Background/Purpose: Common variable immunodeficiency (CVID) is one of the most common symptomatic primary immunodeficiency syndrome with an incidence of ~1 in 25,000 people. CVID is a heterogenous collection of syndromes, which are all characterized by impaired B-cell differentiation, resulting in defective immunoglobulin production and an increased susceptibility to infection. Autoimmune conditions are common in CVID, and the prevalence of autoimmune-associated inflammatory arthritis in patients with CVID is estimated to be around 10%. This study aims to further delineate the features of autoimmune inflammatory arthritis in patients with CVID.

Methods: Using ICD-9 and ICD-10 diagnostic codes, we identified patients with CVID at the University of Virginia from 2007 to 2018. We then reviewed individual charts to identify the patients who met the Revised European Society for Immunodeficiency (ESID) criteria for CVID. From this cohort of patients, we identified those who met the criteria for any one among various autoimmune inflammatory arthropathies. We reviewed the charts of the patients who met all these criteria for details on the treatment and outcomes of both autoimmune inflammatory arthritis and CVID.

Results: A total of 95 patients met the strict criteria for CVID. Of these, six patients (6.31%) were found to have autoimmune inflammatory arthritis; four had rheumatoid arthritis (RA) and two had psoriatic arthritis (PsA). The male-to-female ratio was 1:1. All the patients were non-Hispanic and Caucasian. All the patients were non-Hispanic and Caucasian. The median time between the diagnoses of autoimmune arthritis (median age = 44.5 years) and CVID (median age = 52.5 years) was 129 months (10.7 years). Four patients (66.6%) were initially diagnosed with autoimmune arthritis and had rheumatoid arthritis. Of these, three patients were on methotrexate and two patients were managed with anti-TNF and abatacept. Five patients (83.3 %) received intravenous immunoglobulin (IVIG). Of all of the patients diagnosed with CVID and autoimmune arthritis, none developed a major infection (requiring a lengthy course of antibiotics or hospitalization) while on treatment. Two patients (33.3%) developed other autoimmune diseases, namely, autoimmune thrombocytopenia (ITP) and autoimmune thyroiditis.

Conclusion: Autoimmune inflammatory arthritis was present in 6.31% of the patients in our CVID cohort. Despite the majority of patients being on immunosuppressants and IVIG, none of them developed a major infection during the study. To our knowledge, this study is the first to address the risk of infection in patients with CVID who received immunosuppressants and IVIG. However, the conclusions were limited by the low sample size. Therefore, there is a need for a prospective longitudinal cohort study. Increased vigilance for autoimmune arthritis complications is important as survival outcomes are worse in CVID patients with non-infectious complications. Further evaluation of these patients to understand the mechanism underlying immune dysregulation is essential, as this may promote targeted therapies and improve clinical outcomes.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/infectious-complications-of-immunosuppressive-therapy-in-patients-with-common-variable-immunodeficiency-cvid-and-inflammatory-arthritis/