FRI0283 Are Patients with Extended Interstitial Lung Disease Better Target for The Treatment in SSC?
Authors: H. Takei, H. Yasuoka, K. Yamaoka, T. Takeuchi
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Systemic sclerosis (SSc) is characterized by systemic excessive fibrosis, and interstitial lung disease (ILD) is an important organ involvement strongly associated with morbidity and mortality<sup>1)</sup>. Recent report showed that extent of ILD could predict prognosis of patients with SSc-ILD, and 70% as %FVC or 20% involvement determined by HRCT were reported as thresholds for the differentiation of patients into limited (LD) or extended (ED) ILD<sup>2)</sup>. On that note, adequate target for the introduction of the treatment is still unclear. <h3>Objectives</h3> To elucidate the better target for the treatment of patients with SSc-ILD. <h3>Methods</h3> This was a single-center study. Patient with SSc-ILD treated with cyclophosphamide during 2008 to 2015 in our hospital were consecutively involved. SSc patients were fulfilled 1980 American College of Rheumatology (ACR) classification criteria<sup>3)</sup> or 2013 ACR/EULAR classification criteria<sup>4)</sup>. Patients were stratified into 2 groups, extensive or limited lung disease, based on the disease extent over 20% or less using CT scan<sup>2)</sup>, respectively. Baseline data of pulmonary function tests and other clinical parameters were retrospectively collected from medical records, and response at 6 months after the treatment was evaluated by the change of data of pulmonary function tests before and after the treatment. Each parameter was compared between ED and LD groups. <h3>Results</h3> Sixteen patients with SSc-ILD who were treated with cyclophosphamide were involved. Mean age was 57.3±15.1 years and ratio of female patients and diffuse cutaneous SSc were 81%, 50%, respectively. Autoantibody profiles were 2 with anticentromere antibodies (13%), 8 with anti-topoisomerase I antibodies (50%), 1 with anti-U1 ribonucleoprotein antibody (6%) and 5 with antinuclear antibody positive without SSc-specific antibodies (31%). Disease duration at diagnosis of ILD was 26.4±31.9 months. Based on the criteria, 9 (56%) were stratified as ED and 7 (44%) as LD. At baseline, %FVC was higher (86.1±14.2 vs 67.2±16.8%, p&lt;0.05) and disease duration was shorter in LD (12.6±24.8 vs 37.2±33.8 months, p&lt;0.05) but other clinical parameters including age, sex, smoking, autoantibody profiles, mean cumulative dose of prednisolone or cyclophosphamide and serum KL-6 levels were comparable between 2 groups. Response to treatment was better in patients with LD compared to ED; improvement of %FVC (6.80±5.59 vs 0.04±3.92%, p&lt;0.05), favorable outcome of %DLCO but not significant (3.37±12.77 vs 0.70±6.18%). <h3>Conclusions</h3> SSc patients with LD, who had earlier and milder disease, showed better short-term response to treatment compared to those with ED. These results suggest that earlier intervention could lead to better prognosis in patients with SSc. <h3>References</h3> Ann Rheum Dis. 2007;66:940–4. Am J Respir Crit Care Med. 2008;177:1248–54. Arthritis Rheum 1980;23:581–90. Ann Rheum Dis 2013;72:1747–55. <h3>Disclosure of Interest</h3> None declared