Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin

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

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.

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

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.

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]

Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin

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Idiopathic Inflammator Myopathie Dr.Mark Boulos June5 07fin