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Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin

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Idiopathic Inflammatory Myopathies Dr. Mark I. Boulos PGY 1 – Neurology Rheumatology Rounds Tuesday, June 5, 2007
Objectives ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Case ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
A few months later… ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
What’s going on? ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Answer: E. Myopathy ,[object Object]
Lower vs. Upper Motor Neuron Weakness *Disuse atrophy can develop after initial presentation Down-going Up-going (Babinski’s sign) Toes Decreased or absent Increased (spasticity) Tone Present Absent Fasciculations Present Absent* Atrophy Diminished or absent Hyperactive +/- clonus Reflexes Lower Motor Neuron (Anterior horn cells to peripheral nerves) Upper Motor Neuron (Brain to corticospinal tract)
Distinguishing Lower Motor Weakness from Muscle Weakness ,[object Object],[object Object],Absent May be present Sensory signs/symptoms Often preserved Diminished Reflexes Absent May be present Fasciculations Proximal > distal Distal > proximal Distribution Due to Myopathy Due to Neuropathy
Source: www.uptodate.com
Common Conditions that can Result in Myopathy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Idiopathic Inflammatory Myopathies ,[object Object],[object Object],[object Object]
Idiopathic Inflammatory Myopathies ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Bohan & Peter (1975). NEJM. Tanimoto et al. (1995). J Rheumatology .
Epidemiology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Wortmann RL. Primer on Rheum Dis . 12th edition. 2001:369–376.
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Polymyositis Wortmann RL. Primer on Rheum Dis . 12th edition. 2001:369–376.
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Polymyositis (continued) ,[object Object],[object Object],[object Object],[object Object],Wortmann RL. Primer on Rheum Dis . 12th edition. 2001:369–376.
[object Object],[object Object],[object Object],[object Object],[object Object],Dermatomyositis Wortmann RL. Primer on Rheum Dis . 12th edition. 2001:369–376.
Gottron’s Sign
Heliotrope rash
Shawl Sign Source: DermAtlas, Johns Hopkins University www.dermatlas.med.jhmi.edu
Inclusion Body Myositis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Wortmann RL. Primer on Rheum Dis . 12th edition. 2001:369–376 .
[object Object],[object Object],[object Object],Juvenile Dermatomyositis Wortmann RL. Primer on Rheum Dis . 12th edition. 2001:369–376.
[object Object],[object Object],[object Object],[object Object],MYOSITIS ASSOCIATED WITH MALIGNANCY Wortmann RL. Primer on Rheum Dis . 12th edition. 2001:369–376.
[object Object],[object Object],MYOSITIS ASSOCIATED WITH OTHER COLLAGEN VASCULAR DISEASES Wortmann RL. Primer on Rheum Dis . 12th edition. 2001:369–376.
Back to our case… ,[object Object]
High clinical suspicion for Polymyositis … ,[object Object],[object Object],[object Object],[object Object]
Polymyositis / Dermatomyositis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Bohan & Peter (1975). NEJM.
[object Object],[object Object],Laboratory Findings Wortmann RL. Primer on Rheum Dis . 12th edition. 2001:369–376.
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],EMG Findings Wortmann RL. Primer on Rheum Dis . 12th edition. 2001:369–376.
Muscle Biopsy: Histology and Immunochemistry ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Source: Dr. R. Shupak, St. Michael ‘s Hospital Pathogenesis of Idiopathic Inflammatory Myopathy Rheumatology Rounds: April 5, 2005 Rolak LA. Neurology Secrets. 2005: 63-7.
Source: Dr. R. Shupak, St. Michael ‘s Hospital Pathogenesis of Idiopathic Inflammatory Myopathy Rheumatology Rounds: April 5, 2005
Back to our case… ,[object Object],[object Object],[object Object]
Differential Diagnosis of the Motor Unit by EMG
Back to our case… ,[object Object]
Myositis-Specific Autoantibodies (MSA) Source: Dr. R. Shupak, St. Michael ‘s Hospital Pathogenesis of Idiopathic Inflammatory Myopathy Rheumatology Rounds: April 5, 2005
Myositis-Specific Autoantibodies Source: Dr. R. Shupak, St. Michael ‘s Hospital Pathogenesis of Idiopathic Inflammatory Myopathy Rheumatology Rounds: April 5, 2005
MSA And Associated Disease Features ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Source: Dr. R. Shupak, St. Michael ‘s Hospital Pathogenesis of Idiopathic Inflammatory Myopathy Rheumatology Rounds: April 5, 2005 Briani et al. (2006). Autoimmunity.
Myositis-Associated Antibodies (MAA) ,[object Object],[object Object],Source: http://www.emedicine.com/neuro/topic85.htm Sjögren syndrome and systemic lupus erythematosus (SLE) Ro/SSA 60 kd Ro/SSA 52 kd La/SSB Connective tissue–disease overlap syndrome snRNP Other connective tissue diseases Anti-Ku Scleroderma Anti-PM/Scl Myositis Overlapping with… MAA
[object Object],Back to our patient…
Immunotherapeutic strategies ,[object Object],[object Object],[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
1) Selective, antigen-specific immunotherapies ,[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
1) Selective, antigen-specific immunotherapies ,[object Object],[object Object],[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
2) Semi-specific therapies ,[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
3) Conventional, non-specific immunosuppressive agents ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
Therapeutic targets in PM, DM, IBM and the available biological agents directed against them ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
Therapeutic targets in PM, DM, IBM and the available biological agents directed against them ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Therapeutic targets in PM, DM, IBM and the available biological agents directed against them Dalakas MC. (2006). Neuromuscular Disorders.
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Therapeutic targets in PM, DM, IBM and the available biological agents directed against them Dalakas MC. (2006). Neuromuscular Disorders.
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Therapeutic targets in PM, DM, IBM and the available biological agents directed against them Dalakas MC. (2006). Neuromuscular Disorders.
[object Object],[object Object],[object Object],[object Object],[object Object],Therapeutic targets in PM, DM, IBM and the available biological agents directed against them Dalakas MC. (2006). Neuromuscular Disorders.
Therapeutic Targets Dalakas MC. (2006). Neuromuscular Disorders.
Treatment Strategy for DM & PM ,[object Object],[object Object],[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
More on IVIg… ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Dalakas MC et al. (1993). NEJM.
This just in… IVIg & IBM ,[object Object],[object Object],Sparks S et al. (2007). BMC Neurology.
Treatment Failure ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
Other Management Considerations ,[object Object],[object Object],[object Object],[object Object]
Back to our case… ,[object Object],[object Object],[object Object],[object Object],[object Object]
One month later … ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Two months later … ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Steroid myopathy versus disease activity ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
Steroid myopathy versus disease activity Examples of two differing scenarios ,[object Object],[object Object],[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
Steroid myopathy versus disease activity ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Dalakas MC. (2006). Neuromuscular Disorders.
How can MRI help my patient?
Use of MRI in Patients with Inflammatory Myopathies ,[object Object],[object Object],[object Object],[object Object],Park JH, Olsen NJ. (2001). Curr Rheumatol Rep.
Use of MRS in Patients with Inflammatory Myopathies ,[object Object],[object Object],[object Object],[object Object],Park JH, Olsen NJ. (2001). Curr Rheumatol Rep.
Advances in MRI ,[object Object],[object Object],[object Object],[object Object],[object Object],Olsen NJ, Qi J, Park JH. (2005). Curr Rheumatol Rep.
Back to our case… ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Happy Ending… ,[object Object],[object Object],[object Object],[object Object],[object Object]
Thanks for your attention! ,[object Object]

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Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin

  • 1. Idiopathic Inflammatory Myopathies Dr. Mark I. Boulos PGY 1 – Neurology Rheumatology Rounds Tuesday, June 5, 2007
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  • 7. Lower vs. Upper Motor Neuron Weakness *Disuse atrophy can develop after initial presentation Down-going Up-going (Babinski’s sign) Toes Decreased or absent Increased (spasticity) Tone Present Absent Fasciculations Present Absent* Atrophy Diminished or absent Hyperactive +/- clonus Reflexes Lower Motor Neuron (Anterior horn cells to peripheral nerves) Upper Motor Neuron (Brain to corticospinal tract)
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  • 19. Shawl Sign Source: DermAtlas, Johns Hopkins University www.dermatlas.med.jhmi.edu
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  • 30. Source: Dr. R. Shupak, St. Michael ‘s Hospital Pathogenesis of Idiopathic Inflammatory Myopathy Rheumatology Rounds: April 5, 2005
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  • 32. Differential Diagnosis of the Motor Unit by EMG
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  • 34. Myositis-Specific Autoantibodies (MSA) Source: Dr. R. Shupak, St. Michael ‘s Hospital Pathogenesis of Idiopathic Inflammatory Myopathy Rheumatology Rounds: April 5, 2005
  • 35. Myositis-Specific Autoantibodies Source: Dr. R. Shupak, St. Michael ‘s Hospital Pathogenesis of Idiopathic Inflammatory Myopathy Rheumatology Rounds: April 5, 2005
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  • 50. Therapeutic Targets Dalakas MC. (2006). Neuromuscular Disorders.
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  • 62. How can MRI help my patient?
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