Pulmonary Hypertension in Patients with Common Variable Immunodeficiency

Area of Science:

• Immunology
• Pulmonology
• Cardiology

Background:

• Common variable immunodeficiency (CVID) is associated with infectious, inflammatory, and autoimmune conditions.
• Pulmonary hypertension (PH) is a rare but serious complication of CVID with poorly understood characteristics.

Purpose of the Study:

• To characterize the clinical, functional, hemodynamic, radiologic, and histologic features of CVID-associated PH.
• To investigate the outcomes of patients with CVID-associated PH.

Main Methods:

• Retrospective analysis of 10 CVID-associated PH patients from the French PH Network.
• Evaluation included clinical data, pulmonary function tests, imaging (HRCT), and lymph node pathology.

Main Results:

• CVID-associated PH predominantly affected women (9:1 ratio), presenting at a median age of 36.5 years with a 12-year delay from CVID diagnosis.
• Patients exhibited severe hemodynamics, frequent portal hypertension (6/10), and decreased diffusing capacity for carbon monoxide.
• Radiology showed mediastinal lymphadenopathy and interstitial lung changes; lymph node pathology revealed granulomas and lymphoid hyperplasia.

Conclusions:

• PH is a potential complication of CVID, likely driven by its inflammatory nature.
• CVID-associated PH is precapillary and multifactorial, involving portal hypertension, vascular remodeling, lung parenchymal disease, and extrinsic compression.
• These findings support classifying CVID-associated PH within group 5 of the current PH classification system.