AB0791 Specific features of skin involvement in patients with systemic sclerosis and associated pulmonary arterial hypertension
Authors: N. Yudkina, A. Volkov, T. Martynyuk, E. Nasonov
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Pulmonary arterial hypertension, associated with systemic sclerosis (SSc-PAH), is a severe irreversible manifestation of the disease, potentially fatal in its' late stage. It was shown that survival in SSc-PAH pts is much worse than in pts with idiopathic pulmonary arterial hypertension (IPAH). Unfavourable outcomes due to late recognition can be explained by predominance of subtle, clinically poor manifest SSc types, especially in terms of cutaneous and vascular syndromes. <h3>Objectives</h3> To assess the clinical features and survival rates in pts with systemic sclerosis sine scleroderma (ssSSc), associated with PAH. <h3>Methods</h3> 14 pts with ssSSc-PAH were analysed in comparison with 54 pts with clinically manifest skin involvement SSc-PAH (3 pts with diffuse (dcSSc-PAH) and 51 pts with limited cutaneous involvement (lcSSc-PAH)), and 48 pts with IPAH. <h3>Results</h3> Pts with IPAH were younger than both type SSc-PAH – 37 (28; 44), 48 (37; 56) and 54 (48; 62) y, respectively. In SSc-PAH pts with skin involvement and the diagnosis of PAH was established earlier (within 18 (10; 44) mo) than in pts with ssSSc (23 [15; 47] mo), although differences are not statistically significant. The PAH functional class was slightly higher in ssSSc-PAH, than in IPAH and SSc-PAH, the differences are not significant. Raynaud's phenomenon (RP) was present in all SSc-PAH pts, although in cutaneous SSc pts digital ischaemic lesions were more frequent (51% vs 14%, p=0.03), as well as contractures (53% vs 7%, p=0.006). There were no other differences in clinical features between the groups. Anticentromere antibodies (ACA) were present in 7 (50%) pts with ssSSc-PAH and in 36 (65%) pts with cutaneous SSc. Anti-topoisomerase-I antibodies (anti-Scl-70) were found only in 2 pts with lcSSc. More than 1 type of autoantibodies was detected in the majority of SSc pts. A wide range of antinuclear ABs was found in pts with ssSSc-PAH with prevailing ACA (in 7 pts), as well as anti-Sm, anti-La ABs, anti-nucleosome ABs (in one case), anti-Ro ABs (in 5 pts), anti-RNP-70 ABs – in 4 pts, anti-dsDNA ABs – in 2 pts, RF – in 3pts. SSc diagnosis was established according to ACR-EULAR 2013 classification criteria. The following diagnostic criteria were present in ssSSc-PAH pts: RP (in all pts), ulcers (3), scars (2), telangiectasia (10), PAH (14), SSc-associated ABs (7), capillaroscopic lesions (12). The mean total score was 11 (9;12) while ≥9 scores are required for SSc diagnosis, 100% pts with ssSSc-PAH met ACR-EULAR 2013 criteria, thus, justifying the SSc confirmation in this group of pts. There were significant differences in survival rates between IPAH pts and pts with various types of SSc-PAH (log-rank test, p=0.06). 5 year survival in ssSSc-PAH was somewhat lower, than in SSc-PAH – 50.6% vs 64.9%, respectively; IPAH pts had the best survival rates of 82.5%, and these differences are close to significant. <h3>Conclusions</h3> Clinical features and survival ssSS-PAH are very similar to those in pts with cutaneous SSc-PAH with the exception of skin involvement and associated symptoms (digital ischaemic lesions and contractures). Rheumatologists should be aware of such specific features as similar survival rates in cutaneous and ssSSc pts, and late recognition of PAH in pts with ssSSc, as well as its similarity with IPAH. <h3>Disclosure of Interest</h3> None declared