AB0841 CONCORDANCE AND PROGNOSTIC RELEVANCE OF DIFFERENT DEFINITIONS OF SYSTEMIC SCLEROSIS INTERSTITIAL LUNG DISEASE PROGRESSION

Background Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc) with variable natural history and prognosis. Several definitions SSc-ILD progression have been proposed including minimal clinical important worsening forced vital capacity (FVC MCIW), EUropean Scleroderma Trials Research group (EUSTAR) progression, Outcome Measures in Rheumatology Clinical (OMERACT) Erice ILD working progression. The aim the study was to assess concordance prognostic value these different patients overall specific subsets. Objectives Progression status assessed consecutive over period 24 months mortality related compared between progressors non-progressors according subsequent 60 months. Severe baseline pulmonary functional impairment defined for combination FVC<80% alveolar diffusion CO (DLco) <60% predicted. Methods Results retrospective analysis included 245 (age 54.6±13.2 years, male 18.8%) 54.7% prevalence od diffuse cutaneous variant median duration 4 years (IQR 2-8). Disease ≤ 3 severe impairment, artery systolic pressure (PASP) 40 mmHg were reported 47.8%, 18.0% 17.1% patients, respectively. Twenty-six deaths among enrolled FVC MCIW (HR 2.27, 95% IC 1.03 - 4.97, p=0.041), OMERACT 2.90, 1.28 6.57, p=0.011), criteria 11.02, 2.38 51.08, p=0.002) associated whole cohort. Prognosis prediction, considering status, challenging (independently from adopted criteria) >3 mild function test PASP ≥40 mmHg. performed better ≤3 limited variant, <40 mmHg, while impairment. Conclusion are not interchangeable, since up one third could be misdiagnosed using criteria. Regardless used, progressor presented more frequently skin disease, shorter worse lung. REFERENCES: NIL. Acknowledgements: Disclosure Interests None Declared.