Adult Common Variable Immunodeficiency.

Area of Science:

• Immunology
• Clinical Medicine

Background:

• Common Variable Immunodeficiency (CVID) presents with hypogammaglobulinemia and impaired antibody production, leading to recurrent infections.
• Adult-onset CVID is underdiagnosed, particularly in China, hindering clear understanding of its characteristics.
• Recurrent respiratory infections are a hallmark of CVID, often leading to delayed diagnosis in adults.

Purpose of the Study:

• To characterize the clinical and immunological features of adult-onset CVID in a Chinese cohort.
• To evaluate the efficacy of intravenous immunoglobulin (IVIg) therapy for managing CVID in adult patients.

Main Methods:

• Retrospective analysis of 8 adult CVID patients diagnosed at a single center in China.
• Data collection included demographics, clinical presentations, and immunological markers (immunoglobulin levels, CD4/CD8 ratio).
• Diagnosis adhered to European Society for Immunodeficiency Diseases criteria.

Main Results:

• The average age at diagnosis was 43 years, with a significant diagnostic delay of 10.5 years.
• Patients exhibited low serum levels of IgG, IgA, and IgM, and 7 out of 8 had a reduced CD4/CD8 ratio.
• All patients experienced recurrent respiratory infections, which were substantially reduced by regular IVIg infusions every three weeks.

Conclusions:

• Delayed diagnosis is common in adult CVID, with pulmonary infections being the most frequent initial presentation.
• Intravenous immunoglobulin replacement therapy is effective in controlling recurrent infections and managing CVID in adults.