scholarly journals Clinical and Immunological Features of 65 Iranian Patients with Common Variable Immunodeficiency

2005 ◽  
Vol 12 (7) ◽  
pp. 825-832 ◽  
Author(s):  
Asghar Aghamohammadi ◽  
Abolhasan Farhoudi ◽  
Mostafa Moin ◽  
Nima Rezaei ◽  
Ali Kouhi ◽  
...  
Keyword(s):  
Otitis Media ◽  
Common Variable Immunodeficiency ◽  
Bacterial Infections ◽  
Diagnostic Delay ◽  
Primary Immunodeficiency Disease ◽  
Serum Immunoglobulin ◽  
Recurrent Infections ◽  
Baseline Serum ◽  
Common Variable

ABSTRACT Common variable immunodeficiency (CVID) is a primary immunodeficiency disease characterized by hypogammaglobulinemia and recurrent bacterial infections. The records of 65 patients with CVID (37 males and 28 females) in the age range of 24 to 537 months were reviewed. By the year 2003, 11 patients had died and seven patients could not be located. The total follow-up period was 221 patient-years. The median diagnostic delay (time between onset and diagnosis) in our patient group was 60 months. At the time of diagnosis, the baseline serum immunoglobulin G (IgG), IgM, and IgA levels were below the level normal for the patients' age; the medians for this group were 120, 10, and 0 mg/dl, respectively. All of the patients presented with infectious diseases at the time of onset, the most common of which were otitis media, diarrhea, pneumonia, and sinusitis. Acute and recurrent infections were also found in almost all of the patients, particularly involving respiratory and gastrointestinal systems. The most common infections, before diagnosis and during follow-up, were pneumonia, acute diarrhea, acute sinusitis, and otitis media. CVID should be considered in any patient with a history of recurrent infections and decreased levels of all serum immunoglobulin isotypes.

Chronic urticaria and common variable immunodeficiency (CVID): an association to remember

2021 ◽  
Vol 14 (2) ◽  
pp. e239733
Author(s):  
Claudia Villatoro Santos ◽  
Michael Yacoub
Keyword(s):  
Otitis Media ◽  
Chronic Urticaria ◽  
Common Variable Immunodeficiency ◽  
Caucasian Woman ◽  
Recurrent Infections ◽  
Modest Improvement ◽  
Hiv Test ◽  
Oral Steroids ◽  
History Of ◽  
Common Variable

The patient is a 64-year-old Caucasian woman with idiopathic chronic urticaria who presented to her primary care physician’s office with mucoid otitis media. Medical history was significant for hypertension, hyperlipidaemia, allergic rhinitis, pre-diabetes, gastro-oesophageal reflux, paroxysmal atrial fibrillation, chronic kidney disease, diverticulosis with prior diverticulitis and history of recurrent infections. Her chronic urticaria was initially treated with antibiotics, antihistamines and oral steroids, but later she developed refractory urticaria requiring dapsone with modest improvement. When she presented with mucoid otitis media, immunoglobulin levels were found to be decreased. The pneumococcal vaccine antibody challenge confirmed the diagnosis of common variable immunodeficiency (CVID). Her HIV test was negative. She was started on intravenous immunoglobulin infusions and her chronic urticaria stabilised.In patients with chronic urticaria refractory to treatment, especially with a history of recurrent infections, a diagnosis of CVID should be considered, regardless of HIV status.

scholarly journals Common variable immunodeficiency disorder associated with bronchiectasis: a case report

2021 ◽  
Vol 8 (3) ◽  
pp. 565
Author(s):  
Mohammad Zahirul Islam Khan ◽  
Kamrul Laila ◽  
Mohammed Mahbubul Islam ◽  
Mohammad Imnul Islam ◽  
Shahana Akhter Rahman
Keyword(s):  
Health Care Providers ◽  
Common Variable Immunodeficiency ◽  
Delayed Diagnosis ◽  
Gene Mutations ◽  
Growth Failure ◽  
Recurrent Infections ◽  
Care Providers ◽  
Immunodeficiency Disorder ◽  
Specific Antibody Production ◽  
Common Variable

Common variable immunodeficiency disorder (CVID) is the commonest type of primary immunodeficiency disorders (PIDs) characterized by hypogammaglobulinemia, defective specific antibody production and increased susceptibility of recurrent infections. Autoimmunity, neoplasm and lymphoproliferative disorders are usually associated with CVID. In most cases, the cause is unknown, but multiple gene mutations (10%) may be associated with CVID. Here, we report an eight years old girl with CVID presented with recurrent infections, growth failure, generalized lymphadenopathy and hepatosplenomegaly. Chest examination and radiological findings of this girl were consistent with bronchiectasis. Lack of awareness among health care providers is the reason for delayed diagnosis of several years for this girl. Therefore, it is essential to raise awareness regarding PID patients among the physicians to improve the quality of life.  

scholarly journals 597 Long Term Follow-up of Patients with Common Variable Immunodeficiency (Cvid) in Rio De Janeiro, Brazil

2012 ◽  
Vol 5 ◽  
pp. S189
Author(s):  
Amanda Seba ◽  
Norma de Paula Motta Rubini ◽  
Albertina Varandas Capelo ◽  
Eliane Miranda da Silva ◽  
Marilza Campos de Magalhães ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Rio De Janeiro ◽  
Long Term Follow Up ◽  
Common Variable

IgE deficiency may indicate underlying hypogammaglobulinaemia?

2014 ◽  
Vol 67 (9) ◽  
pp. 832-833 ◽  
Author(s):  
Shireen McVicker ◽  
M Yousuf Karim
Keyword(s):  
Early Diagnosis ◽  
Common Variable Immunodeficiency ◽  
Immunoglobulin E ◽  
Lung Damage ◽  
Serum Immunoglobulin ◽  
Serum Ige ◽  
New Diagnosis ◽  
Immunodeficiency States ◽  
Common Variable ◽  
Low Serum

Low serum immunoglobulin E (IgE) (<2 kU/L) found during allergy investigations may be a marker for other immunodeficiency states, in particular common variable immunodeficiency. The latter is characterised by recurrent infections, mainly respiratory, resulting in structural lung damage making early diagnosis important. We looked through 4013 samples retrospectively over a 12-month period to identify samples with IgE <2 kU/L. We identified 74/4013 (1.84%) with serum IgE levels <2 kU/L. Only 20 samples had serum immunoglobulin quantification and serum electrophoresis requested. Three of these samples were from the same patient, 10/18 (56%) had one or more classes of immunoglobulin above/below reference range for age and two of these had new diagnosis of immunodeficiency. Serum IgE <2 kU/L can be a marker for hypogammaglobulinaemia or common variable immunodeficiency. As early diagnosis is important to reduce morbidity and mortality, very low serum IgE should trigger further investigation—that is, serum protein electrophoresis and immunoglobulin quantification.

Common variable immunodeficiency syndrome: a difficult diagnostic problem

2007 ◽  
Vol 121 (12) ◽  
pp. 1204-1206 ◽  
Author(s):  
C Spyridakou ◽  
S Berry ◽  
J Phillipps
Keyword(s):  
Early Diagnosis ◽  
Respiratory Tract ◽  
Otitis Media ◽  
Common Variable Immunodeficiency ◽  
Chronic Sinusitis ◽  
Otitis Media With Effusion ◽  
Tract Involvement ◽  
Immunodeficiency Syndrome ◽  
Common Variable ◽  
Common Variable Immunodeficiency Syndrome

AbstractObjective:We present a rare case of an adult male with undiagnosed common variable immunodeficiency syndrome who presented to the ENT department with refractory chronic sinusitis and otitis media with effusion.Method:Case report and a review of the international literature concerning common variable immunodeficiency syndrome in adults and upper respiratory tract involvement.Results:A 37-year-old male presented to the ENT department with refractory chronic sinusitis and otitis media with effusion. The diagnosis of common variable immunodeficiency syndrome was delayed, and difficult, primarily due to minimal symptoms with no lower respiratory tract involvement and negative past or family medical history.Conclusion:Early diagnosis helps to prevent morbidity and even mortality. This article highlights how difficult and important it is to make an early diagnosis of common variable immunodeficiency syndrome. Early diagnosis will only be made by increased awareness of this condition.

scholarly journals Inflammatory Duodenal Polyposis Associated with Primary Immunodeficiency Disease: A Novel Case Report

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Irfan Ali Shera ◽  
Sheikh Mudassir Khurshid ◽  
Mohd Shafi Bhat
Keyword(s):  
Primary Immunodeficiency ◽  
Bacterial Infections ◽  
Primary Immunodeficiency Disease ◽  
Lymphoid Hyperplasia ◽  
Rare Entity ◽  
Rare Form ◽  
Duodenal Polyps ◽  
Immunodeficiency Disease ◽  
Common Variable ◽  
Duodenal Polyposis

Agammaglobulinemia is a rare form of B-cell primary immunodeficiency disease characterized by reduced levels of IgG, IgA, or IgM and recurrent bacterial infections. Agammaglobulinemia is most commonly associated with diffuse nodular lymphoid hyperplasia. Duodenal polyps are a rare entity; however, due to wide use of esophagogastroduodenoscopy, incidental diagnosis of duodenal polyps appears to be increasing. Although inflammatory duodenal polyposis has been reported in the literature, its association with common variable immunodeficiency has not been reported till date to the best of our knowledge. We report a case of a 59-year-old male with chronic symptoms of agammaglobulinemia associated with inflammatory duodenal polyposis.

scholarly journals Serum B cell activating factor and interleukin 10 levels in common variable immunodeficiency: Relationship with clinical findings

2019 ◽  
Vol 76 (1) ◽  
pp. 42-49
Author(s):  
Radovan Mijanovic ◽  
Sladjana Andrejevic ◽  
Vladimir Jurisic ◽  
Branka Bonaci-Nikolic
Keyword(s):  
Autoimmune Diseases ◽  
B Cell ◽  
Common Variable Immunodeficiency ◽  
Healthy Subjects ◽  
Serum Levels ◽  
Pulmonary Diseases ◽  
Recurrent Infections ◽  
B Cell Activating Factor ◽  
Chronic Pulmonary Diseases ◽  
Common Variable

Background/Aim. Common variable immunodeficiency (CVID) is an immunologically and clinically heterogeneous disorder. Disturbed cytokine production is implicated in dysfunctional immune response. The aim of this study was to investigated B-cell activating factor (BAFF) and interleukin (IL)- 10 levels in CVID patients. Methods. The study included 28 CVID patients diagnosed and followed during a 20-year period (mean follow-up 14.5 years). Control groups consisted of 4 patients with X-linked agammaglobulinemia (XLA) and 21 healthy subjects. According to clinical characteristics, the CVID patients were divided into four groups which partly overlap: chronic pulmonary diseases (n = 21), splenomegaly (n = 13), autoimmune diseases (n = 9) and patients with recurrent infections despite regular intravenous immunoglobulin (IVIg) substitution (n = 4). The serum levels of BAFF and IL-10 were measured by commercial ELISA. Results. The BAFF levels were found to be higher in all CVID patients compared to the healthy controls (p < 0.01). The most significant differences were observed in the patients with pulmonary diseases and splenomegaly (p < 0.0001). Also, concentrations of IL-10 were higher in all CVID patients in comparison with the XLA patients (p < 0.05) and healthy subjects (p < 0.01). A statistically significant positive correlation (r = 0.86; p < 0.01) was found between the levels of BAFF and IL-10 in the CVID patients with autoimmune diseases. We demonstrated that the CVID patients with chronic pulmonary diseases had higher levels of IL-10, while the CVID patients with recurrent infections had higher BAFF concentrations in comparison to the patients without these features (p < 0.05). Conclusion. In spite of the limited number of patients, this is the first report from Serbia, examining the serum levels of BAFF and IL-10 in the CVID patients. Our study showed significantly increased concentrations of serum BAFF and IL-10 in the patients with CVID compared to the healthy subjects. Further studies are needed to confirm our findings that the BAFF levels are more pronounced in patients with recurrent infections while IL-10 levels are higher in patients with chronic pulmonary diseases.

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