Gastrointestinal Manifestations in Patients with Common Variable Immunodeficiency

Kazemi, Ahmad; Aghamohammadi, Asghar; Parvaneh, Nima; Rezaei, Nima; Mahjoob, Fatemeh; Bashashati, Mohammad; Movahedi, Masoud; Fazlollahi, Mohammad Reza; Zandieh, Fariborz; Roohi, Zahra; Abdollahzade, Sina; Salavati, Ali; Kouhi, Ali; Talebpour, Bahram; Daryani, Nasser Ebrahimi

doi:10.1007/s10620-006-9736-6

Cited by 111 publications

(92 citation statements)

References 30 publications

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“…Affected patients are very susceptible to recurrent infection because of low levels of Ig in serum and to reduced specific antibody responses to protein and polysaccharide antigens [3][4][5][6]. Patients may also experience a wide variety of clinical complications, including autoimmunity, inflammation, lymphoid malignancy, granuloma, and enteropathy, all of which are included in recent classifications of CVID into clinical phenotypes with different prognoses [7][8][9][10][11].…”

Section: Common Variable Immunodeficiency (Cvid) Is the Most Common Cmentioning

confidence: 99%

T-Cell Abnormalities in Common Variable Immunodeficiency

Azizi

Rezaei

Kiaee

et al. 2016

J Investig Allergol Clin Immunol

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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. Key words: Common variable immunodeficiency. T cell. Helper T cells. Regulatory T cells. ResumenLa 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. Palabras clave: Inmunodeficiencia común variable. Células T. Células T helper. Células T reguladoras.

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Section: Common Variable Immunodeficiency (Cvid) Is the Most Common Cmentioning

confidence: 99%

T-Cell Abnormalities in Common Variable Immunodeficiency

Azizi

Rezaei

Kiaee

et al. 2016

J Investig Allergol Clin Immunol

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“…Many of the inflammatory disorders mimic the classic forms of the disease (in the absence of immunodeficiency) such as coeliac disease, inflammatory bowel disease and pernicious anaemia [3,48,[71][72][73][74], but differ in the pathogenesis and are unresponsive to Ig treatment. Different explanations may justify the occurrence of these conditions in patients with antibody deficiencies despite appropriate Ig replacement therapy.…”

Section: Benefit In Gastrointestinal Diseasementioning

confidence: 99%

Immunoglobulin treatment in primary antibody deficiency

Maarschalk-Ellerbroek

Hoepelman

Ellerbroek

2011

International Journal of Antimicrobial Agents

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The primary antibody deficiency syndromes are characterised by recurrent respiratory tract infections and the inability to produce effective immunoglobulin (Ig) responses. The best known primary antibody deficiencies are common variable immunodeficiency (CVID), X-linked agammaglobulinaemia (XLA), immunoglobulin G (IgG) subclass deficiency, and selective antibody deficiency with normal immunoglobulins (SADNI).Therapy in these patients consists of prophylactic antibiotics and/or Ig replacement therapy. Diagnostic delay remains common owing to limited awareness of the presenting features and may result in increased morbidity and mortality. Replacement therapy with immunoglobulins increases life expectancy and reduces the frequency and severity of infections, but the effect on end-organ damage is still unknown. Both intravenous immunoglobulin (IVIg) and subcutaneous immunoglobulin (SCIg) treatment appear to be safe, with comparable efficacy. A starting dose of 300-400 mg/kg/month in IVIg and 100 mg/week for SCIg is recommended. IgG trough levels should be >5 g/L for patients with agammaglobulinaemia and 3 g/L greater than the initial IgG level for patients with CVID; however, the clinical response should be foremost in choosing the dose and trough level. Infusion-related adverse reactions are generally mild owing to improved manufacturing processes. In this paper, aspects of Ig replacement therapy in primary antibody-deficient patients will be addressed.

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“…Gastrointestinal symptoms or intestinal lesions are frequently seen (up to 50%) in patients with CVID [4,12], diarrhoea, abdominal pain, weight loss and anaemia being the most common [5][6][7][8]. In 30% of cases, gastrointestinal involvement was demonstrated to be the result of infestations with Giardia lamblia, a protozoan parasite whose presence in the small intestine of these patients is facilitated by the absence of secretory immunoglobulin (Ig) A, a hallmark in most patients with CVID.…”

Section: Discussionmentioning

confidence: 99%