Myosites du sujet âgé
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1 Myosites du sujet âgé Olivier Benveniste Département de Médecine Interne Centre de Référence Maladies Neuro-Musculaires Equipe Muscle Inflammatoire U974 Groupe Hospitalier Pitié-Salpêtrière
2 Myositis classification Most of the literature is based on this paper:
3 Peter and Bohan criteria for PM or DM 1. Symmetrical proximal muscle weakness; 2. Muscle biopsy abnormalities : 1. Muscle fiber destruction; 2. Muscle fiber regeneration; 3. Perivascular and interstitial inflammatory infiltrates with muscle fiber destruction. 3. Elevation of CPK, Transaminases, LDH or aldolase activity; 4. Electromyography changes: 1. Fibrillation potentials (on needle insertion at rest); 2. Complex repetitive discharges (on needle insertion at rest); 3. Positive sharp waves (on needle insertion at rest); 4. Short duration, low amplitude complex (polyphasic) potentials on contraction. 5. Typical skin rash. DEFINITE: any 4 of the criteria PROBABLE: any 3 of the criteria
4 Example, 84 years old patient 75 y, proximal deficit, difficulties to climb stairs 79 y, proximal and distal muscle weakness (anterior tibialis, quadriceps, finger and wrist flexors, ankle dorsiflexors) 82 y, use a stick Swallowing tbs
5 CK: 450 U/l EMG: myogenic syndrome Muscular biopsy Old patient 2 Diagnosis : polymyositis. (definite with Peter and Bohan criteria) Treatment : 2 years 1) Corticosteroids 2) IVIg No success 2 nd biopsy
6 sibm features
7 Content of protein inclusions Many proteins related to neurodegenerative diseases: Amyloid deposits b-amyloid and bapp IBM phosphorylated tau a1act a-synuclein prion protein ApoE ab-crystallin Parkin copper zinc superoxide dismutase manganese superoxide dismutase apoptotic regulators (Bcl-2, Bcl-x and BAX) Lipoprotein receptors Ubiquitins AD Werner Stenzel
8 Unanswered question Is IBM a degenerative or an inflammatory myopathy? Treg CD28- ICOS? Plasma cells IFN-g Dalakas M, Nat Clin Pract Neurol. 2006
9 sibm clinical features
10
11 Evaluation 9 months Visit 1 Visit 2 4 years Visit 3
12 Baseline visit: strength measurements % predicted Upper limb Grip * left Wrist flexion * right Wrist extension * Elbow flexion * Elbow extension Lower limb Ankle flexion * Ankle extension Knee flexion * Knee extension
13 Relation between knee extension strength and 6MWD 6MWD (% predicted normal) Knee extension strength (% predicted normal)
14 Neurology 2014 Finger flexor or quadriceps weakness, and Endomysial inflammation, and Invasion of nonnecrotic muscle fibres or rimmed vacuoles 90% sensitivity and 96% specificity
15
16 Characteristics of 136 patients Variable Result Gender : male (n=136) 78 (57.3%) Age at first symptoms, years (n=136) 61 [55-69] First symptoms (n=136) Muscle weakness only Swallowing troubles only Muscle weakness and swallowing troubles Previous diagnosis (n=136) None Polymyositis Amyotrophic Lateral Sclerosis Dystrophy Other Delay between first symptoms and diagnosis, months (n=136) 119 (87.5%) 6 (4.4%) 11 (8.1%) 94 (69.1%) 23 (16.9%) 3 (2.2%) 4 (2.9%) 12 (8.8%) 59 [29-95]
17 Survival from first signs 81 years
18 Description of received treatments by 71 (52%) sibm patients Molecules and duration Results Corticosteroids (prednisone, 1 mg/kg/day) 65 (92%) (n=63) Intravenous Immunoglobulins 40 (56%) (n=39) Azathioprine 19 (27%) (n=17) Methotrexate 23 (32%) (n=21) Combination of treatment Corticosteroids only Corticosteroids and other drugs Other drugs only Duration of treatment, months [n=69] 19 (27%) 46 (65%) 6 (8%) 41 [ ] ~ 3.5 years
19 Comparison of treated and untreated sibm patients Status at the last visit Untreated (n=65) Treated (n=71) p CK, u/ml (n=87) 367 [ ] 209 [ ] 0.11 Grip test (n=76) 13.4 [ ] 13.5 [ ] 0.84 Walton (n=113) 4 [3-6] 6 [3-6] RMI (n=88) 11 [9-13] 10 [4-11] IWCI (n=71) 50 [30-65] 40 [25-50] 0.04 Current handicap for walking (n=136) None 1 or 2 canes Wheelchair 20 (31%) 26 (40%) 19 (29%) 13 (18%) 26 (37%) 32 (45%) 0.10
20 Estimates of covariate effect on each transition in the multi-state model Transition HR (95%CI) p Age at first symptoms (> 60 yrs vs <60 yrs) No handicap walking with aid No handicap wheelchair Walking with aid wheelchair Alive - Death 1.98 ( ) 0.62 ( ) 1.35 ( ) 3.65 ( ) Treatment (Yes vs No) No handicap walking with aid No handicap wheelchair Walking with aid wheelchair Alive - Death 2.05 ( ) 2.09 ( ) 1.74 ( ) 1.47 ( )
21 Discussion / conclusions Onset Diagnosis = Walk support Wheelchair Death 5 y. 9 y. 61 y. 66 y. 75 y. 80 y. IS treatments don t seem to ameliorate the handicap - Are treated patients more severely affected? - Are treatments deleterious? IS treatments don t seem to delay the dates of wheelchair or death
22 IBM: 6 controlled prospective studies Authors N Intervention Efficacy Dalakas, IVIg or placebo No, 3 mo Walter, IVIg or placebo No, 6 mo Dalakas, CS + IVIg or placebo No, 3 mo Muscle study Group, 2001 and Beta IFN or placebo No, 6 mo Rutkove, 2002 Badsrising, Oxandrolone or placebo MTX or placebo No, 3 mo No, 12 mo
23 Treatment of IBM, in practice : Physiotherapy +++ If «young» patient, rapid evolution, many inflammatory infiltrates on biopsy Prednisone (1 mg/kg/d) MTX For a limited duration (3 to 6 months) If swallowing troubles IVIg Cricoid myotomy
24
25 sibm: Th1 Signature and Treg deficiency % of positive cells Controls sibm sibm FoxP3 CD4 Dapi Y Allenbach, PLoS One 2014
26 B cells T cells CD4+ CD8+ Cell count % of Tregs Effect of Rapamycin in our mouse model PBS 1mg 3mg PBS 1mg 3mg 2.5E7 1.5E7 PBS Rapa 3mg 0.5E7 PBS Rapa 3mg N Prevel: PLoS One 2014
27
28 Translational Medicine Grant DGOS-Inserm Inclusion criteria: 45 y.o. Defined IBM Exclusion Criteria: Allergy to rapamycin Intolerance to rapamycin Loss of walking capability Rapamycin or placebo 0,3 mg/kg For 12 months n=44 Clinical Evaluation of Muscle Strength MRC scale RMI scale Walton scale IWCI IBMFRS Clinical Research Evaluation Myometric study of quads Whole body IRM TOLERANCE Clinical evaluation Biological evaluation Mode D action Facs Dosage of cytokines Treg Pre-study observation D0 D10 M1 M2 M3 M6 M9 M12 MRI MRI
29 ClinicalTrials.gov Efficacy and Safety of Bimagrumab/BYM338 at 52 Weeks on Physical Function, Muscle Strength, Mobility in sibm Patients (RESILIENT) Phase 2, 3: 240 patients Sponsor: Novartis Pharmaceuticals ClinicalTrials.gov Identifier: NCT
30 New classification of myositides Dermatomyositis, 30% paraneoplastic Inclusion body myositis Polymyositis Overlap myositis (Troyanov) Myositis associated to a connective tissue disease Myositis with associated Abs (PmScl, Ku ) Myositis with specific Abs (anti-synthetases, anti-srp ) Immune mediated necrotizing myopathies (Hoogendijk) with anti-srp+, anti-hmgcoa Reductase+ (poststatines), or paraneoplastic
31 Polymyositis Overlap Myositis Ku SAE ASA YRS OJ KS Zo Jo-1 PL-12 EJ PL-7 U1-RNP PM-Scl ILD MDA-5 Mi-2 Cancer NXP2 TIF-1g Cancer Dermatomyositis 5 -Nucleotidase Inclusion Body Myopathy HMGCR SRP Immune Mediated Necrotizing Myopathy
32 Arthritis, Sept patients 38 necrotizing myopathies - 4 anti-synthetase - 6 anti-srp - 16 anti-p200/100
33
34 En ELISA HMGCoAR : 100% + patients anti-p200/100 Specificity : Immunoprecipitation W blot
35 N = 1966 with 763 now under statines, 322 had statines, 881 no statines
36 Medicine 2014
37 Characteristics of the patients First signs: - Muscle weakness: 87% - Isolated increase of CK: 13% (n=5) Paris n=45 Baltimore n=50 Age (year) 44 ± ± 16 Patients < Women 81% 62% Statines + 44% 72.7%
38 Myalgia: 75% Muscle weakness: 87% (Paris) vs 95% (Baltimore) Bilateral, proximal, severe 3/5: 72% Rapidly progressive in < 6 mo: 47% Axial: 61% Bedridden: 10% No facial weakness Dysphagia: 41% Amyotrophia: 15% Scapula wings: 1 patient Muscle involvement
39 Acknowledgements Werner Stenzel, Berlin, D David Hilton-Jones, Oxford, UK Baziel GM van Engelen, Nijmegen, NL Olivier Boyer, Rouen, F and my colleagues from: Bruno Eymard Pascal Laforêt Baptiste Hervier Yves Allenbach
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