Evidence- and Consensus-Based Recommendations for the Screening, Diagnosis, and Management of Secondary Hypogammaglobulinemia in Patients With Systemic Autoimmune Rheumatic Diseases by the Taiwan College of Rheumatology Experts

Abstract

Secondary hypogammaglobulinemia (SHG) is characterized by reduced serum immunoglobulin (Ig) levels and is typically caused by immunosuppressive therapy or certain diseases. Patients with systemic autoimmune rheumatic diseases (SARDs) and hematological malignancies are particularly susceptible to developing SHG due to underlying diseases and the use of immunosuppressive medications, such as B-cell-targeted agents. Consequently, SHG significantly contributes to increased risks of severe infections and mortality in SARDs patients. Considering the lack of a unified strategy for managing SHG, the Taiwan College of Rheumatology (TCR) aimed to formulate consensus recommendations for the screening, diagnosis, and management of SHG. These recommendations were developed based on emerging evidence during a face-to-face meeting of the TCR committee (nine immunologists and rheumatologists), and utilizing the modified Delphi process. This meeting involved a comprehensive review of the current evidence, using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) methodology. Thirteen consensus recommendations were developed to emphasize the importance of early detection and optimal treatment of SHG. Furthermore, effective prevention of infections through risk assessment alongside timely and regular monitoring of IgG levels was highlighted. The recommendations also included anti-infective therapies and intravenous Ig replacement, offering valuable guidance to rheumatologists in managing SHG. This consensus will be regularly updated as newer evidence emerges.

Keywords: B‐cell‐targeted therapy; consensus recommendations; immunoglobulin replacement therapy; immunosuppression; infection; secondary hypogammaglobulinemia; systemic autoimmune rheumatic diseases.

FIGURE 1

TCR consensus statement formulation process. *Composed of nine rheumatologists and immunologists. GRADE, grading of recommendations, assessment, development and evaluation; TCR, Taiwan College of Rheumatology.

FIGURE 2

The screening, monitoring, management, and follow‐up algorithm of SHG in SARD patients. ABW, adjusted body weight; BCTT, B‐cell‐targeted therapy; IBW, ideal body weight; Ig, immunoglobulin; IgRT, immunoglobulin replacement therapy; IVIg, intravenous immunoglobulin; LLN, lower limit of normal; R, recommendation; SARDs, systemic autoimmune rheumatic diseases; SHG, secondary hypogammaglobulinemia.