Assessment of Long-term Temporal Evolution of Chest CT Findings in Granulomatous-lymphocytic Interstitial Lung Disease.

PURPOSE: The purpose of this study is to document the long-term natural history of granulomatous-lymphocytic interstitial lung disease (GLILD), including evolving chest CT features and prevalence of pulmonary fibrosis at follow-up of patients with longstanding disease. MATERIALS AND METHODS: This retrospective longitudinal study compares the CT features of GLILD in a cohort of patients with at least 2 chest CTs performed >24 months apart. A radiologic database identified 22 patients (median age 45.8 years, 13 females and 9 males) with common variable immunodeficiency (CVID) and a clinico-radio-pathologic or clinico-radiologic diagnosis of GLILD. Two chest radiologists jointly reviewed the chronologically first (CT1) and last (CT2) available chest CT examinations for each subject, scoring the severity of different pulmonary nodule types, features of pulmonary fibrosis, ground-glass opacity, and consolidation. Total severity score (TSS) for each category of CT features at CT1 and CT2 was compared using the Wilcoxon signed-rank test. RESULTS: On follow-up averaging 135 months (range 33-235 months) from CT1, CT2 studies demonstrated significantly decreased nodule TSS (P=0.01), significantly increased fibrosis TSS (P=0.0001), and relatively stable ground-glass opacity TSS (P=0.81) and consolidation TSS (P=0.72). Nodule TSS decreased in 73% of patients, in which peribronchial nodules < or ≥10 mm and perilymphatic nodules < or ≥5 mm improved. Fibrosis TSS increased in 82% of patients who developed new or worsening traction bronchiectasis, architectural distortion, and honeycombing. CONCLUSION: CT features of GLILD evolve over time, with new or worsening fibrosis recognized in 82% of patients at long-term follow-up.