Common variable immunodeficiency: different faces
of the same disease

Elżbieta Grześk; Anna Dąbrowska; Anna Urbańczyk; Marlena Ewertowska; Mariusz Wysocki; Sylwia Kołtan

doi:10.5114/ada.2021.110067

eISSN: 2299-0046
ISSN: 1642-395X

Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii

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abstract:

Original paper

Common variable immunodeficiency: different faces of the same disease

Elżbieta Grześk

¹

,

Anna Dąbrowska

¹

,

Anna Urbańczyk

¹

,

Marlena Ewertowska

¹

,

Mariusz Wysocki

¹

,

Sylwia Kołtan

¹

Department of Paediatrics, Haematology and Oncology, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Torun, Poland

Adv Dermatol Allergol 2021; XXXVIII (5): 873-880

DOI: https://doi.org/10.5114/ada.2021.110067

Online publish date: 2021/11/05

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Introduction
Common variable immunodeficiency (CVID) is one of the primary humoral immunodeficiencies. Despite the inborn nature, the first symptoms may appear in both children and adults. It is characterized by hypogammaglobulinaemia, severe infections, autoimmunity, allergies, and a predisposition to cancer. A delay in diagnosis is a significant problem: the time from the first symptoms of the disease to diagnosis and the implementation of proper treatment is usually very long. The consequence can be irreversible complications, which is why it is so important to promote knowledge on this immunodeficiency.

Aim: To present the clinical and laboratory manifestation of primary immunodeficiencies such as common variable immunodeficiency.

Material and methods
The study presents the clinical and laboratory phenotype of 14 patients diagnosed with CVID, aged 5 to 58 years. A detailed medical history was taken, and clinical symptoms, immunological test results and complications were analysed in each patient. According to the ESID guidelines, in the differential diagnosis process of CVID the secondary hypogammaglobulinaemia was excluded.

Results
The follow-up period ranged from 39 to 133 months (median: 79 months). The median delay for the entire group was 5 years, which was shorter in children than in adults. In the presented group, the infectious phenotype (pneumonia, sinusitis) was dominant. Autoimmune and allergic diseases, malignant tumours and enteropathies have also been observed.

Conclusions
The diagnostic delay is still too long, especially in adults, which can lead to serious and irreversible complications. Early diagnosis and appropriate treatment with intravenous and subcutaneous immunoglobulins reduces the frequency of infections and their potential complications.

keywords:

Common variable immunodeficiency: different faces of the same disease

Elżbieta Grześk 1 , Anna Dąbrowska 1 , Anna Urbańczyk 1 , Marlena Ewertowska 1 , Mariusz Wysocki 1 , Sylwia Kołtan 1

immunodeficiency, clinical course, diagnosis, treatment, immunoglobulins

Elżbieta Grześk

¹

,

Anna Dąbrowska

¹

,

Anna Urbańczyk

¹

,

Marlena Ewertowska

¹

,

Mariusz Wysocki

¹

,

Sylwia Kołtan

¹