SPRING Project
Systemic sclerosis Progression INvestiGation
Project Leaders
Clodoveo Ferri - Cattedra di Reumatologia Università di Modena e Reggio Emilia, clferri@unimore.it
Marco Matucci Cerinic - Cattedra di Reumatologia Università di Firenze, cerinic@unifi.it
Background
Systemic sclerosis (SSc) is a disease of multifactorial etiology still largely unknown, whose pathogenesis includes alterations of the immune system, diffuse microangiopathy and an overproduction of collagen by fibroblasts. The incidence and prevalence of the disease have not been sufficiently investigated in our country, and also the expression and the clinical evolution present with an extremely variable picture. From a therapeutic point of view, SSc is one of the rheumatic diseases that is the most difficult to treat with a particularly severe prognosis; early diagnosis may prove decisive for the quality of life of patients and survival.
The innovative feature of the project
At present, there is no registry that collects the data of Italian SSc patients. This would enable the study of the geographic distribution, clinical and serological characteristics of the disease and its possible relationship with possible causal factors in the context of the Italian population characterised by marked differences both of a genetic and environmental-climatic nature. A prospective evaluation of patients would enable the identification of risk factors for disease evolution from the earliest to the full-blown stages and with organ involvement.
Objectives
General objective
Describe the clinical, laboratory and instrumental characteristics of patients with SSc and identify the characteristics associated with the evolution of the disease from the early pre-scleroderma stages (very early systemic sclerosis) to the full-blown stages with organ involvement ending in death.
SPECIFIC OBJECTIVES:
Descriptive objectives
- Evaluate the frequency of the clinical manifestations of the different stages of the disease
Cross-sectional objectives
- Identify patients with SSc, including the early stages of the disease (apparently isolated Raynaud’s phenomenon or other cutaneous and/or organ symptoms typical of the disease) in the various regions of Italy
- Role of the possible (co-) causative factors in the disease in general and of patients of different geographical origins within the national territory.
Longitudinal objectives
- Evaluate the incidence of 'vascular' complications such as cutaneous ulcers, pulmonary hypertension and scleroderma renal crisis
- Assess mortality and causes of death
- Assess the direct and indirect costs of the disease and the quality of life of patients