SAT0283 CLINICAL AND SEROLOGICAL FEATURES OF SYSTEMIC SCLEROSIS IN ITALIAN AND EGYPTIAN PATIENTS
Authors: Vasiliki Liakouli, Hesham Hamoud, Piero Ruscitti, Mahmoud Ibrahim Risha, Mohamed Elsayed Hanafy, Khaled Elnoafly, Rasha Ali, Gihan Omar, Adel Elsayed, Abdelazeim Elhefny, Mervat Abogabal, Sameh Mobasher, Paola Cipriani, Roberto Giacomelli
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Clinical and serological variations exist in the severity of Systemic Sclerosis (SSc) patients, according to different geographic areas. <h3>Objectives:</h3> To evaluate clinical and serological features in 2 cohorts of Italian and Egyptian SSc patients and to identify factors associated with Interstitial lung disease (ILD), a leading cause of mortality, by using multivariate logistic regression analysis. <h3>Methods:</h3> An Italian center and 3 Egyptian centers participated in SSc patient recruitment in 2017. The cross-sectional demographic, clinical, and laboratory data were collected and defined according to severity score and activity index, previously developed [1, 2]. The database included 152 consecutive Italian patients, 135 women (88.8%) and 17 men (11.2%) and 197 consecutive Egyptian SSc patients, 177 women (89.8%) and 20 men (10.2%), all of whom fulfilled the classification criteria proposed by LeRoy and Medsger [3]. <h3>Results:</h3> We found that Egyptians SSc patients were younger (41.18±12.5 vs 58.59±12.6 yrs), had an earlier onset of the first non Raynaud's Phenomenon symptom (7.28±5.89 vs 14.99±11.11) and a more severe Modified Rodnan Skin Score (MRSS&gt;14) (79.27% vs 13.6%). A greater percentage of Egyptian patients presented the active form of the disease (53.3% vs 15.5%) and had a Pulmonary Arterial Pressure (PAPs) estimated by echocardiography ≥40mmHg (37.5% vs 10.6%), than Italian patients. Furthermore, Egyptian patients affected from the limited form of the disease (lcSSc), presented a higher MRSS (67.2% vs 2.6%), higher PAPs levels (34.5% vs 9.2%), an active form (41.7% vs 7.8%) and history of past/current ulcers (62.2% vs 30.6%) than Italian lcSSc patients. Severity score was higher in Egyptian patients than in the Italians (General: 22.9% vs 6.2%,Peripheral vascular: 48.2% vs 23.9%, skin: 19.8% vs 3.6%, joint/tendon: 19.9% vs 8.5%, muscle: 8.1% vs 2.6%, lung: 41.1% vs 25.3%, heart: 11.2% vs 4.1%). Kidney involvement was more severe in the diffuse cutaneous form (dcSSc) of the disease in Egyptians patients than Italians (2% vs 0.4%). In the multivariate analysis, PAPs ≥40mmHg (OR: 2.622, 95%CI:1.031-6.660, p=0.043), past/current ulcers (OR:3.450, 95%CI:1.265-9.409, p=0.016), presence of anticentromere (ACA) (OR:3.846, 95%CI:1.488-9.940, p=0.005) and anti-topoisomerasi I (SCL70) (OR:5.012, 95%CI:1.740-14.441, p=0.003) antibodies and heart involvement (OR:3.018, 95%CI:1.448-6.288, p=0.003) were associated with ILD in the Egyptian database, suggesting a potential role as predictive biomarkers. Advanced age (OR:1.036, 95%CI:1.009-1.063, p=0.008), dcSSc (OR:4.726, 95%CI:2.021-11.049, p=0.001) and joint involvement (OR:1.695, 95%CI:1.094-2.627, p=0.018) were independently associated with ILD in the Italian patients. <h3>Conclusion:</h3> This cross-sectional study analyzed the demographic, clinical and laboratoristic variations in Egyptians and Italian patients and their association with ILD, suggesting possible differences to be further investigated in adequately powered studies. <h3>References</h3> [1] Valentini, et al. Ann Rheum Dis2001 [2] Medsger TA Clin Exp2003 [3] LeRoy EC, et al. J Rheumatol. 1988Feb <h3>Disclosure of Interests:</h3> Vasiliki Liakouli Grant/research support from: PFIZER, Speakers bureau: SANOFI, Hesham Hamoud: None declared, Piero Ruscitti Grant/research support from: Pfizer, Speakers bureau: MSD, BMS, Lilly, Sobi, Mahmoud Ibrahim Risha: None declared, Mohamed Elsayed Hanafy: None declared, khaled elnoafly: None declared, Rasha Ali: None declared, Gihan Omar: None declared, Adel Elsayed Grant/research support from: This study was funded by EVAPHARMA., Abdelazeim Elhefny: None declared, Mervat Abogabal: None declared, Sameh Mobasher: None declared, Paola Cipriani Speakers bureau: ACTELION, PFIZER, Roberto Giacomelli Grant/research support from: Pfizer, Actelion, Speakers bureau: Actelion, Bristol-Myers Squibb, Merck Sharp &amp; Dohme, Abbvie, Pfizer, Sobi, Roche