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FRI0416 Characteristics and capillaroscopic findings of systemic sclerosis patients with severe peripheral vascular involvement receiving specific vasodilator treatment
  1. Y Yalçınkaya,
  2. AU Ünal,
  3. Z Ertürk,
  4. U Gazel,
  5. S Kaymaz,
  6. A Aksoy,
  7. P Atagündüz,
  8. N İnanç,
  9. H Direskeneli
  1. Department of Internal Medicine, Division of Rheumatology, Marmara University, School of Medicine, Istanbul, Turkey

Abstract

Background Severity of peripheral vascular involvement (PVI)is known as an important determinig cause of morbidity in sytemic sclerosis (SSc). Different vasodilating agents have been found to reduce severity and contribute to healing of digital ulcers (DU)in SSc.

Objectives We aimed to evaluate the characteristics and capillaroscopic patterns of the patients with severe PVI under different vasodilator therapeutic regimens.

Methods The patients were grouped as “severe PVI” if score of PVI is ≥2 (PVT=2,digital pitting scars; PVT=3, digital tip ulcerations and PVT=4, digital gangrene; Medsger) and “non-severe PVI” if score of PVI is ≤1 (PVI=0,no Raynaud's; PVI=1, Raynaud's requiring vasodilators). We included patients with severe PVI who received cyclic iloprost and bosentan and/or sildenafil and compared to non-severe PVI. Nail fold video-capillaroscopy (NVC)was assessed qualitatively (Cutalo et al., early, active and late patterns).

Results Severe PVI group more frequently had diffuse cutaneous form, contractures, lung disease, anti-Scl70 positivity and high acute phase response and exposed to immunosuppressives (table-1).

Table 1.

Demographics and characteristics of SSc Patients

Scores of telangiectasia, skin, activity and severity were lower in non-severe group. NVC late pattern was frequent and early pattern was rare in severe PVI groups. Forty-one% (9/22) of patients received second oral agent for PVI. Monotherapy and combination groups had similar scores (table-2).

Conclusions NVC late pattern was frequent in severe PVI group with long disease duration, exposed to intensive immunosuppresives and received specific vasodilators for PVI. Disease activity and severity were higher in severe PVI group. One third of the severe group required oral combination therapy for PVI. Monotherapy and combination groups were similar in terms of severity. NVC is a useful method to monitor digital ischemia and severe organ involvement in SSc.

Disclosure of Interest None declared

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