Abstract

Background:Systemic Sclerosis (SSc) is a rare, multisystemic connective tissue disease associated with significant morbidity. Early recognition of patients at risk for adverse prognosis may help towards optimized monitoring and treatment, thus improving disease outcome.Objective:To correlate nailfold videocapillaroscopy (NVC) findings (‘early’, ‘active’, ‘late’ scleroderma patterns and non-specific capillary abnormalities) with major organ involvement and prognosis in patients with systemic sclerosis (SSc).Methods:Patients from the Scleroderma cohort followed at the Rheumatology clinic of the University Hospital of Heraklion will be included. The study will include a prospective and a retrospective part. Prospective part: All newly diagnosed patients will undergo NVC at baseline and subsequently every six months. We will review demographics, clinical features and autoantibodies status. Major organ involvement will be monitored (Pulmonary Function Test, DLCO, heart echocardiogram, chest XR, modified Rodnan skin score) at baseline and then every 6–12 months. Retrospective part: Existing SSc patients with available NVC data at diagnosis will be included. We will correlate the NVC findings at the time of diagnosis with disease outcomes such as major organ involvement, end stage organ failure, need for hospitalization, and death. We will also correlate longitudinal changes of the NVC patterns with treatment responses and outcomes.

Highlights

  • Systemic sclerosis (SSc) is a rare, multisystemic connective tissue disease associated with significant morbidity

  • We have previously reported on mortality of Systemic Sclerosis (SSc) in Crete, Greece over a 5-year period (2010-2015).[1]

  • Normal pattern was reported in 27.3% and non-specific capillary abnormalities were reported in 55.5% of those patients.[3]

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Summary

March 2019 Volume 30 Issue

©Repa A, Avgoustidis N, Kougkas N, Bertsias G, Zafiriou M, Sidiropoulos P.

INTRODUCTION
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