FRI0372 The ducas: proposal for a digital ulcer assessment score in scleroderma
C Bruni, T Ngcozana, F Braschi, S Guiducci, S Bellando-Randone, YA Suliman, J Grotts, CP Denton, DE Furst, M Matucci-Cerinic
2017
Poster Presentations
unpublished
Interstitial lung disease (ILD) is currently the primary cause of death in systemic sclerosis (SSc). Thoracic high-resolution computed tomography (HRCT) is considered the gold standard for diagnosis. Recent studies have proposed several clinical algorithms to predict the diagnosis and prognosis of SSc-ILD. Objectives: To test the clinical algorithms to predict the presence and prognosis of SSc-ILD, and to evaluate the association of extent of ILD with mortality in a cohort of SSc patients.
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... ds: Prospective cohort study, including 177 SSc patients assessed by clinical evaluation, laboratory tests, pulmonary function tests, and HRCT. Three clinical algorithms, combining lung auscultation, chest radiography and % predicted forced vital capacity (FVC)[1], were applied for the diagnosis of different extents of ILD on HRCT. Univariate and multivariate Cox proportional models were used to analyze the association of algorithms [1,2] and the extent of ILD on HRCT with the risk of death using hazard ratios (HR). Results: The prevalence of ILD on HRCT was 57.1% and 79 patients died (44.6%) in a median follow-up of 11.1 years. For identification of ILD with extent ≥10 and ≥20% on HRCT, all algorithms presented a high sensitivity (>89%) and a very low negative likelihood ratio (<0.16). For prognosis, survival was decreased for all algorithms, especially the algorithm C (HR 3.47, 95% CI 1.62-7.42), which identified the presence of ILD based on crackles on lung auscultation, findings on chest X-ray or FVC <80%. Extensive disease as proposed by Goh and Wells (extent of ILD >20% on HRCT or, in indeterminate cases, FVC <70%) had a significantly higher risk of death (HR 3.42, 95% CI 2.12 to 5.52). Survival was not different between patients with extent of 10 or 20% of ILD on HRCT, and analysis of 10-year mortality suggested that a threshold of 10% may also have a good predictive value for mortality. However, there is no clear cutoff above which mortality is sharply increased. Conclusions: Clinical algorithms had a good diagnostic performance for extents of SSc-ILD on HRCT with clinical and prognostic relevance (≥10 and ≥20%), and were also strongly related to mortality. Therefore, they probably could be used to obviate the requirement of HRCT in some cases. References: [1] Steele R, Hudson M, Lo E, Baron M. Clinical decision rule to predict the presence of interstitial lung disease in systemic sclerosis. Arthritis Care Res (Hoboken) 2012;64:519-24. [2] Goh NSL, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, et al. Interstitial lung disease in systemic sclerosis: A simple staging system. Am
doi:10.1136/annrheumdis-2017-eular.5735
fatcat:6pddf4a7ffardpi5vg2v4pvzgm