AB0569 SPECIFIC FEATURES OF VASCULAR DISORDERS IN SYSTEMIC SCLEROSIS-OVERLAP SYNDROMES.
Authors: O. Desinova, M. Starovoytova, L. P. Ananyeva
Journal: Annals of the Rheumatic Diseases (2020)

Background: The Systemic Sclerosis-overlap syndromes are a typical representative of the well-known group of overlap syndromes in rheumatology, causing great difficulties in diagnostic and therapeutic aspects. Objectives: To identify specific features of vascular disorders in patients with Systemic Sclerosis-overlap syndromes. Methods: 100 patients (pts) were followed, including 68 pts with Systemic Sclerosis-polymyositis/dermatomyositis (SSc-PM/DM) (f:m 52: 16), aged 17 to 74 years (mean 43±13.9) and mean disease duration 5 [2-7] years, and 32 pts with Systemic sclerosis-Rheumatoid arthritis (SSc-RA) (f:m 31:1), aged 17 to 74 years (mean age 48±14.8) and average disease duration 11.5 [5-18,5] years. The comparison group consisted of 100 pts with Systemic sclerosis (SSc) (f:m 90:10), aged 51 to 75 years (mean 51±12.9) and mean disease duration 10.6 [5-13,5] years. Results: In 1/3 of pts with SSc-PM/DM, the Raynaud’s phenomenon (RP) preceded the emergence of skin lesions and was the first symptom of the disease (33%), developing in the first year in the majority (68%) of cases, being a unique sign of the disease in only 6 (9 %) pts, while in all other cases it was associated with articular involvement, skin lesions and muscle disease. In the first year of the disease 7 pts (10%) already had signs of digital ischemia (scars, ulcers), and by the end of 3 years such signs were present in 1/3 of SSc-PM/DM pts (32%). 29 pts (43%) developed recurrent digital trophic disorders, such as digital scars in 12 (18%), digital ulcers in 16 (23.5%) and digital necrosis of the distal phalanx in 1 pts (1.5%). In 25 (78%) SSc-RA pts there was a gradual, monosymptomatic – with isolated RP - onset of the disease, similar to chronic cutaneous cSSc. In 29 SSc-RA (91%) pts, RP developed in the first year, and in 23 (72%) it was the first sign of the disease, and in 12 (37.5%) it preceded the development of skin and joint symptoms. Vascular disorders progressed with increasing duration and activity of the process. 7 patients (22%) developed recurrent digital trophic disorders - predominantly finger scars, while 3 (9%) pts had digital ulcers, developing in 11.66±7.62 years on average from the onset of the disease. Correlation analysis revealed a link between patients’ age of and presence of telangiectasias ( p &lt;0.02). Digital trophic disorders were significantly more frequent in patients with longer disease duration ( p &lt;0.05). Comparing of SSc-overlap syndromes and SSc pts showed, that although RP was present in all of them, nevertheless digital vascular-trophic disorders such as scars, ulcers ( p &lt;0.02) and necrosis ( p &lt;0.001) were less likely to develop in pts with SSc-overlap syndromes, similarly, telangiectasia and osteolysis were also significantly less frequent ( p =0.04) distinguishing SSc-overlap syndromes from limited SSc (Table). Although, SSc-overlap syndromes pts had more severely affected muscles and joints as compared to SSc. Table 1. Comparative characteristics of vascular disorders in SSc and SSc patients Analyzed features SSc n=100 (%) SSc- overlap syndromes n=100 (%) SSc-PM/DM n= 68 (%) SSc-RA n=32 (%) Raynaud phenomenon 100 (100) 100 (100) 68 (100) 32 (100) Digital trophic disorders: scars 19 (19) 19 (19) 12 (18) 7 (22) ulcers 34 (34)** 19 (19)** 16 (23,5) 3 (9)* necrosis 16 (16)*** 1 (1)*** 1 (1,5)*** 0 (0)*** Telangiectasia 60 (60)* 46 (46)* 34 (50) 12 (37,5)* Osteolysis 37 (37)* 24 (24)* 16 (23,5) 8 (25) * р&lt;0,05, ** р&lt;0,03, *** р&lt;0,001 Conclusion: Digital trophic disorders (ulcers, necrosis), as well as telangiectasia and osteolysis are significantly less likely to develop in SSc-overlap syndromes than in SSc, which should be taken into account when panning therapeutic strategies in this category of patients. Disclosure of Interests: None declared