T-cell abnormalities in common variable immunodeficiency

Common variable immunodeficiency (CVID) is the most common clinical primary immunodeficiency. It is characterized by a defect in B-cell differentiation to plasma and memory B cells. Moreover, numerous T-cell abnormalities have been reported and include decreased T-cell count and proliferative response, increased T-cell activation and apoptosis, and abnormalities in cytokine production. The aims of this review are to describe phenotypic and functional defects in T cells in CVID patients and to review the literature with respect to the effects of immunoglobulin replacement on the T-cell component in CVID patients (AU)

La inmunodeficiencia común variable (CVID) es la inmunodeficiencia primaria más frecuente. Se caracteriza por un defecto en la diferenciación de linfocitos B hacia células plasmáticas y linfocitos B memoria. Se han descrito numerosas alteraciones en los linfocitos T de estos pacientes, tales como disminución en el número de linfocitos T y en sus respuestas proliferativas, aumento en la activación de células T y en la apoptosis, así como alteraciones en la producción de citokinas. El objetivo de esta revisión es describir las alteraciones funcionales y fenotípicas de los linfocitos T en los pacientes con CVID y revisar la bibliografía en relación a los efectos que la administración de inmunoglobulinas produce en los linfocitos T de los pacientes con CVID (AU)

Luces y sombras de la inmunodeficiencia común variable / No disponible

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Autoimmune Phenotype in Patients With Common Variable Immunodeficiency

Antecedentes y objetivo: Las enfermedades autoinmunes se presentan asociadas, con una alta incidencia, en los pacientes con inmunodeficiencia común variable (IDCV), respecto a la población normal. El objetivo de este estudio fue describir los hechos clínicos del fenotipo autoinmune en pacientes con IDCV. Métodos: Se revisaron las historias clínicas de todos los pacientes diagnosticados de IDCV del Medical Center Hospital de Teherán en el periodo de 2000-2010. Los pacientes fueron clasificados en dos grupos: con y sin enfermedades autoinmunes asociadas. Resultados: De los 52 pacientes estudiados, un 26.9% (14 pacientes) habían mostrado al menos una manifestación de enfermedad autoinmune durante el tiempo del estudio. Las citopenias autoinmunes y la artritis reumatoide juvenil fueron las manifestaciones más frecuentes en nuestra serie. Encontramos en nuestros pacientes asociaciones significativas entre enfermedades infiltrativas polilinfocíticas (p=0.017), incremento de niveles de IgM sérica (p<0.001) y disminución de cifras de IgE (p=0.04) con desarrollo de autoinmunidad, así como una disminución de las células B memoria (p<0.001). Conclusión: La autoinmunidad puede considerarse una de las manifestaciones iniciales en los pacientes con IDCV, por lo que se aconseja explorar el sistema inmunológico humoral mediante test in vitro, en aquellos pacientes con historias de infecciones de repetición. Por otra parte la presencia de enfermedades infiltrativas polilinfocíticas y la disminución de las células B memoria en pacientes con IDCV, pueden predisponer al desarrollo de una enfermedad autoinmune (AU)

Background and objective: Autoimmune disorders occur with a higher incidence in common variable immunodeficiency (CVID) patients than in the general population. To describe the clinical features of the autoimmune phenotype in patients with CVID. Methods: The hospital records of all diagnosed CVID patients referred to the Children’s Medical Center Hospital in Tehran, Iran between 2000 and 2010 were reviewed. Patients were also classified according to the presence or absence of autoimmune disease. Results: Of 52 patients studied, 26.9% (n=14) had shown at least 1 autoimmune manifestation during the study period. Autoimmune cytopenias and juvenile rheumatoid arthritis were the most common form of autoimmunity in our series. Autoimmunity was significantly associated with polyclonal lymphocytic infiltrative disorders (P=.017), increased serum Immunoglobulin (Ig) M levels (P<.001), decreased IgE values (P=.04) and diminished switched memory B-cell count (P<.001). Conclusions: Because autoimmunity is one of the first manifestations in CVID, humoral immune system tests should be considered in autoimmune patients with a history of recurrent infection. The presence of polyclonal lymphocytic infiltrative disorders and decreased switched memory B-cells may predispose CVID patients to autoimmunity (AU)

[Effectiveness of new preventive means for maintaining the functional state of organism in air operators].

The study was designed to assess the psycho-physiological and immune status in 60 male aviation specialists aged 24-45 years given biologically active food additives "Intra" and "Adaptol" It was shown that regular consumption of these additives promoted mobilization of functional reserves of the organism, improved time-related activity of nervous processes, increased lability of the central part of the visual analyzer, and improved results of subjective assessment of postural equilibrium control. Results of the study indicate that food additives "Intra", "Adaptol" possess immunocorrective potential and may be used to restore functions of various organs and their systems in a human operator under extreme conditions.

Recurrent multi-organism keratoconjunctivitis manifesting as a first presentation of common variable immune deficiency (CVID).

Common variable immune deficiency (CVID) is characterized by hypogammaglobulinemia, impaired antibody production, and recurrent bacterial infections. Reports of ocular manifestations of CVID are rare. The authors discuss a patient with recurrent keratoconjunctivitis that presented as an initial manifestation of CVID. The diagnosis of CVID should be entertained where multiple recurrences of keratoconjunctivitis are unresponsive to antibiotic and steroid therapy and when other causes of immune deficiency have been excluded. Intravenous immunoglobulin (IVIG) was used successfully as treatment and is required lifelong as prophylaxis against further episodes of keratoconjunctivitis as well as the other infectious and inflammatory complications of this primary immunodeficiency syndrome.

Reactions of immune system to physical exercises.

The great attention to reactions of immune system to the physical exercises in sportsmen is linked to the growth of training volumes, to the increase of competition numbers and to the elevation of morbidity. Immune deficiency may be considered as the detonator of pathological processes among which acute respiratory diseases (ARD) are investigated most completely in sports medicine. Other pathologies require long-term observations, but it is not so simple to do due to the frequent renewal of sports groups. Besides ARD, there are reports about the growth of cases of poliomyelitis, endotoxemia, allergic and autoimmune disorders. Immune reactions in sportsmen are developed at the background of fever, impaired balance of ergotrophic hormone activity and in a number of cases under conditions of systemic endotoxemia. We have described the extreme type of immune deficiency in sportsmen, in which we could not determine different isotypes of Ig. The phenomenon of Ig disappearance is reproduced under the experimental conditions that opened the way to study its mechanisms. Physical exercises decrease function of immunocompetent cells, their antiviral resistance, antigen presentation and expression of class II MHC molecules. With the involvement of macrophages hyperproduction of IL-6 is developed in muscle tissues. After physical exercises other cytokines also change the state of immunity. Also, neuropeptides getting in touch the links between endocrine and immune systems may make a contribution to immunosuppression. The immunosuppression may be prevented by use of special carbohydrate diets and by administration of complexed preparations. The prophylaxis is capable to control the morbidity, profoundly to increase the training volumes and to enhance the labor efficiency.