Investigation of Articular Disease Activity and Hand Involvement in Systemic Sclerosis

Abstract

Systemic sclerosis (SSc) is a connective tissue disease characterised by autoimmune phenomena, generalised vasculopathy and fibrosis. Its heterogeneous manifestations include skin, musculoskeletal, and internal organ involvement. The cardiopulmonary, renal and gastrointestinal manifestations are the main causes of mortality, while skin and musculoskeletal involvement mainly cause disability and reduce quality of life. The severity of a systemic connective tissue disease is determined by disease activity, the potentially reversible phenomena of the disease and by the irreversible organ damage. The main therapeutic goal in the management of rheumatic diseases is reducing disease activity in order to minimize damage. On the ground of joint inflammation and fibrotic processes contractures evolve early in the course of the disease, mostly affecting the hands. Currently there is very limited evidence based therapy for arthritis in SSc. Moreover, there is an unmet need for validated tools to measure joint related disease activity in both clinical practice and in drug trials. In the management of RA the “treat to target” attitude was facilitated by the development and validation of simple tools measuring disease activity such as the Disease Activity Score of 28 Joints using Erythrocyte Sedimentation Rate (DAS28-ESR). Similarly to RA, articular involvement, such as joint swelling, tenderness, morning stiffness and contractures primarily affect the hands in SSc. The Cochin Hand Function Scale (CHFS) is a patient self-assessment questionnaire that allows quick and efficient measurement of hand function and disabilities regarding activities of daily living. Our aim was to analyse articular disease activity as well as to validate tools for measuring joint involvement in SSc focusing on the hands.