Improvement of Hand Dysfunction by Arthritis in Systemic Sclerosis

Study Overview
=================
Brief Summary
-----------------
Systemic sclerosis (SSc) is an orphan, multiorgan disease affecting the connective tissue of the skin and several internal organs. Beside skin involvement, digital ulcers, tendinitis, calcinosis and flexion contractures, the presence of hand arthritis is a major contributor to impairment of hand function in systemic sclerosis. Several immunomodulatory drugs used in other rheumatic diseases (including methotrexate, leflunomide, azathioprine, mycophenolate mofetil and low-dose corticosteroids) can potentially improve arthritis and consequently hand function in systemic sclerosis. For the assessment of arthritis, the CDAI (clinical disease activity index) is validated in rheumatoid arthritis, and may be useful for SSc-related arthritis, too. This observational trial is part of the collaborative project DeSScipher, one out of five observational trials to decipher the optimal management of systemic sclerosis. Aim of this observational trial is to: investigate the efficacy and safety of different treatments on hand dysfunction in systemic sclerosis patients with hand arthritis and to validate the CDAI for arthritis in systemic sclerosis.

Official Title
-----------------
The DeSScipher Project - to Decipher the Best Treatment for Systemic Sclerosis - Observational Trial 2: Improvement of Hand Dysfunction by Arthritis in Systemic Sclerosis

Conditions
-----------------
Systemic Sclerosis, Arthritis

Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Juvenile and adult Systemic sclerosis patients, with diagnosis according to the ACR/EULAR adult SSc criteria and PRES/ACR/EULAR juvenile SSc criteria respectively Clinical signs of arthritis (defined as ≥2 tender and swollen joints) Exclusion Criteria: Presence of significant, long standing articular pain due to other cause than autoimmune disease Presence of hand disability caused by other, than autoimmune disease
Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| methotrexate<br>methotrexate with or without low-dose corticosteroids | |
| other DMARDs<br>other DMARDs (leflunomide, azathioprine, mycophenolate mofetil) with or without low-dose corticosteroids | |
| low-dose corticosteroids<br>low-dose corticosteroids without DMARDs | |
| No DMARD or corticosteroids<br>No DMARD or corticosteroid treatment | |
| CPH/CSA - Exploratory cohort<br>cyclophosphamide or cyclosporine-A with or without other DMARDs or low-dose corticosteroids | |
| Biologics - Exploratory cohort<br>Biologic therapy with or without other DMARDs or low-dose corticosteroids | |
| Combinations - Exploratory cohort<br>Combination of two or more DMARDs with or without low-dose corticosteroids | |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Improvement of HAQ-DI (CHAQ-DI in jSSc) by at least -0.21 in one year | Improvement from baseline in Health Assessment Questionnaire - Disability Index (Child Health Assessment Questionnaire - Disability Index in juvenile systemic sclerosis) by at least -0,21(moderate improvement) in one year | 12 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Improvement of the CHFS in one year | Improvement of the Cochin Hand Function Scale in one year | 12 months | 
| Improvement of the CDAI in one year | Improvement of the Clinical Disease Activity Index in one year | 12 months | 
| Improvement of the SDAI in one year | Improvement of the Simplified Disease Activity Index in one year | 12 months | 
| Improvement of the DAS28(We) in one year | Improvement of the Disease Activity Score 28 (using 4 variables, including erythrocyte sedimentation rate) in one year | 12 months | 
| Improvement of the DAS28(CRP) in one year | Improvement of the Disease Activity Score 28 (using 4 variables, including C-reactive protein) in one year | 12 months |