[Clinic of humoral primary immunodeficiencies in adults. Experience in a tertiary hospital].

BACKGROUND

Primary immunodeficiencies (PID) are characterized by alteration of the components of the immune system. Humoral deficiencies represent 50%. The most common are selective IgA deficiency, Bruton agammaglobulinemia, and common variable immunodeficiency (CVID).

OBJECTIVE

To describe the epidemiological and clinical characteristics of adults with humoral PID, cared for in a Primary Humoral Immunodeficiencies Clinic.

METHODS

A descriptive cross-sectional study that included a year of analysis, including 35 patients with humoral PID, 31 with CVID, and 4 with Bruton agammaglobulinemia. Data were analyzed with descriptive statistics.

RESULTS

Of 35 patients studied, 31 had CVID (88.5%) and 4 (11.5%) Bruton agammaglobulinemia; 21 were men and 14 women. The age at onset of symptoms was 22.7 years, and the delay in diagnosis was 7.2 years. 11.4% of CVID patients died during the study; 4 had malignancies, 22.8% autoimmune diseases, and 48.5% gastrointestinal disorders. Patients with Bruton agammaglobulinemia presented no comorbidities.

CONCLUSIONS

Unlike reports in the literature, in the study group, CVID was the most common cause of humoral PID, predominantly in men; the most common gastrointestinal disorder was intestinal functional disorder.