Common variable immunodeficiency syndrome with right aortic arch: a case report.

Area of Science:

• Immunology
• Cardiovascular Science
• Rare Diseases

Background:

• Common variable immunodeficiency syndrome (CVID) is a primary immunodeficiency primarily affecting adults, characterized by hypogammaglobulinemia.
• It leads to increased susceptibility to recurrent infections across various sites.
• The condition necessitates a high index of suspicion in patients with persistent infections.

Observation:

• A 41-year-old female presented with a long-standing history of recurrent sinopulmonary infections, otitis media, diarrhea, and cystitis.
• Diagnostic workup confirmed Common variable immunodeficiency syndrome.
• Radiological imaging revealed an incidental finding of a right aortic arch and aberrant left subclavian artery.

Findings:

• This case presents the first reported instance of Common variable immunodeficiency syndrome co-occurring with a right aortic arch.
• The patient's symptoms were consistent with CVID, managed through immunoglobulin replacement therapy.
• The anatomical vascular anomaly did not appear to directly influence the presentation or management of CVID.

Implications:

• Highlights the importance of considering CVID in adults with recurrent infections, even with unusual co-occurring conditions.
• Suggests that rare vascular anomalies like right aortic arch may be incidentally found in patients with primary immunodeficiencies.
• Underscores the need for comprehensive evaluation in patients with recurrent infections to identify underlying causes and associated conditions.