Common variable immunodeficiency from the perspective of rheumatology

Yıl 2022, Cilt: 5 Sayı: 2, 534 - 538, 15.03.2022

Tuba Yuce Inel , Gercek Can

https://doi.org/10.32322/jhsm.1053500

Öz

Aim: Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by impaired B cell differentiation and immunoglobulin production. In addition to increased susceptibility to infection, patients with CVID have an increased tendency to autoimmune disease. Immune dysregulation in these patients may lead to granulomatous disease, malignancy, allergy and autoimmune manifestations. In this study, it was aimed to increase the awareness of rheumatologists about the main signs and symptoms of CVID.
Material and Method: Adult patients followed in the rheumatology department between January 2015 and September 2021 were included in the study. Demographic and clinical characteristics (infections, pulmonary and extrapulmonary granulomatous involvement, autoimmune manifestations), laboratory and imaging findings and treatments of the patients were analyzed.
Results: Ten adult patients with CVID were included in the study. At least one autoimmune manifestation was observed in 80% of the patients. In the follow-up period, 40% of the patients developed arthritis. Involvement of lower extremity joints such as knee and ankle was more prominent. While all patients were given 0.8 g/kg/3 weeks of intravenous immunoglobulin, 80% required immunosuppressive therapy for autoimmune manifestations.
Conclusion: Autoimmune diseases can be seen in patients with CVID, and sometimes this may be the first presentation of CVID. Heterogeneous clinical findings of the disease may lead to delay in diagnosis. Clinicians should be more careful about the different manifestations of CVID to avoid delay in diagnosis.

Anahtar Kelimeler

Common variable immunodeficiency, arthritis, autoimmunity

Kaynakça

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