AB0808 Multicentric study of systemic scleroderma in tunisia
Authors: S. Hammami, S. Bouomrani, O. Berriche, A. Barhoumi
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Scleroderma is an autoimmune connective tissue disorder which is characterised by fibrosis of visceral organs, skin and blood vessels. This condition can be localised or systemic. Its estimated prevalence is 250 cases in a million and it is more common in women than in men. <h3>Objectives</h3> The aim of this retrospective multicenter study was to analyse demographic, clinical, laboratory features and outcome of SSc in Tunisia throughout 8 Departments of Internal Medicine and to compare them with those of other geographic groups. <h3>Methods</h3> One hundred and eight cases of SSc were recorded (American College of Rheumatology criteria) during a 15 years period. They were 93 women and 15men with an average age at SSc onset of approximately 46.9 years.<sup>17–75</sup> <h3>Results</h3> Only 18 patients had limited cutaneous SSc, 11 patients had a CREST syndrome. Our Tunisian patients were characterised by a high frequency of cutaneous signs: sclerodactily (80.6%), proximal sclerosis (81%), telangiectasia (34.3%), Raynaud’s syndrome (91%), pigmentation disorders (36%), ulceration (24%) and subcutaneous calcification (4.6%). The other clinical manifestations were dysphagia in 54% (n=59), pulmonary involvement in 55.6% (n=60), cardiac manifestation in 27% (n=29), arthritis in 19.4% (n=21), renal involvement in 13.8% (n=15), and neurological involvement in 12 cas. 78.7% were antinuclear antibody (ANA) positive, 25% were Antitopoisomerase-I antibodies (anti-Scl-70 antibody) positive and 10% with anti-centromere antibodies. SSc was associated to Sjögren’s syndrome n=27, systemic lupus erythematosus (n=8), Polymyositis (n=5) and Rheumatoid arthritis (n=1) Treatment regimen included, calcium channel blockers (65.7%), steroids (47%), Colchicine (47.2%), D penicillamine (21%), immuosuppressive therapy was added in patient with partial control (n=13). Median follow-up period was 3.7 years. With the above treatment protocol, (29.6%) patients achieved disease control on treatment, (43.3%) had partial control while (26.8%) showed no response or progressive disease, Six patients died, three of them with scleroderma renal crisis. <h3>Conclusions</h3> The findings of this study documents The high frequency of extensive cutaneous sclerosis, Potential limitations and biases in our study need discussion. Specific recruitment of patients in tertiary referral centres may be the source of selection bias. Patients were evaluated by different doctors. The therapeutic management and outcome monitoring were heterogeneous. This study remains the most representative of Tunisian Scleroderma patients recruited from all parts of Tunisia <h3>References</h3> [1] Rongioletti F, Ferreli C, Atzori L, Bottoni U, Soda G. Scleroderma with an update about clinico-pathological correlation.G Ital Dermatol Venereol. 2018Jan 24. [] 2Diao M, Ndiaye MB, Kane A, Bodian M, Tchintchui NC, Mbaye A, Mounir Dia M, Sarr M, Kane A, Abdou Ba S. Pulmonary hypertension in scleroderma: about 12 cases. Pan Afr Med J. 2012;11:9. Epub 2012 Jan 18 <h3>Acknowledgements</h3> Al the group of the scleroderma Study STMI <h3>Disclosure of Interest</h3> None declared