Background The substantial variability in the evolution of scleroderma-related interstitial lung disease (SSc-ILD) is well known. Patients with early active SSc are at great risk for progressive ILD. Less is known about progression of ILD disease over time in the second 5 years from disease onset than the first years.
Objectives The aim of this study was to investigate high resolution computer tomography (HRCT) findings over time and to compare SSc pts with long standing progressive ILD and with those without worsening in radiological abnormalities
Methods Of the 150 patients included in prospective lung study program in our clinic in 2006-2008, 77 pts (72 female, 5 male) who had both baseline and ∼5 years HRCT scans were analyzed. The comparison of baseline and follow up HRCT scans for each pts was carried out by one expert (L.VN) who expressed over time changes as worse in 22 pts, better in 16 pts, or no change in 39 pts. Scan changes were criteria for categorization of pts into two groups: those who were estimated as “worse” (group A - 22 pts) and others who were “not worse” (group B - 55 pts, 71%). Pulmonary function tests and extra pulmonary clinical variables were estimated at the time of HRCT evaluation. All pts received corticosteroids; cyclophosphamide used 55% (group A) and 27% (group B)of pts.
Results Baseline characteristics were not significantly different between groups A and B (age 41±13yrs vs 37±13; duration of the disease at entry 8 yrs ±6,6 vs 7±6,5; prevalence of diffuse SSc–36% vs 29%; follow up 58,3 mo ±11 vs 59±11,7; predicted forced vital capacity % (p FVC%) 86±18 vs 89,7±17, with exception of the high frequency of anti-topomerase-1 antibody in group A (77% vs 42%, p<0,05). The overall severity of the disease (assessed using the revised Medsger scale) was similar at baseline in both groups (6,7±2,7 vs 6,4±2,6, p>0,05) and increased in group A at the end of the follow up (8,1±2,3 vs 6,5±2,15, p<0,05) suggesting that worsening in CT abnormality was also accompanied by an overall deterioration of the disease. Pts of group A displayed a decline in pFVC% in contrast with increasing in pFVC% in group B (82±2 vs 95, 8±21, p<0, 05). More severe decline of diffusion capacity of the lung for carbon monoxide (pDLCO%) was observed in group A than in group B at baseline (53,4±17 vs 63,9±16,6, p<0,05) and at the follow up (46,9±15vs 59,3±12,7, p<0,05). Systolic pressure in pulmonary artery (mm.Hg) was in normal range in group B but displayed significantly increase in group A (28.2±4.6 vs 36.2±10.5, p<0,05). The ratio of FVC/DLCO increased in both group: from 1,68±0,47 to 1,85±0,55 (p<0,05) in group A and from 1,5±0,53 to 1,7±0,5 in group B (p<0,05).
Conclusions The most pts in this study exhibited stabilization or improvement in HRCT features of ILD and FVC in normal range during long term follow up. Substantial proportion of pts (29%) with high frequency of topomerase-1 antibody displayed progression of lung fibrosis and there was clear concordance of worsening in CT-findings with other signs of disease progression over time. Although all changes were of moderate magnitude they results in a severe impairment in gas transfer at the end of follow up. Our observations raise the possibility that the patients with mild slow progression of SSc-ILD may provide distinct disease phenotype.
Disclosure of Interest None declared