Background Interstitial lung disease (ILD) is the leading cause of systemic sclerosis (SSc) related morbidity and mortality,surpassing scleroderma renal crisis, which has become a rarity following the advent of angiotensin-converting-enzyme (ACE) inhibitors.High resolution computed tomography (HRCT) is the gold standard for diagnosis of in systemic sclerosis (SSc) related interstitial lung disease (SSc-ILD).
Objectives To evaluate multidetectorcomputed tomographic (CT) patterns of lung disease inpatients with systemic sclerosis (SSc) using semi-quantitative estimation of disease extent on CT in correlation to Pulmonary function tests (PFTs).
Methods 40 female patients who met the American College of Rheumatology SSc criteria were recruited and consulted from the outpatient clinic of Rheumatology & Rehabilitation Department. Their age ranged from 17-57.All eligible participants underwent clinical examination, PFTs and scanning using a 16-row multidetector CT scanner (GE Lightspeed 16, General Electric Medical Systems, Milwaukee, Wisconsin, USA)at full inspiration, in supine position. The CT features were quantified visually using Semi-quantitative scoring of total, ground glass and reticular pattern extents by two observersin blinded manner. The disease extent was assessed at five representative levels, proposed by Desai et al: origin of great vessels (i), carina (ii), pulmonary venous confluence (iii), between levels (iii) and (v) and 1cm above the right hemidiaphragm. Forced expiratory volume in 1 sec (FEV1), forced vital capacity (FVC) (% pred. values)and FEV1/FVCwere measured.Analyses were performed using SPSS software. Correlation was made using Pearson correlation and p value 0.05 was considered significant.
Results The coarseness of fibrosis in patients with SSc (average coarseness score7.65 Range, 0.0 –15), The proportion of ground-glass opacification (average proportion, 27.75%; range, 0.0%–75%).Honey-comb pattern was seen in 21 (52.5%) of 40 patients. Extent of disease was. Restrictive deficit found in 30 patients (mild to moderate in 19/40 and severe in 11/40). Significant relation was found between the extentof disease and the percentage predicted FVC (r=0.373, p 0.018) and FEV1/FVC (r=0.593, p 0.000).
Conclusions Scoring of systemic sclerosis (SSc) related interstitial lung disease (SSc-ILD) could be applicable as a routineevaluation of patients with SSc. Good correlation found between extent of HRCT (MDCR)findings and functional pulmonary impairment.
Disclosure of Interest None declared
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