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Treatment of Refractory Sever Systemic Scleroderma by Injection of Allogeneic Mesenchymal Stem Cells

Phase 1
Completed
Conditions
SYSTEMIC SCLERODERMA
ALLOGENEIC MESENCHYMAL STEM CELLS
ADULT
Interventions
Biological: INJECTION OF ALLOGENEIC MESENCHYMAL STEM CELLS
Registration Number
NCT02213705
Lead Sponsor
Assistance Publique - Hôpitaux de Paris
Brief Summary

The main ailm of this phase I-II study is to evaluate toxicity and efficacy of allogenic mesenchymal stem cell therapy to treat severe systemic sclerosis. In practice this treatment will be given to patients with a rapidly evolutive disease or refractory to cyclophosphamide.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
20
Inclusion Criteria
  • Age> 18 years and <70 years.
  • Established diagnosis of systemic sclerosis according to the criteria of the American College of Rheumatology
  • SSc of poor prognosis, involving life-threatening with sever visceral impairment (cardiac, pulmonary or renal) AND " a) contraindicating the use of or b) resistant to " immunosuppressive therapy conventionally used in severe forms of the disease according to the European recommendations of EUSTAR (www.eustar.org) and EBMT (www.ebmt.org) which then rely on high doses of iv cyclophosphamide (either in monthly bolus at least six months or by intensification and autograft of Hematopoietic Stem Cells) or SSc with fibrosing lung damage threatening the vital prognosis which excludes a lung transplant.

These forms of severe and serious SSc WITH at least 6 months follow-up after completion of prior immunosuppressive therapy by high doses of iv cyclophosphamide when they were made, combine to varying degrees : rapidly progressive skin lesions with a score of Rodnan> 15 and one or more of the major visceral lesions defined as follows :

  1. Respiratory disease :

    DLCO <60% or FVC ≤70% of the theoretical value and the presence of interstitial lung disease (abnormalities on chest radiograph and / or lung HRCT with thin sections). It is necessary to ensure that non-related etiologies to scleroderma were eliminated; example: obstructive lung disease (chronic obstructive pulmonary disease or pulmonary emphysema). If the fibrosing lung disease threatens the vital prognosis, we will ensure of the exclusion of a possible lung transplant.

    And/or

  2. Heart disease:

congestive heart failure reversible, ventricular or atrial rhythm disturbances defined as recurrent episodes of atrial fibrillation or atrial flutter, recurrent paroxysmal atrial tachycardia or ventricular tachycardia, atrioventricular block of second or third degree, pericardial effusion with high abundance needing specific treatment of medical type (introduction of steroids) or surgical type (drainage). It is necessary to ensure that non-related etiologies to scleroderma were removed.

  • Signed informed consent.
  • Presence of a consenting intrafamilial MSC donor
  • Affiliation to social security.
Exclusion Criteria
  • Pregnancy or absence of appropriate contraception throughout the study.
  • Respiratory Disease:
  • systolic Pulmonary arterial pressure (PASP)>55mmHg (on echocardiography or after right heart catheterization);
  • DLCO <30% of the theorical ;
  • Respiratory failure defined by oxygen arterial pressure at rest (PaO2) <8 kPa (<60 mmHg) and / or a blood pressure of carbon dioxide at rest (PaCO2)> 6.7 kPa (> 50 mmHg) without oxygen therapy.

Renal Disease:

  • Calculated creatinine clearance <20 ml/mn/m2
  • Sequelae cystopathy post treatment by cyclophosphamide
  • Heart disease:
  • Clinical sign of a congestive heart failure refractory ;
  • Left ventricular ejection fraction <35% at myocardial scintigraphy or echocardiography;
  • Pulmonary arterial hypertension confirmed by right catheterization or suspected pulmonary hypertension with systolic PAP at echography > 40 mmHg
  • Chronic atrial fibrillation requiring oral anticoagulant therapy;
  • Uncontrolled ventricular arrhythmia;
  • Pericardial effusion with hemodynamic compromise assessed by echocardiography.
  • Hepatic Disease:
  • Hepatic impairment defined as a persistent increase in transaminases or bilirubin to 3 times normal.
  • Psychiatric disorders, including drug taking and alcohol abuse.
  • Active neoplasia or concomitant myelodysplasia, antecedent of neoplasia.
  • Bone marrow failure defined by neutropenia <0.5 x 109 / L, thrombocytopenia <50 x 109 / L, anemia <8 g / dL, CD4 lymphopenia <200 x 106 / L.
  • Uncontrolled systemic hypertension.
  • Uncontrolled acute or chronic infection, HIV1, 2 or HTLV-1, 2seropositivity.
  • Chronic hepatitis B or C active.
  • Significant exposure to bleomycin, toxic oils, vinyl chloride, trichloroethylene or silica; eosinophilia-myalgia syndrome, eosinophilia fasciitis.
  • Risk of poor patient compliance.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
INJECTION OF ALLOGENEIC MESENCHYMAL STEM CELLSINJECTION OF ALLOGENEIC MESENCHYMAL STEM CELLSAdministration of allogeneic MSCs in the treatment of severe diffuse SSc or rapidly progressive and refractory to conventional treatments by prior cyclophosphamide
Primary Outcome Measures
NameTimeMethod
Immediate Toxicity10 days

Immediate Toxicity/tolerance defined as grade 3 or above toxicity base on the CTCAE - Cancer Therapy Evaluation Program (CTEP), observed during the first 10 days (http://ctep.cancer.gov/protocolDevelopment/electronic_applications/docs/ctcaev3.pdf)

Secondary Outcome Measures
NameTimeMethod
Antibody3, 6, 9, 12, 15, 18, 24 months

Antibody response

Medium-term tolerance2 years

Treatment-related event-free survival at 2 years. Treatment-related event (morbidity) being defined by the onset of clinical events induced by the procedure and not explained by the natural or expected course of the scleroderma disease.

Progression free survival2 years

Defined as the time in days from the day of inclusion until the occurrence of changes compared to the initial assessment, documented and re-evaluated at two successive examinations 3 months

Survival2 years

Time from inclusion to death

Platelet1, 2, 3, 4, 8, 12, 16, 20, 24 weeks

Platelet blood count

SHAQ3, 6, 9, 12, 15, 18, 24 months

Scleroderma Health Assessment Questionnaire (SHAQ)

Clinical response3, 6, 9, 12, 15, 18, 24 months

Defined by a 25% improvement in modified Rodnan Score and/or ≥10% in DLCO or FVC compared to baseline state without the need to reintroduce other immunosuppressants.

CBC1, 2, 3, 4, 8, 12, 16, 20, 24 weeks

complete blood count (CBC)

Lymphocyte3, 6, 9, 12, 15, 18, 24 months

Lymphocyte subpopulation blood count measured by flow cytometry

Rodnan score3, 6, 9, 12, 15, 18, 24 months

modified Rodnan Score

Clinical progression3, 6, 9, 12, 15, 18, 24 months

Occurence of visceral involvement, defined as any of the following:

* Pulmonary: diffusion capacity of CO (DLCO and DLCO/VA), forced vital capacity (FVC), total lung capacity (TLC), residual volume (RV), pulmonary artery pressure (measured by echocardiography or right heart catheterisation), arterial blood gases (pO2, pCO2, p(A-a)O2) in ambient air,

* Myocardial: ECG, left ventricular function measured by echocardiography (and cardiac MRI and/or Gating in the event of cardiac abnormality or suspected LV dysfunction on ultrasound) with semi-quantitative analysis of the degree of cardiac damage according to the cardiac score (based on the presence or absence of left axial deviation on the electrocardiogram and/or moderate or significant pericardial effusion according to the echocardiogram),

* Renal: 24-hour proteinuria, creatinine clearance,

* Patient's weight in kg,

Trial Locations

Locations (1)

Saint-Louis Hospital

🇫🇷

Paris, France

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