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Myositis are rare diseases for which the development of a cohort associated with a bank of biological samples (biobank) will allow for the conduct of researches to better delineate the underlying pathophysiology and find cures. This prospective cohort of patients with myositis will allow for identification of factors favouring the occurrence of myositis, whether they are constitutional (genetic) or acquired (environmental or drug). Different subgroups of myositis used for prognostication will be identified based on clinico-demographical variables, the nature of the organs involved beyond peripheral muscles (cardiac, diaphragm) and biomarkers abnormalities
Myositis is a rare autoimmune disease in which the immune system mistakenly attacks the patient's own peripheral muscles. This aggression manifests by muscle inflammation and necrosis responsible for a motor deficit of varying severity. The treatments available today are insufficient and are non-specific. Biological criteria, issued from simple blood or muscle tests are missing, and they will help to define the activity of the disease and the efficacy of treatments. The MASC protocol will include patients with myositis, and investigators will collect clinical, radiological, electrophysiological, histological and biological data to be used for researches aiming at better understanding this entity. A biobank (muscle biopsy, DNA, serum, plasma, PBMCs) will be acquired on this prospective cohort. The study itself will be composed of a baseline visit and monthly to yearly follow-up visits which will assess:
This prospective study will also aim at:
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| Measure | Description | Time Frame |
|---|---|---|
| Characterisation of the different myositis subgroups based on clinical, radiological, electrophysiological and histobiological evaluations | Characterisation of the different myositis subgroups based on clinical, radiological, electrophysiological and histobiological evaluations | baseline: first 30 days after inclusion |
| Measure | Description | Time Frame |
|---|---|---|
| Characterisation of the natural history of myositis subgroups :responses to treatments, prognosis factors, evolution | Characterisation of the natural history of myositis subgroups :responses to treatments, prognosis factors, evolution | up to twenty years after inclusion |
| Characterisation of an immune system signature, using peripheral blood mononuclear cells and muscular biopsies, DNA and RNA sequencing, and autoantibodies |
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Inclusion Criteria:
Exclusion Criteria:
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Major patients with myositis included during consultation visits or routine hospitalizations in the Internal Medicine Department, Reference Center for Neuromuscular Pathology, at the Pitié Salpêtrière Hospital
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Joe-Elie SALEM, MD PhD | Contact | 142178531 | +33 | joe-elie.salem@aphp.fr |
| Olivier Benveniste, PU PH | Contact | olivier.benveniste@aphp.fr |
| Name | Affiliation | Role |
|---|---|---|
| Olivier Benveniste, PU PH | Groupe Hospitalier Pitie-Salpetriere | Study Director |
| Yves Allenbach | Groupe Hospitalier Pitie-Salpetriere | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Département de médecine interne et immunolgie clinique, Hôpital Pitié Salpêtrière | Recruiting | Paris | 75013 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 40569849 | Derived | Power JR, Dolladille C, Ozbay B, Procureur A, Ederhy S, Palaskas NL, Lehmann LH, Cautela J, Courand PY, Hayek SS, Zhu H, Zaha VG, Cheng RK, Alexandre J, Roubille F, Baldassarre LA, Chen YC, Baik AH, Laufer-Perl M, Tamura Y, Asnani A, Francis S, Gaughan EM, Rainer PP, Bailly G, Flint D, Arangalage D, Cariou E, Florido R, Narezkina A, Liu Y, Sandhu S, Leong D, Issa N, Piriou N, Heinzerling L, Peretto G, Crusz SM, Akhter N, Levenson JE, Turker I, Eslami A, Fenioux C, Moliner P, Obeid M, Chan WT, Ewer SM, Kassaian SE, Johnson DB, Nohria A, Ben Zadok OI, Moslehi JJ, Salem JE; International ICI-Myocarditis Registry. Immune checkpoint inhibitor-associated myocarditis: a novel risk score. Eur Heart J. 2026 Mar 5;47(9):1050-1062. doi: 10.1093/eurheartj/ehaf315. | |
| 38883792 |
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| ID | Term |
|---|---|
| D009220 | Myositis |
| ID | Term |
|---|---|
| D009135 | Muscular Diseases |
| D009140 | Musculoskeletal Diseases |
| D009468 | Neuromuscular Diseases |
| D009422 | Nervous System Diseases |
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biopsy residual samples
Characterisation of an immune system signature, using peripheral blood mononuclear cells and muscular biopsies, DNA and RNA sequencing, and autoantibodies |
| baseline: first 30 days after inclusion |
| Risk factors for All-cause mortality depending on patient's and disease characteristics | Risk factors for All-cause mortality depending on patient's and disease characteristics including clinical, radiological electrophysiological, histo-biological and immunological as well as treatment received stratified by each subgroup of myositis | up to twenty years after inclusion |
| Change of the quality of life, using quality of life questionnaires, depending of patients and disease characteristics | Change of the quality of life, using quality of life questionnaires, depending of patients and disease characteristics (HAQ (Health Assessment Questionnaire)global health status scale (0-100)) | up to twenty years after inclusion |
| Change of activity impairment using an evaluation of daily life activity by both patient and physician using a Visual Analogue Scale depending of patients and disease characteristics | Using analogue Scale depending of patients and disease characteristics: Physician's assessment of disease activity in the muscle area (VAS 0-10) Physician's evaluation of the disease activity in the skin area (VAS 0-10) Physician's evaluation of general signs of disease activity (VAS 0-10) Physician's assessment of disease activity in rheumatology (VAS 0-10) Physician's assessment of disease activity in the digestive area (VAS 0-10) Physician's assessment of disease activity in the pulmonary area (VAS 0-10) Physician's assessment of disease activity in the cardiac area (VAS 0-10) Doctor's assessment of disease activity in the extra-muscular area (VAS 0-10) Overall evaluation of the disease activity (muscular and extra-muscular) by the PHYSICIAN (VAS 0-10) Global evaluation of the disease activity by the PATIENT (VAS 0-10) | up to twenty years after inclusion |
| Characterisation of a quality-of-life scale using biological data (CPK), muscle weakness (muscle testing) and other visceral involvements | Using MM8 testing (0-150) visceral involvements | up to twenty years after inclusion |
| Characterisation of a global activity scale using biological data (CPK), muscle weakness (muscle testing) and other visceral involvements | Using MM8 testing (0-150) | up to twenty years after inclusion |
| Incidence of major cardio-vascular events | Incidence of major cardio-vascular events | up to twenty years after inclusion |
| Consequences on outcomes of major cardio-vascular events | Major cardiovascular will include:
| up to twenty years after inclusion |
| Correlation of myositis with the development of extra-muscular diseases including but not limited to dermatological, rheumatological, cardiological and pneumological associated diseases | Correlation of myositis with the development of extra-muscular diseases including but not limited to dermatological, rheumatological, cardiological and pneumological associated diseases | up to twenty years after inclusion |
| Characterisation of respiratory function with pulmonary function test and thoracic tomodensitometry | Using spirometry (Vital capacity in the sitting position) | up to twenty years after inclusion |
| Characterisation of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Vital capacity in supine position) | up to twenty years after inclusion |
| Characterisation of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (FEV1/VC ratio) | up to twenty years after inclusion |
| Characterisation of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Total lung capacity by Plethysmographic) | up to twenty years after inclusion |
| Characterisation of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Inspiratory capacity) | up to twenty years after inclusion |
| Characterisation of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (maximum static inspiratory pressure as % of predicted value) | up to twenty years after inclusion |
| Characterisation of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Sniff nasal inspiratory pressure in % of predicted value) | up to twenty years after inclusion |
| Characterisation of diaphragmatic failure with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Vital capacity in the sitting position ) | up to twenty years after inclusion |
| Characterisation of diaphragmatic failure with pulmonary function test and thoracic tomodensitometry | Using Spirometry ( Vital capacity in supine position ) | up to twenty years after inclusion |
| Characterisation of diaphragmatic failure with pulmonary function test and thoracic tomodensitometry | Using Spirometry (FEV1/VC ratio) | up to twenty years after inclusion |
| Characterisation of diaphragmatic failure with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Total lung capacity by Plethysmographic) | up to twenty years after inclusion |
| Characterisation of diaphragmatic failure with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Inspiratory capacity) | up to twenty years after inclusion |
| Characterisation of diaphragmatic failure with pulmonary function test and thoracic tomodensitometry | Using Spirometry (maximum static inspiratory pressure as % of predicted value) | up to twenty years after inclusion |
| Characterisation of diaphragmatic failure with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Sniff nasal inspiratory pressure in % of predicted value) | up to twenty years after inclusion |
| Follow up of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Vital capacity in supine position) | up to twenty years after inclusion |
| Follow up of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Vital capacity in the sitting position) | up to twenty years after inclusion |
| Follow up of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (FEV1/VC ratio) | up to twenty years after inclusion |
| Follow up of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Total lung capacity by Plethysmographic) | up to twenty years after inclusion |
| Follow up of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Inspiratory capacity) | up to twenty years after inclusion |
| Follow up of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (maximum static inspiratory pressure as % of predicted value) | up to twenty years after inclusion |
| Follow up of respiratory function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Sniff nasal inspiratory pressure in % of predicted value) | up to twenty years after inclusion |
| Follow up of diaphragmatic function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Sniff nasal inspiratory pressure in % of predicted value) | up to twenty years after inclusion |
| Follow up of diaphragmatic function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (maximum static inspiratory pressure as % of predicted value) | up to twenty years after inclusion |
| Follow up of diaphragmatic function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Inspiratory capacity) | up to twenty years after inclusion |
| Follow up of diaphragmatic function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Total lung capacity by Plethysmographic) | up to twenty years after inclusion |
| Follow up of diaphragmatic function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (FEV1/VC ratio) | up to twenty years after inclusion |
| Follow up of diaphragmatic function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Vital capacity in the sitting position) | up to twenty years after inclusion |
| Follow up of diaphragmatic function with pulmonary function test and thoracic tomodensitometry | Using Spirometry (Vital capacity in supine position) | up to twenty years after inclusion |
| Département de pharmacologie clinique, Hôpital Pitié Salpêtrière | Recruiting | Paris | 75013 | France |
|
| Derived |
| Power JR, Dolladille C, Ozbay B, Procureur AM, Ederhy S, Palaskas NL, Lehmann LH, Cautela J, Courand PY, Hayek SS, Zhu H, Zaha VG, Cheng RK, Alexandre J, Roubille F, Baldassarre LA, Chen YC, Baik AH, Laufer-Perl M, Tamura Y, Asnani A, Francis S, Gaughan EM, Rainer PP, Bailly G, Flint D, Arangalage D, Cariou E, Florido R, Narezkina A, Liu Y, Sandhu S, Leong D, Issa N, Piriou N, Heinzerling L, Peretto G, Crusz SM, Akhter N, Levenson JE, Turker I, Eslami A, Fenioux C, Moliner P, Obeid M, Chan WT, Ewer SM, Kassaian SE; International ICI-Myocarditis Registry; Johnson DB, Nohria A, Zadok OIB, Moslehi JJ, Salem JE. Predictors and Risk Score for Immune Checkpoint-Inhibitor-Associated Myocarditis Severity. medRxiv [Preprint]. 2024 Jun 3:2024.06.02.24308336. doi: 10.1101/2024.06.02.24308336. |