Polyautoimmunity Reflecting Immune Dysregulation in Common Variable Immunodeficiency
<b>Background</b>: Common variable immunodeficiency (CVID) is the most frequent symptomatic inborn error of immunity (IEI) in adulthood. Other than recurrent infections, CVID may present with non-infectious complications such as enteropathy, lymphoproliferation, malignancy, and autoimmun...
Saved in:
| Main Authors: | , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2025-02-01
|
| Series: | Biomedicines |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2227-9059/13/3/552 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| Summary: | <b>Background</b>: Common variable immunodeficiency (CVID) is the most frequent symptomatic inborn error of immunity (IEI) in adulthood. Other than recurrent infections, CVID may present with non-infectious complications such as enteropathy, lymphoproliferation, malignancy, and autoimmune diseases. Patients could have a single autoimmune disease (monoautoimmunity) or two or more autoimmune diseases (overt polyautoimmunity). “Latent polyautoimmunity” corresponds to the presence of autoantibodies without a clinically evident autoimmune disease. <b>Methods</b>: The aim of this retrospective study was to describe autoimmunity and polyautoimmunity in a population of 81 CVID adult patients, enrolled from January 2008 to July 2022 (mean follow-up: 8.5 years). <b>Results</b>: We documented at least one autoimmune disorder in 40 patients (49.4%). Moreover, 15 subjects (37.5% of patients with autoimmunity and 18.5% of all CVID population) presented polyautoimmunity. Despite the humoral immune deficiency, we detected different autoantibodies in CVID patients with or without a concomitant autoimmune disease. In both groups with monoautoimmunity and polyautoimmunity, cytopenias were the most common manifestation. Conversely, enteropathy was recorded only in patients with polyautoimmunity (27%, <i>p</i> = 0.006). Patients with polyautoimmunity showed a significantly lower mean age at diagnosis (−12 years, <i>p</i> = 0.018) compared to those with monoautoimmunity. We documented a higher frequency of autoimmunity in CVID patients who had increased diagnostic (+5.6 years) and therapeutic (+7.2 years) delay (<i>p</i> = 0.093 and 0.054, respectively). <b>Conclusions</b>: Polyautoimmunity is a frequent condition in patients affected by CVID. An early start of Ig replacement therapy could help prevent autoimmune complications. |
|---|---|
| ISSN: | 2227-9059 |