OP0145 Scleroderma related interstitial lung disease and mycophenolate : long term outcomes

• Published in: Annals of the rheumatic diseases Vol. 77; no. Suppl 2; p. 123
• Main Authors: Janardana, R., Irodi, A., Chebbi, P.P., RV, L., danda, D., Mahasampath, G.
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group LTD 01.06.2018
• Subjects: Computed tomography; Cyclophosphamide; Fibrosis; Long term; Lung diseases; Mycophenolate mofetil; Mycophenolic acid; Scleroderma; Thorax
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/annrheumdis-2018-eular.6804

BackgroundInterstitial lung disease [ILD] is a leading cause of mortality in scleroderma.1 Scleroderma lung study-II clearly illustrates the equivalent efficacy and a better side-effect profile of mycophenolate mofetil [MMF] as compared to cyclophosphamide.ObjectivesTo study the long term outcomes of mycophenolate mofetil in scleroderma related interstitial lung disease (SSc-ILD) in terms of change in forced vital capacity (FVC)To determine the effect of MMF on longitudinal high resolution computed tomography (HRCT) scores.MethodsAll patients of SSc-ILD from 2013 till date who had a baseline FVC and follow up FVC were taken for analysis. All patient received an average dose of 2 g/day of MMF for a median duration of 2 years and were tapered as per the standard protocol. FVC was measured using standard protocols. The FVC change was computed as percentage relative change from baseline FVC value. According to American Thoracic society recommendations, improvement is defined as an increase in FVC ≥10%, stabilisation by change in FVC <10% and worsening by a reduction in FVC ≥10%.ResultsWe had 88 patients with a baseline and followup FVC data. Of these 66 patients had a 1 year follow up; 46 patients had a 2 year followup and 29 patients had a 3 year followup data. The absolute median (IQR) increase in the FVC value at the end of 1 year, 2 years, 3 years were 4.15 (-2.3 to 10.5), 2.85 (-3.4 to 7.2) and 3.8 (-0.6 to 10.4) respectively. At the end of 1 year, 2 years and 3 years 89.4%; 82.6% and 75.9% respectively had a stable or improved relative FVC change from baseline.Of the 52 individuals who had a baseline as well as repeat HRCT, stable/improved scores in ground glass opacity, fibrosis and honey combing was seen in 80.8%, 86.5% and 86.5% respectively. There was no difference in the extent of FVC change between those with limited vs extensive disease.Abstract OP0145 – Table 1Baseline CharacteristicsValue Age, yr. mean(SD)33.8 (±11.3) Female sex, n (%)75 (85.2%)Type of SScLimitedDiffuseSine scleroderma5716Disease duration, months, mean (SD)46.6 (±42.1)AntibodiesScl-70Anti centromere 701MRSS, mean (SD) n=5220.4(±13.2)FVC% predicted, mean (SD)61.2 (±17.9)HRCT pattern [n=85]NSIP (cellular)NSIP (fibrotic)UIP68116HRCT determined disease extent [median(IQR)] ; n=52Maximum fibrosis score (0–4)Maximum ground glass opacity (0–4)Maximum honeycombing (0–4) 0.33 (0–1.3)1 (0–1.5)0 (0.08)Maximum fibrosis score n=521%–25%26%–100%29 (55.8%)23 (44.2%)HRCT change over 2 yearsFrequency (Percentage)Ground Glass opacityStable/ImprovedWorsened42 (80.8%)10 (19.2%)FibrosisStable/ImprovedWorsened45 (86.5%)7 (13.5%)Honey combingStable/ImprovedWorsened45 (86.5%)7 (13.5%)Abstract OP0145 – Figure 1 Relative change in FVC from base line at different time pointsConclusionsA vast majority of individuals of scleroderma ILD patients on MMF in our cohort had a stable disease or improvement over short and long term followup both in terms of FVC change from baseline as well as HRCT scoring.Reference[1] Kazerooni EA, Martinez FJ, Flint A, et al. Thin-section CT obtained at 10-mm increments versus limited three-level thin- section CT for idiopathic pulmonary fibrosis: Correlation with pathologic scoring AJR. Am J Roentgenol1997Oct;169(4):977–83.Disclosure of InterestNone declared