Radiologic progression with minimal functional decline in cystic fibrosis: Insights from a prospective study.

backgroundTimely identification and monitoring of lung disease progression are key components of effective management in patients with cystic fibrosis (CF). Pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans are commonly used to assess disease progression in these patients. Determining which modality provides the most sensitive and reliable assessment is crucial for establishing effective disease monitoring guidelines, particularly in India, where national guidelines are lacking.

aimTo assess the progression of lung disease in patients with CF using a combination of HRCT and PFTs.

methodsA total of 32 patients with CF were prospectively enrolled from the outpatient clinic and followed longitudinally. Clinical and physiological parameters, including age, spirometric indices, and HRCT based Bhalla scores, were evaluated at baseline and after one-year follow-up. Paired comparisons between baseline and follow-up HRCT scores, as well as baseline and follow-up PFT parameters, were performed using a two-tailed paired

resultsOf the 32 patients, 18 were female and 14 were male, with a mean age of 86.33 months (range: 48-192 months). The mean Bhalla score demonstrated a statistically significant increase from 5.50 at baseline to 8.25 at one-year follow-up (

conclusionDespite significant radiological progression of structural lung damage detected by HRCT, pulmonary function parameters remained relatively stable. These findings suggest that PFTs may be less sensitive in detecting progressive structural deterioration in CF, underscoring the value of HRCT as a sensitive tool for disease monitoring and early detection of worsening lung disease.