R.F. KHAKIMOVA¹, A.V. LUNTSOV², G.R. KAMASHEVA¹, N.SH. KURMAEVA^1, 2, K.I. SAMIGULLINA¹

 ¹Kazan State Medical University, Kazan

²Republic Clinical Hospital, Kazan

Contact details:

Khakimova R.F. — MD, Professor of the Department of Clinical Immunology and Allergology

Address: 49 Butlerova St., Kazan 420012, Russian Federation, tel.: +7 (843) 236-06-52, e-mail: Khakimova@yandex.ru

 Selective IgA deficiency (sIgAD) is one of the most common primary immunodeficiencies (PID) with a predominant impairment of antibody production.

The purpose — to analyze the clinical features of sIgAD in adults.

Material and methods. We observed 21 patients with sIgAD in Republic Center for Clinical Immunology of Republic Clinical Hospital. The diagnosis was made according to the latest clinical guidelines. The paper presents two clinical cases to demonstrate the diversity of sIgAD clinical manifestations.

Results. SIgAD was diagnosed at the age of 18 and older in 80.9% of patients, in other cases diagnosis was verified in childhood. Recurrent respiratory and gastrointestinal tract infections were common reasons for physician visits. Infectious syndrome was associated with various non-infectious manifestations: allergic diseases (5; 23.8%), autoimmune disorders (4; 19%), and hematological disorders (2; 9.5%). Diagnostic delays were shown in all cases: the time interval between the symptoms onset and diagnosis was over 10 years in 57.1% of patients, in 28.6% — from 5 to 10 years, in 14.3% — less than 5 years.

Conclusions. Infectious syndrome is the leading clinical manifestation of SIgAD in adults, which is often combined with allergic and autoimmune diseases. Given the diversity of clinical manifestations, the SIgAD awareness is essential for allergist-immunologists as well as other healthcare professionals (physicians, pulmonologists, gastroenterologists, hepatologists, otorhinolaryngologists, and endocrinologists).

Key words: рrimary immunodeficiency, selective IgA deficiency, adults.

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