Interstitial Lung Disease in Common Variable Immunodeficiency.

Area of Science:

• Pulmonology
• Immunology
• Pathology

Background:

• Interstitial lung disease (ILD) is a frequent complication in common variable immunodeficiency (CVID).
• ILD diagnosis in CVID often relies on imaging due to limited histopathology availability.
• ILD in CVID can co-occur with bronchiectasis and autoimmune conditions.

Purpose of the Study:

• To analyze the histopathological findings and clinical outcomes of CVID patients with biopsy-proven ILD.
• To characterize the spectrum of ILD subtypes and associated autoimmune manifestations in CVID.
• To assess the mortality and morbidity associated with ILD in a CVID cohort.

Main Methods:

• Retrospective review of 637 CVID patients followed over four decades.
• Analysis of lung biopsy data from 46 CVID patients with confirmed ILD.
• Examination of clinical data including age at diagnosis, IgG levels, B cell counts, autoimmune features, and survival.

Main Results:

• Granulomas (54.4%) and lymphoid interstitial pneumonia (28.3%) were the most common ILD findings on biopsy.
• Autoimmune manifestations were present in 60.9% of patients.
• Mortality was high, with 19.6% of patients deceased, and 6.5% underwent lung transplantation.

Conclusions:

• ILD represents a significant complication in CVID, associated with substantial morbidity and mortality.
• The diverse histopathological findings underscore the complexity of ILD in CVID.
• Further research into ILD pathogenesis and progression in CVID is essential for optimizing treatment strategies.