AB0655 SCLERONET®: A PATIENT-CENTERED MULTIDISCIPLINARY APPROACH TO SYSTEMIC SCLEROSIS

Abstract

Background: Systemic Sclerosis (SSc) is a rare connective tissue disease which may affect almost the whole body1. Because of its clinical complexity and heterogeneity, a multidisciplinary approach is helpful to patients. Objectives: The project aims at providing evidence that a multidisciplinary approach allows to early detect and rightly treat diseases linked to SSc determining an improvement of the Quality of Life (QoL) for patients. Methods: GILS, the Italian SSc Patient Organisation, selected 4 expert centers in Milan (where over one thousand patients are treated), among those able to diagnose and cure SSc in its different and difficult aspects. Inside these hospitals excellences have been identified: immuno-rheumatologists (overall management of SSc), cardiologists (cardio-pulmonary involvement and Pulmonary arterial Hypertension - PAH), maxillofacial and plastic surgeons (lipofilling), odontologists (difficult dental cure due to microstomia), dermatologists (prevention of skin cancer), ophthalmologists (dryness of the eye), vasculars (digital ulcers - DUs), pneumologists (lung fibrosis), gynaecologists (dryness of the vagina) and nutritionists (malabsorption). Specialists can share, care and cure inside this net. A monthly meeting is held with patient representatives (PRs). No private data are disclosed. Hospitals General and Sanitary Managers must give a positive evaluation every year in order to continue ScleroNet® project. Results: The monthly meeting allows to understand strength and threads and find the way to improve ScleroNet®. The PRs point out the unmet needs2, to keep ScleroNet® patient-centered and to focus the attention on the QoL whose improvement has been described by patients but not yet supported for all interventions by questionnaires. Common inclusion and exclusion criteria have been established. Cardiologists performed 110 assessments through EKG, echocardiography and cardiopulmonary exercise testing. These tests gave the following diagnosis: 16 non-ischemic cardiomyopathy (after BNP and troponin dosage as well as cardiac MRI) got an immunosuppressive therapy; 22 PAHs; 34 diastolyc disfunctions; 23 respiratory disease. The rest did not receive a specific diagnosis but still in follow up. 70 perioral lipofillings have been carried out: body weight rose by 2 to 3 kg after 4 months in patients with low BMI thanks to a greater mouth opening. Moreover, a better elasticity of the perioral area led to better oral hygiene, speech and self-perception. 115 patients before anti-TNF therapy have been screened to prevent non-melanoma skin cancer; 6 the diagnosis. 10 lipofillings for DUs nonresponsive to pharmacological therapy. UCLA/MUST questionnaires were given to selected patients in order to evaluate the GI involvement and undergo a nutritionist evaluation. A non-rheumatologist made his first SSc diagnosis due to the personal increased knowledge. Conclusion: ScleroNet® provides a fruitful practical support to patients in order to receive the best care and cure inside the experts centers in a delimited area, according to their specific problems. ScleroNet® seems to enable the experts to networking specifically on SSc, giving the opportunity of sharing their knowledge. Without ScleroNet® the timing of treatment and/or diagnosis would have been delayed. Nevertheless, due to the fact that it is a work-in-progress and some experts have been enrolled in a second stage, data are available only for some interventions. The use of validated questionnaires and indicators are necessary to measure and prove the value of ScleroNet®.