Background:systemic sclerosis (SSc) in a chronic autoimmune disease characterized by endothelial dysfunction, diffuse microangiopathy, and fibrosis of skin and visceral organs. Typical cardiac involvement may includes microvascular ischemia, contraction band necrosis, and patchy fibrosis, leading mainly to arrythmias and conduction defects, diastolic dysfunction, or right ventricular failure (secondary to pulmonary arterial hypertension) [1]. Valvular diseases are poorly described and generally not considered a typical sign of SSc [2-4].Objectives:we aimed to describe valvular alterations in a multicentre cohort of SSc patients.Methods:we consecutively recruited 118 SSc patients (M/F: 14/104, mean age 56.7±12.4 years, median disease duration 10 years, limited/diffuse skin subsets: 95/23, anti-centromere/anti-Scl70/others autoantibodies: 35/37/46) in 3 Rheumatology Centres in Sicily, Italy, from January to December 2019.Considering the cardiovascular risk factors, 40 (34%) patients were smokers, 7 (6%) diabetics, 12 (10%) showed hypercholesterolemia, 38 (32%) arterial hypertension, while none was obese. Transthoracic echocardiogram was carried out in all patients during their follow-up.Results:valvular abnormalities were as follow: mitral valve: insufficiency 85 (72%) cases - mild in 77/85, stenosis 2 (2%) - mild in 25/28, sclerosis/tickening 36 (30%), and calcification 9 (8%) patients; aortic valve: insufficiency 28 (24%), stenosis 4 (3%), sclerosis 29 (25%), and calcification 7 (6%) patients; tricuspid valve: insufficiency 91 (77%) cases, no cases of stenosis, sclerosis 5 (4%), and calcification 1 (1%) patients; pulmonary valve: insufficiency in 13 (11%) patients.As expected, tricuspid insufficiency (TI) was associated with pulmonary arterial hypertension (PAH) (moderate TI in 20% of patients with every TI and PAH vs. 4% of patients with TI without PAH, p=0.019).Aortic sclerosis (AS) was associated with the presence of arthritis (AS in 35% of patients with arthritis vs. 16% of patients without, p=0.029).No association was found with age, gender, disease duration, skin subset, autoantibodies, capillaroscopic patterns, presence of digital ulcers, lung, renal, or digestive involvements.Conclusion:in this multicentre SSc cohort study, we found that cardiac valve alterations are very common, even though generally not clinically relevant. The presence of PAH was associated with more severe TI. Finally, AS was associated with arthritis that could be considered sign of chronic inflammatory state, which is often linked with accelerated atherosclerosis and remodeling process of aortic valve [5].References:[1]Lambova S. Cardiac manifestations in systemic sclerosis. World J Cardiol 2014; 6:993-1005.[2]D’Angelo W, Fries JF, Masi AT, Shulman LE. Pathologic observations in systemic sclerosis (scleroderma).A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med 1969; 46:428-440.[3]Kazzam E, Caidahl K, Hallgren R, et al. Mitral regurgitation and diastolic flow profile in systemic sclerosis. Int J Cardiol 1990; 29:357-363[4]Wranicz J, Zielińska M, Cygankiewicz I, et al. Early cardiovascular involvement in patients with systemic sclerosis (SSc). Med Sci Monit. 2002; 8:CR78-82.[5]Coté N, Mahmut A, Bosse Y, et al. Inflammation is associated with remodeling of calcific aortic valve disease. Inflammation 2013; 36:573-581.Disclosure of Interests:Michele Colaci: None declared, Claudia Schinocca: None declared, Ylenia Dal Bosco: None declared, Maria Letizia Aprile: None declared, Giuliana Guggino Grant/research support from: Pfizer, Celgene, Speakers bureau: Celgene, Sandoz, Pfizer, Ilenia De Andres: None declared, Alessandra Azzurra Russo: None declared, Domenico Sambataro: None declared, Gianluca Sambataro: None declared, Lorenzo Malatino: None declared
Increased plasma soluble CD40 ligand concentrations in systemic sclerosis and association with pulmonary arterial hypertension and digital ulcers
