Abstract
Objective: There are few reports of adult common variable immunodeficiency (CVID) in Taiwan, and the autoimmune manifestations in such patients were investigated.Methods: Patients with CVID and aged over 18 were included based on a review of their medical records at National Cheng Kung University Hospital from 2001 to 2011. The demographic characteristics, clinical presentations, laboratory data, radiological images and microbiological examinations of the patients were analyzed.Results: Six patients, five females and one male, were identified. Average ages at disease onset and diagnosis were 22.3 ± 19.0 and 27.1 ± 17.7 years, respectively, with an average delay in diagnosis of 4.8 ± 2.9 years. There were 4 patients with autoimmune manifestations including arthritis (juvenile idiopathic arthritis and rheumatoid arthritis) in two, biopsy-proven colitis in two and thrombocytopenia in one. Administration of intravenous immunoglobulin (IVIg) significantly ameliorated autoimmune-related arthritis in these patients. Infection episodes with encapsulated bacteria were documented in all cases, and half of these patients had bronchietasis. Mortality occurred in two cases, with one due to infection during clinical follow-up.Conclusion: Autoimmune manifestations are frequently observed in adult CVID patients from southern Taiwan, and intensive IVIg treatment can improve the autoimmune-related arthritis in such patients.
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