Clinical Research Directory

Inclusion Criteria: 1. Provide signed and dated informed consent; 2. Willing to comply with all study procedures and be available for the duration of the study; 3. Male or female, aged ≥ 18 and ≤ 70 years of age 4. SSc patients according to American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) 2013 classification criteria for SSc 5. Severe disease with either: * disease duration of 2 years or less with a modified Rodnan skin score (mRss) ≥ 20 and (abnormal CRP \> 5 mg/l and/or hemoglobin \< 11 g/dL), or * mRSS ≥ 15 without any restriction as to disease duration plus at least one major organ involvement as defined by: 1. respiratory involvement consisting of lung diffusion capacity for carbon monoxide (DLCO) and/or forced vital capacity (FVC) \< 80% predicted and evidence of interstitial lung disease : bronchiolar involvement, areas of ground-glass contusions or fibrosis (chest X-ray and/or high resolution computed tomography (HRCT) scan) and/or moderate Pulmonary hypertension with baseline resting systolic pulmonary arterial pressures \> 35 mmHg and below 50 mmHg by cardiac echocardiography, or mean pulmonary artery pressure \> 20 mmHg and \< 40 mm Hg on right heart catheterization; 2. renal involvement consisting of past renal crisis, microangiopathic hemolytic anemia, and/or renal insufficiency not explained by other causes than SSc; 3. cardiac involvement consisting of reversible congestive heart failure, atrial or ventricular rhythm disturbances such as recurrent episodes of atrial fibrillation or flutter, recurrent atrial paroxysmal tachycardia, 2nd or 3rd degree AV-block, mild to moderate pericardial effusion and/or presence of MRI involvement (Increased T1 or T2 mapping, late gadolinium enhancement, septal D sign). All causes of organ involvement should be attributed to SSc. 6. Contraindication, inadequate response or unwillingness to undergo AHSCT(determined by patient and physician judgement) 7. Contraindication, inadequate response or unwillingness or adverse events necessitating discontinuation of conventional immunosuppressive therapy (MMF, methotrexate); 8. Women of reproductive potential must use highly effective contraception; 9. Men of reproductive potential must use condoms; 10. Health insurance. Exclusion Criteria: 1. Age \< 18 years and \> 70 years 2. Pregnancy or unwillingness to use adequate contraception; 3. Life-threatening end-organ damage defined as: DLCO (corrected for hemoglobin) \< 30% predicted; Left ventricular ejection fraction \< 40% by cardiac echocardiography; Pulmonary hypertension with baseline resting systolic pulmonary arterial pressures \> 50 mmHg by cardiac echocardiography, or mean pulmonary artery pressure \> 40 mmHg on right heart catheterization; glomerular filtration rate \< 30mL/min 4. Active Hepatitis (ASAT, ALAT \> 3 normal) 5. Neoplasms of less than 5 years, except for basal cell or in situ cervix carcinoma or concurrent myelodysplasia, 6. Uncontrolled hypertension 7. Uncontrolled acute or chronic infection 8. HIV-1 or HIV-2 infection 9. Body Mass Index \< 16.5 kg/m2 10. Severe psychiatric disorder 11. Bone marrow insufficiency, defined as neutropenia \< 1 x 109/L, thrombopenia \< 50 x 109/L, anemia \< 8 g/dL, lymphopenia \< 0,5 x 109/L 12. Inability to provide informed consent 13. Patient included in another interventional clinical trial 14. Patient under tutelle