Evidence of pulmonary arterial hypertension in two patients with common variable immunodeficiency

Common variable immunodeficiency is a chronic illness plagued with recurrent infections and the potential to develop autoimmune disease. These patients may manifest a spectrum of complications ranging from hematologic malignancy to chronic parenchymal lung disease. Regular immunoglobulin replacement therapy improves immunologic debility but does not mitigate other features of this disease. Here, we discuss a complication of common variable immunodeficiency not previously characterized in the literature. We present two cases of advanced pulmonary vascular disease associated with common variable immunodeficiency treated with pulmonary vasodilators.

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Topics

Immunology/ Immune Cell Signaling/ Inflammation/ Immunotherapy
Pathology and Pathophysiology
Pulmonary Arterial Hypertension
Right Ventricle: Structure, Function and Dysfunction

Authors

Jessica Huston, Joyce Johnson, Anna Hemnes, Meredith Pugh

Published in:

Pulmonary Circulation Vol 10 : No 2 cover image

April 2020

Pulmonary Circulation Vol 10 : No 2

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