AB0890 EARLY SYSTEMIC SCLEROSIS PATIENTS SHOW A SLOW DISEASE COURSE

Abstract

BackgroundSystemic sclerosis is a heterogenous immune-mediated connective tissue disease characterized by vasculopathy and progressive tissue and organ fibrosis. [1]ObjectivesOur aim was to determine characteristics of Finnish patients with systemic sclerosis (SSc) and early SSc that were followed during 1996-2018 in two university hospitals in Finland.MethodsThe data of patients with SSc with ICD-10 diagnostic code (M34) were collected retrospectively from medical registers. The patients were reclassified into different subsets based on the ACR/ EULAR (American College of Rheumatology and European League Against Rheumatism) 2013 classification criteria of the SSc. Early SSc was defined as a condition that did not fulfil the classification criteria (less than 9 points). Diffuse (dcSSC), limited cutaneous (lcSSc), overlap and sine scleroderma subsets were differentiated by their clinical phenotype.Results336 patients were obtained after re-evaluation of data. Out of 336 patients 40 (11.9%) patients had dcSSc, 222 (66.1 %) lcSSc, 16 (4.8%) SSc-overlap syndrome, 2 (0.6%) SSc sine scleroderma and 56 (16.7%) early SSc. The early SSc group did not develop any of the most severe manifestations such as interstitial lung disease (ILD), the incidence of which was 32.7/1000 patient years in the whole SSc group, n=280, pulmonary arterial hypertension (PAH) (14.3/1000 patient years), renal crisis (RC) (3.5/1000 patient years), myocarditis (1.2/1000 patient years) during the 4.3 [IQR 1.9-6.7] years of follow-up. The SSc group had significantly more other manifestations as digital ulcers (41.1 vs. 6.7/ 1000 patient years, p<0.001, SSc and early SSc, respectively), reduced mouth opening (44.3 vs. 10.1, p<0.001) and manometry positive oesophageal dysmotility (21.1 vs. 10.3, p=0.007) than patients with early SSc. ILD was diagnosed 2.8 (SD 4.5) years, PAH 5.6 (SD 8.7) years and RC 0.2 (SD 0.3) years after the onset of the first non-Raynaud’s phenomenon symptom in SSc patients. The patients with dcSSc had worse survival than lcSSc patients (after 5 years 81.9% vs 91.6%, after 10 years 67.3% vs 82.9%, p<0.001 and HR=2.7 (1,6-4,4), p<0.001). The SSc-overlap and early SSc subsets did not have difference in survival when compared to lcSSc subset.ConclusionDisease course of patient with early SSc was slow or mild since none of them developed any severe complication during the median follow-up time of 4.3 years.Reference[1]Cutolo M, Soldano S, Smith V. Pathophysiology of systemic sclerosis: current understanding and new insights. Expert Rev Clin Immunol. 2019;15:753–64.Table 1.The rates of different organ manifestations as reported cases per patient year for SSc and early SSc patientsSScEvents/1000 pyEarly SScEvents/1000 pyp-valuePatients28056Vasculitis4/275 (1.5)1.60/55 (0)0.00.611Gangrene18/274 (6.6)7.51/54 (1.8)3.30.218Amputation20/274 (7.3)8.01/55 (1.8)3.20.147Digital ulcer81/275 (29.5)41.12/55 (3.6)6.7<0.001Decreased mouth opening79/274 (28.8)44.33/55 (5.2)10.1<0.001Arthritis60/273 (22.0)29.314/55 (25.5)57.30.597Myositis3/273 (1.1)1.20/55 (0)0.00.650Cardiac9/273 (3.3)3.50/55 (0)0.00.234Gastrointestinal105/275 (38.2)67.315/55 (27.3)72.70.166Manometry positive46/57 (80.7)21.13/9 (33.3)10.30.007Renal crisis9/274 (3.3)3.50/54 (0)0.00.365ILD68/276 (24.6)32.70/55 (0)0.0<0.001ILD and transplantation1/67 (1.5)0.4---PAH35/275 (12.7)14.30/55(0)0.00.007PAH and transplantation1/35 (2.9)0.4---Calcinosis60/275 (21.8)29.79/55 (16.4)34.00.468Acro-osteolysis18/275 (6.5)7.70/55 (0)0.00.094Results are expressed as n (%) for qualitative variablesFigure 1.Kaplan-Meier survival curves from the first non-Raynaud’s phenomenon symptom for lcSSc and dcSSc patientsAcknowledgements:NIL.Disclosure of InterestsMarkus Käyrä: None declared, Saara Kortelainen Speakers bureau: Boehringer Ingelheim, UCB, Consultant of: Boehringer Ingelheim, Johanna Paltta Speakers bureau: UCB, Laura Pirilä Speakers bureau: Abbvie, Boehringer Ingelheim, Jansen-Cilag, Novartis Finland, Sandoz, Eli Lilly, Swedish Orphan Biovirtum, Consultant of: Abbvie, Boehringer Ingelheim, Jansen-Cilag, Novartis Finland, Sandoz, Eli Lilly, Swedish Orphan Biovirtum, Hannu Vähänikkilä: None declared, Riitta Kaarteenaho Speakers bureau: Boehringer-Ingelheim, Roche, Consultant of: Boehringer-Ingelheim, MSD, Johanna Huhtakangas Speakers bureau: Boehringer Ingelheim, Abbvie, Amgen, Novartis, Consultant of: Boehringer Ingelheim, Abbvie, Amgen, Novartis.