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


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.


Common variable hypogammaglobulinemia, respiratory failure, pulmonary hypertension

Full Text



  • Gathmann B, Mahlaoui N; CEREDIH; Gérard L, et al. European Society for Immunodeficiencies Registry Working Party. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol 2014; 134: 116-26; https://doi.org/10.1016/j.jaci.2013.12.1077
  • Sullivan KE, Puck JM, Notarangelo LD, et al. USIDNET: a strategy to build a community of clinical immunologists. J Clin Immunol 2014; 34: 428-35; https://doi.org/10.1016/10.1007/s10875-014-0028-1
  • Quinti I, Soresina A, Spadaro G, et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol 2007; 27: 308-16; https://doi.org/10.1007/s10875-007-9075-1
  • Thoré P, Jaïs X, Savale L, et al. Pulmonary Hypertension in Patients with Common Variable Immunodeficiency. J Clin Immunol 2021; 41: 1549-62; https://doi.org/10.1007/s10875-021-01064-w
  • Thoré P, Humbert M, Montani D. Pulmonary hypertension: A rare but severe complication of common variable immunodeficiency. Ann Allergy Asthma Immunol 2021; 127: 512-3; https://doi.org/10.1016/j.anai.2021.06.016
  • Daniil Z, Karetsi E, Zakynthinos E, et al. Pulmonary arterial hypertension in a patient with common variable immunodeficiency and unilateral bronchiectasis: Successful treatment with iloprost. Eur J Intern Med 2007; 18: 333-5; https://doi.org/10.1016/j.ejim.2006.11.014
  • Arslan S, Ucar R, Yavsan DM, et al. Common variable immunodeficiency and pulmonary amyloidosis: a case report. J Clin Immunol 2015; 35: 344-7; https://doi.org/10.1007/s10875-015-0151-7
  • Seidel MG, Kindle G, Gathmann B, et al. The European Society for Immunodeficiencies (ESID) Registry Working Definitions for the Clinical Diagnosis of Inborn Errors of Immunity. J Allergy Clin Immunol Pract 2019; 7: 1763; https://doi.org/10.1016/j.jaip.2019.02.004
  • Bonilla FA, Barlan I, Chapel H, et al. International Consensus Document (ICON): Common Variable Immunodeficiency Disorders. J Allergy Clin Immunol Pract 2016; 4: 38; https://doi.org/10.1016/j.jaip.2015.07.025
  • Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 2019; 53: 1801913; https://doi.org/10.1183/13993003.01913-2018
  • D’Andrea A, Stanziola A, Di Palma E, et al. Right Ventricular Structure and Function in Idiopathic Pulmonary Fibrosis with or without Pulmonary Hypertension. Echocardiography 2016; 33: 57; https://doi.org/10.1111/echo.12992
  • Nowak J, Hudzik B, Jastrzȩbski D, et al. Pulmonary hypertension in advanced lung diseases: Echocardiography as an important part of patient evaluation for lung transplantation. Clin Respir J 2018; 12: 930; https://doi.org/10.1111/crj.12608
  • Stone AC, Machan JT, Mazer J, et al. Echocardiographic evidence of pulmonary hypertension is associated with increased 1-year mortality in patients admitted with chronic obstructive pulmonary disease. Lung 2011; 189: 207; https://doi.org/10.1007/s00408-011-9293-4
  • Seeger W, Adir Y, Barberà JA, et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol 2013; 62: D109; https://doi.org/10.1016/j.jacc.2013.10.036
  • Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015; 46: 903-75; https://doi.org/10.1183/13993003.01032-2015
  • Hamada K, Nagai S, Tanaka S, et al. Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis. Chest 2007; 131: 650; https://doi.org/10.1378/chest.06-1466
  • Budev MM, Arroliga AC, Wiedemann HP, et al. Cor pulmonale: an overview. Semin Respir Crit Care Med 2003; 24: 233; https://doi.org/10.1055/s-2003-41105
  • Kimura M, Taniguchi H, Kondoh Y, et al. Pulmonary hypertension as a prognostic indicator at the initial evaluation in idiopathic pulmonary fibrosis. Respiration 2013; 85: 456; https://doi.org/10.1159/000345221
  • Rich S. Primary pulmonary hypertension: executive summary. Evian, France. World Health Organization, 1998
  • Hurdman J, Condliffe R, Elliot CA, et al. Pulmonary hypertension in COPD: results from the ASPIRE registry. Eur Respir J 2013; 41: 1292; https://doi.org/10.1183/09031936.00079512
  • Oswald-Mammosser M, Weitzenblum E, Quoix E, et al. Prognostic factors in COPD patients receiving long-term oxygen therapy. Importance of pulmonary artery pressure. Chest 1995; 107: 1193; https://doi.org/10.1378/chest.107.5.1193
  • Weitzenblum E, Schrijen F, Mohan-Kumar T, et al. Variability of the pulmonary vascular response to acute hypoxia in chronic bronchitis. Chest 1988; 94: 772; https://doi.org/10.1378/chest.94.4.772
  • Snijders D, Fernandez Dominguez B, Calgaro S, et al. Mucociliary clearance techniques for treating non-cystic fibrosis bronchiectasis: Is there evidence? Int J Immunopathol Pharmacol 2015; 28: 150-9; https://doi.org/10.1177/0394632015584724


Abstract: 112 views
HTML: 16 views
PDF: 26 views


  • There are currently no refbacks.