigación, Instituto Nacional de Pediatría, MX City, Mexico
Guevara-Cruz M.:
Departamento de Fisiologia de la Nutrición, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico
Blancas-Galicia L.:
Unidad de Investigación en Inmunodeficiencias, Instituto Nacional de Pediatría, MX City, Mexico
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