AB0569 SPECIFIC FEATURES OF VASCULAR DISORDERS IN SYSTEMIC SCLEROSIS-OVERLAP SYNDROMES

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 (ppComparing 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 (ppp=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 patientsAnalyzed featuresSScn=100 (%)SSc- overlap syndromesn=100 (%)SSc-PM/DMn= 68 (%)SSc-RAn=32 (%)Raynaud phenomenon100 (100)100 (100)68 (100)32 (100)Digital trophic disorders: scars19 (19)19 (19)12 (18)7 (22) ulcers34 (34)**19 (19)**16 (23,5)3 (9)* necrosis16 (16)***1 (1)***1 (1,5)***0 (0)***Telangiectasia60 (60)*46 (46)*34 (50)12 (37,5)*Osteolysis37 (37)*24 (24)*16 (23,5)8 (25)* рConclusion: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