AB0792 Autoimmune and inflammatory disturbances in patients with pulmonary arterial hypertension associated with systemic sclerosis. (12th June 2018)

• Record Type: Journal Article
• Authors: Yudkina, N.; Volkov, A.; Martynyuk, T.; Nikolaeva, E.; Nasonov, E.
• Is Part Of: Annals of the rheumatic diseases. Volume 77(2018)Supplement 2
• Page Start: 1529
• Page End: 1529
• Publication Date: 2018-06-12
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-eular.7170
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: The prognosis of patients with pulmonary arterial hypertension associated with systemic sclerosis (SSc-PAH) is significantly worse, than other forms of PAH, and mechanisms of this phenomenon are unknown. Therefore, the isolation of autoimmune disorders is of great importance for early diagnosis and differential diagnosis, as well as the search for new therapeutic targets. Objectives: To identify the autoimmune disorders in patients with SSc-PAH. Methods: The study includes 52 pts with idiopathic pulmonary arterial hypertension (IPAH), 51 pts with SSc-PAH, 65 pts with SSc without PAH. Serum concentrations of the C-reactive protein (CRP), anticentromere antibodies (ACA) and antibodies to topoisomerase-I (anti-Scl-70) were routinely measured. The control group consists of 146 volunteers. Statistical analysis includes univariable logistic regression, ROC analysis and Kaplan-Mayer method. Results: The average age of patients with IPAH was 37.9±10.5 years, SSc-PAH – 52.3±12.7 years, SSc without PAH – 51.2±13.2. Patients did not differ in functional class (FC), which was the main criterion of comparability. Mean values of FC in groups with SSc-PAH and IPAH also did not differ (2.7±0.8 and 2.6±0.7, respectively). ACA was associated with a 15.2-fold increased odds of developing PAH in SSc (OR 15.2, 95% CI 5.4–43.0), on the contrary, presense of anti-Scl-70 associated with low risk of PAH (OR 0.5, 95% CI 0.01 to 0.21). The level of CRP in the serum was significantly higher in patients with PAH than in the control group: 4.1 (1.9, 10.0) and 0.61 (0.25, 1.9), p<0.000001, and also in comparison with patients without PAH (1.9 (0.8, 6.4), p=0.02). In pts with PAH, the level of CRP correlated with FC and right atrium pressure and 6 min walk test distance. The level of CRP was significantly higher in patients with FC III-IV compared with FC I-II and in non-surviving patients. The Kaplan-Mayer analysis showed that pts with CRP level more than 4.75 mg/L at the time of diagnosis of PAH had a significantly lower survival rate (median 48 months) than pts with normal values (median 91 months) (p<0.005), with 67% sensitivity and 61% specificity. Conclusions: SSc-PAH is a unique phenotype combining the manifestations of SSc and PAH, the pathogenetic mechanisms of which modify the course of these states. It is based on a feature of autoimmunity with the predominance of ACA and a low occurrence of anti-Scl-70. An increase in the concentration of CRP, as well as the effect of its baseline level on survival, attests to the significant role of autoimmunity and inflammation in the pathogenesis of this fatal SSc complication. Disclosure of Interest: None declared