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. Author manuscript; available in PMC: 2022 Apr 1.
Published in final edited form as: Ann Allergy Asthma Immunol. 2021 Oct;127(4):513. doi: 10.1016/j.anai.2021.07.024

Consideration of pulmonary hypertension in the evaluation of common variable immunodeficiency

Paul J Maglione 1, Jessica D Gereige 1, Theodore K Lee 1
PMCID: PMC8525548  NIHMSID: NIHMS1745980  PMID: 34593104

We recently published a current overview of common variable immunodeficiency (CVID) diagnostic evaluation.1 This work focused upon the most frequently encountered and studied CVID complications, as these are the likeliest to be clinically useful and best understood. There are numerous CVID-associated complications that we did not cover due to article length limitations, relatively rare occurrence, and a paucity of scientific study, including neurologic manifestations, renal disease, endocrine disorders, among others.2–4 Thus, while doing our best to provide a current summary on CVID diagnostic evaluation in a concise manner, we simply could not cover all the intricacies of this highly complex immune disorder.

Several months after the preparation and publication of our review, a study describing pulmonary hypertension as an uncommon and poorly understood complication of CVID was published.5 We thank the authors of this work for highlighting a medical complication with potentially significant impact upon CVID course and clinical outcome. While this study does not definitively identify the cause of pulmonary hypertension in the 10 subjects studied, there are numerous conceivable etiologies in CVID. Pulmonary hypertension can result from chronic lung disease, including bronchiectasis, chronic obstructive pulmonary disease, and interstitial lung disease – all pulmonary complications that frequently affect CVID patients.6 Alternatively, hepatosplenomegaly with portal hypertension or chronic systemic inflammation, additional complications that may emerge in CVID, may underlie pulmonary hypertension.7, 8 Additional causes of pulmonary hypertension may be present as well. Moreover, the underlying etiology of this complication may differ among individual CVID patients. Further research efforts to understand pulmonary hypertension pathogenesis as well as the best approaches for diagnosis and treatment of this complication in CVID are needed. Until we better comprehend how to incorporate evaluation of pulmonary hypertension into routine CVID evaluation, clinicians must remain mindful of this rare, but dangerous, complication.

Funding Source:

PJM received support from National Institutes of Health grants AI137183 and AI151486, a faculty development grant from the American Association of Allergy, Asthma and Immunology Foundation, and a Boston University Career Investment Award.

Abbreviations/Acronyms:

CVID

common variable immunodeficiency

Footnotes

Conflicts of Interest: The authors declare no conflicts of interest.

References

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