Pulmonary Complications in a Patient with Common Variable Hypogammaglobulinemia

Paolo Ghiringhelli, Fabrizio Foieni, Girolamo Sala, Alessandro Diana, Beatrice Valvo, Mariella Ciola, Michele Zaza, Roberto Bertolini, Cinzia Simoni, Lorenzo Bellintani, Andrea Agostinelli, Federica Macchi, Sara Limbiati, Michela Provisione

DOI: https://doi.org/10.7175/cmi.v17i1.1521

Abstract

Common variable immunodeficiency (CVID) is a rare disorder characterized by primary antibody deficiency leading to hypogammaglobulinemia and increased risk of infections. Pulmonary hypertension (PH) is an unusual complication of CVID and may be associated with chronic hypoxemic respiratory failure.

We described the case of a 47-year-old female patient hospitalized with worsening dyspnea, which had emerged about 10 days before and was associated with productive cough. 26 years before, she was diagnosed with common variable hypogammaglobulinemia, that was treated with intravenous immunoglobulin infusions for at least 20 years. She had suffered from recurrent secondary infections of the respiratory tract with inveterate bronchiectasis, pulmonary hypertension, and chronic respiratory failure.

This case suggests that Internal Medicine wards, due to their global vision of the patient, are well suited to manage subjects with serious, complex, and genetically determined pathologies.

Keywords

Common variable hypogammaglobulinemia, respiratory failure, pulmonary hypertension

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