Common Variable Immunodeficiency Associated With Connective Tissue Diseases: A Report of Two Cases

Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency in adults and is increasingly recognized as a disorder of immune dysregulation. Autoimmune diseases, including connective tissue diseases, may precede the diagnosis of CVID and contribute significantly to morbidity, particularly through pulmonary involvement. We report two adult women patients with connective tissue diseases (rheumatoid arthritis and primary Sjögren’s syndrome) complicated by recurrent respiratory infections, interstitial lung disease, and severe hypogammaglobulinemia. In both cases, the diagnosis of CVID was established after a prolonged diagnostic delay, and immunoglobulin replacement therapy was indicated. These cases highlight the importance of considering CVID in patients with connective tissue diseases who present with recurrent infections or unexplained pulmonary involvement. Early diagnosis and timely initiation of immunoglobulin replacement therapy are essential for improving outcomes and preventing irreversible complications.

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Publication Details

Journal
Cureus
Published
2026-07-01
DOI
https://doi.org/10.7759/cureus.111910
Primary Topic
Immunodeficiency and Autoimmune Disorders
Type
article
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article

Common Variable Immunodeficiency Associated With Connective Tissue Diseases: A Report of Two Cases

K. Marc, Soukaina SMAINI, LOUDIYI Sara
Cureus
Immunodeficiency and Autoimmune Disorders
article

Common Variable Immunodeficiency Associated With Connective Tissue Diseases: A Report of Two Cases

K. Marc, Soukaina SMAINI, LOUDIYI Sara
article en

Abstract

Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency in adults and is increasingly recognized as a disorder of immune dysregulation. Autoimmune diseases, including connective tissue diseases, may precede the diagnosis of CVID and contribute significantly to morbidity, particularly through pulmonary involvement. We report two adult women patients with connective tissue diseases (rheumatoid arthritis and primary Sjögren’s syndrome) complicated by recurrent respiratory infections, interstitial lung disease, and severe hypogammaglobulinemia. In both cases, the diagnosis of CVID was established after a prolonged diagnostic delay, and immunoglobulin replacement therapy was indicated. These cases highlight the importance of considering CVID in patients with connective tissue diseases who present with recurrent infections or unexplained pulmonary involvement. Early diagnosis and timely initiation of immunoglobulin replacement therapy are essential for improving outcomes and preventing irreversible complications.

Cureus
Openalex Percentile: Top 11%
Immunodeficiency and Autoimmune Disorders
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Common Variable Immunodeficiency Associated With Connective Tissue Diseases: A Report of Two Cases — K. Marc, Soukaina SMAINI, et al. · Cureus (2026) | TGRS Research Map | TGRS