Common variable immunodeficiency and autoimmunity – an inconvenient truth

Xiao, Xiao; Miao, Qi; Chang, Christopher; Gershwin, M. Eric; Ma, Xiong

doi:10.1016/j.autrev.2014.04.006

Cited by 69 publications

(70 citation statements)

References 62 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…23 Autoantibodies and auto-reactive B cells can be detected in these patients, even if serum immunoglobulins are very low and specific response to various antigens is impaired. 24 Cytopenia is the most common autoimmune manifestation in these patients with CVID 25 and autoimmune thrombocytopenia (ITP) was found in 7 out of 18 patients in our study. One patient with IgG subclass deficiency had celiac disease.…”

Section: Volume 59 • Number 5 Infection Profiles Of Turkish Patients mentioning

confidence: 93%

Defective pneumococcal antibody response in patients with recurrent respiratory tract infections

Erman¹,

Demirtaş

Bildik

et al. 2017

Turk J Pediatr

View full text Add to dashboard Cite

Streptococcus pneumoniae is a common pathogen responsible for pulmonary infections and the leading cause of mortality and morbidity in patients with particularly B cell immunodeficiencies. Antibody production is the principal protective immune response against S. pneumoniae and measurement of the production of antipolysaccharide antibodies is important in the evaluation of B cell deficiencies. We quantified serotype-specific immunoglobulin G antibodies against seven common pneumococcal serotypes before and three weeks after unconjugated vaccine in 416 patients with recurrent respiratory tract infections; fifty-five (13%) of whom showed impaired antibody response. We could evaluate 41 of these 55 patients for their particular clinical features. Specific antibody deficiency, was diagnosed in 10 of these patients, common variable immunodeficiency in 18, ataxia telangiectasia in 10 and other antibody deficiencies in 7 (transient hypogammaglobulinemia in 4, IgG subclass deficiency in 1, partial and selective IgA deficiency in 1) patients. Evaluation of the antibody response to polysaccharide antigens should be considered early on in patients with recurrent respiratory infections and required particularly for the diagnosis of specific antibody deficiency and the decision of the appropriate treatment approaches.

show abstract

Section: Volume 59 • Number 5 Infection Profiles Of Turkish Patients mentioning

confidence: 93%

Defective pneumococcal antibody response in patients with recurrent respiratory tract infections

Erman¹,

Demirtaş

Bildik

et al. 2017

Turk J Pediatr

View full text Add to dashboard Cite

show abstract

“…Autoimmune diseases can occur with increased frequency in CVID [2][3][4]. Pathogenesis of autoimmunity is unknown in CVID patients, although a loss of switched memory B cells, defective elimination of autoimmune B cells and T cell dysfunction have been implicated [10].…”

Section: Discussionmentioning

confidence: 99%

“…Typically, patients present with recurrent bacterial infections of the respiratory and gastrointestinal tract. They may also develop malignancies, inflammatory and autoimmune complications [2][3][4]. The depletion of switched memory B cells as well as attenuation of regulatory T cells have been shown to be associated with autoimmune disease in patients with CVID [5].…”

Section: Introductionmentioning

confidence: 99%

Juvenile Idiopathic Arthritis (JIA) Presenting as Temporomandibular Joint Arthritis in Patients with Common Variable Immunodeficiency (CVID)

Jolanta¹,

Finn²

2015

Pediat Therapeut

View full text Add to dashboard Cite

CVID is the most common clinically encountered primary antibody deficiency disorder and can be associated with autoimmune complications. We report 2 children with CVID who developed joint symptoms several years after initial diagnosis and were referred to a pediatric rheumatologist, who suspected temporomandibular joint arthritis clinically. Both patients underwent MRI imaging with contrast administration, which confirmed temporomandibular arthritis. They were successfully treated with intra-articular steroid joint injections. Juvenile idiopathic arthritis (JIA) can be one of the autoimmune associations with CVID, but temporomandibular arthritis as the primary manifestation is uncommon, difficult to detect clinically and has not been described previously in CVID. Although jaw pain and discomfort on chewing might indicate underlying temporomandibular joint arthritis, these symptoms might not necessarily be present. If not promptly identified and treated, arthritis of JIA can cause joint damage/disability and therefore needs specialist multidisciplinary management under a pediatric rheumatologist. JIA may not respond to steroid injections alone and might require escalation in therapy with systemic immunosuppressive agents. Little is known about the intricacies of systemic immunosuppressive therapy in patients with CVID who develop JIA. Conclusion:In relation to CVID, juvenile idiopathic arthritis (JIA) can be an associated autoimmune manifestation. JIA presenting as temporomandibular joint arthritis as the first manifestation is unusual but can occur and might be potentially difficult to detect clinically. Pediatricians should be aware of the possibility of JIA in a child with CVID and refer to a pediatric rheumatologist for appropriate management. If not treated promptly, JIA can cause joint destruction and disability.

show abstract

“…27 El diagnóstico y el tratamiento de las citopenias autoinmunes son los mismos que en un paciente inmunocompetente, sin embargo, debemos tomar en cuenta los siguientes aspectos al utilizar inmunosupresores:…”

Section: 24unclassified

“…La esplenectomía deberá considerarse como última opción. 27 Linfoproliferación La linfoproliferación no asociada con malignidad,se presenta en 20 a 30 % de los pacientes e incluye la presencia de hepatomegalia, esplenomegalia y linfadenopatías en general.…”

Section: 24unclassified

Inmunodeficiencia común variable en adultos

O’Farrill-Romanillos

Herrera-Sánchez

Hernández-Fernández

et al. 2017

RAM

View full text Add to dashboard Cite

Las inmunodeficiencias primarias (IDP) son enfermedades de baja incidencia, causadas por defectos en genes involucrados en el desarrollo, mantenimiento y regulación del sistema inmune. La inmunodeficiencia común variable (IDCV) es la inmunodeficiencia sintomática más común de la edad adulta. Tiene una prevalencia aproximada de 1 en 25 000-50 000 en la población general, con un retraso en el diagnóstico entre 6-7 años. Las manifestaciones clínicas de la IDCV, constituyen 6 categorías principales: infecciones, complicaciones pulmonares, enfermedad granulomatosa o linfocítica policlonal, autoinmunidad, enfermedades gastrointestinales y neoplasias. La mayoría de los pacientes debe tener al menos una de las siguientes manifestaciones clínicas (infección, autoinmunidad, linfoproliferación). No obstante, el diagnóstico de IDCV puede ser conferido en pacientes asintomáticos especialmente en casos familiares. Las causas secundarias de hipogammaglobulinemia, deben ser descartadas en cualquier paciente que reúna los criterios diagnósticos para IDCV, ya que el tratamiento puede ser totalmente diferente al que se requiere para la IDCV. Debido a que la IDCV comprende un grupo heterogéneo de síndromes con producción deficiente de anticuerpos de tipo primario, el número potencial de entidades dentro de este grupo se desconoce. Los pacientes con IDCV sufren diversas complicaciones que se consideran pronósticas. En ausencia de directrices claras de su búsqueda, se recomienda que durante la evaluación inicial se indague intencionadamente enfermedad linfoproliferativa, enfermedad pulmonar, hepática y autoinmunidad. Los intervalos en los cuales deben realizarse no están claros, pero se pueden requerir varias evaluaciones al año, de acuerdo con la evolución clínica del paciente.

show abstract

Common variable immunodeficiency and autoimmunity – an inconvenient truth

Cited by 69 publications

References 62 publications

Defective pneumococcal antibody response in patients with recurrent respiratory tract infections

Defective pneumococcal antibody response in patients with recurrent respiratory tract infections

Juvenile Idiopathic Arthritis (JIA) Presenting as Temporomandibular Joint Arthritis in Patients with Common Variable Immunodeficiency (CVID)

Inmunodeficiencia común variable en adultos

Contact Info

Product

Resources

About