scholarly journals A Young Girl with Repeated Episodes of Pneumonia and Intermittednt Diarrhoea - The Primary Immunodeficiency Syndrome

2013 ◽  
Vol 22 (1) ◽  
pp. 24-26
Author(s):  
Abdullah Al-Mamun ◽  
Samim Hasan ◽  
Syed Ahmed Abdullah ◽  
Ahmed Riyad Hossain ◽  
Safiul Alam ◽  
...  
Keyword(s):  
Replacement Therapy ◽  
Primary Immunodeficiency ◽  
Common Variable Immunodeficiency ◽  
Young Girl ◽  
Organ Specific ◽  
Relative Rarity ◽  
Immunoglobulin Replacement ◽  
Immunodeficiency Syndrome ◽  
Work Up ◽  
Common Variable

A description is given of a case of a young girl who presented with repeated episodes of pneumonia since childhood. In addition, she had episodes of intermittent diarrhoea. Her investigative work-up revealed a deficiency of serum immunoglobulins. The profile was suggestive of common variable immunodeficiency. Because of a relative rarity of this disease, it is often misdiagnosed earlier. Late diagnosis and delayed institution of immunoglobulin replacement therapy results in increased morbidity with a wide variety of organ-specific complications and increased mortality. DOI: http://dx.doi.org/10.3329/bjmed.v22i1.13597 Bangladesh J Medicine 2011; 22: 24-26

scholarly journals The prevalence, clinical relevance and origin of autoantibodies in patients with Common variable immunodeficiency on regular immunoglobulin replacement therapy – the results from a prospective observational study

2021 ◽  
Author(s):  
Tomas Milota ◽  
Kotaska Karel ◽  
Lastuvka Petr ◽  
Smetanova Jitka ◽  
Bloomfield Marketa ◽  
...  
Keyword(s):  
Observational Study ◽  
Replacement Therapy ◽  
Common Variable Immunodeficiency ◽  
Clinical Relevance ◽  
Prospective Observational Study ◽  
Immunoglobulin Replacement Therapy ◽  
High Prevalence ◽  
Immunoglobulin Replacement ◽  
Autoimmune Phenomena ◽  
Common Variable

Abstract Background: Common variable immunodeficiency (CVID) is an inborn error of immunity characterized by disturbed immunoglobulin production. Despite of the terrain with severe antibody deficiency, autoantibody-mediated autoimmune phenomena belong to the most frequent autoimmune manifestation. However, many unresolved issues such as prevalence, clinical relevance and origin of autoantibodies detected in CVID patients receiving immunoglobulin replacement therapy (IRT) make the diagnostics of autoimmune complications difficult.Methods: A prospective observational study evaluating the spectrum of 23 different autoantibodies in 38 CVID patients receiving IRT, and in the immunoglobulin solutions used for IRT. Results: The study reveals a high prevalence of anti-GAD (55.3%) and anti-TPO (68.4%) autoantibodes in the cohort of 38 CVID patients on regular IRT. However, the titers of anti-GAD (3.22 vs. 22 kU/L, p≤0.0001) and anti-TPO (109.7 vs. 713 kU/L, p≤0.0001) were significantly lower compared to the newly diagnosed T1D and AIT patients. Moreover, none of the CVID patients with detectable antibodies manifested with T1D and only three patients became suspected of having AIT. A high quantity of anti-GAD (3.24-24.48 kU/L) and anti-TPO (123.6-156.55 kU/L) autoantibodies was found in immunoglobulin solutions for IRT. Conclusions: The study finds a very high prevalence of anti-GAD and anti-TPO autoantibodies in CVID patients receiving regular IRT. Nevertheless, the presence of anti-GAD and anti-TPO is not associated with the manifestation of the respective autoimmune disease. As the high titers of both anti-GAD and anti-TPO were also found in the therapeutics used for IRT, we suggest that the therapeutic immunoglobulins are the source of this false positivity.

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