High occurrence of autoimmune and lymphoproliferative manifestations in adults with common variable immunodeficiency in southernTaiwan

Chrong-Reen Wang; Hung-Wen Tsai; Chi-Chang Shieh

doi:10.1016/j.jfma.2023.10.024

High occurrence of autoimmune and lymphoproliferative manifestations in adults with common variable immunodeficiency in southernTaiwan

J Formos Med Assoc. 2023 Nov 11:S0929-6646(23)00442-4. doi: 10.1016/j.jfma.2023.10.024. Online ahead of print.

Authors

Chrong-Reen Wang¹, Hung-Wen Tsai², Chi-Chang Shieh³

Affiliations

¹ Department of Internal Medicine, National Cheng Kung University Hospital, Tainan, Taiwan. Electronic address: wangcr@mail.ncku.edu.tw.
² Department of Pathology, National Cheng Kung University Hospital, Tainan, Taiwan.
³ Department of Pediatrics, National Cheng Kung University Hospital, Tainan, Taiwan.

PMID: 37957087
DOI: 10.1016/j.jfma.2023.10.024

Abstract

There are no reported case series of common variable immunodeficiency (CVID) from southern Taiwan. A 20-year review was performed in adult CVID from a southern Taiwan medical center. Patients with ages of 18 years or older were enrolled from May, 2003 to April, 2023. Twelve patients were identified, 8 females/4 males aged 23 to 68 (38.9 ± 13.4) with one to 11 years (5.0 ± 3.3) delay of diagnosis after disease onset. There were concomitant autoimmune disorders in 7 (58 %), splenomegaly in 10 (83 %), lymphadenopathy in 4 (25 %) and B-cell lymphoma in 2 (17 %). All received intravenous immunoglobulin (IVIg) infusion with improved autoimmune-mediated arthritis in 2. Patients with higher IgG trough levels (above 500 mg/dL) had a better survival than those with lower IgG trough levels. Adult CVID in southern Taiwan has a high occurrence of autoimmune and lymphoproliferative manifestations. Early diagnosis with IVIg infusion might improve the presentations.

Keywords: Autoimmunity; Common variable immunodeficiency; Intravenous immunoglobulin; Lymphoproliferation; Southern Taiwan.