Idiopathic inflammatory myopathies.

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Mind Map on Idiopathic inflammatory myopathies., created by narjes ali on 10/08/2016.
narjes ali
Mind Map by narjes ali, updated more than 1 year ago
narjes ali
Created by narjes ali over 9 years ago
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Resource summary

Idiopathic inflammatory myopathies.
  1. Polymyositis
    1. Symmetric proximal muscle weakness
      1. Elevated serum muscle enzymes* CK, CK-MB, AST, ALT, LD, Aldolase
        1. Myopathic changes on EMG
          1. Muscle biopsy
            1. -cellular infiltrate is predominantly within the fascicle with inflammatory cells invading individual muscle fibers
              1. cell–mediated, increased numbers of cytotoxic CD8+ T cells, which appear to recognize an antigen on the muscle fiber surface
              2. treatment
                1. PM: 50% were refractory to steroids
              3. Dermatomyositis
                1. Symmetric proximal muscle weakness
                  1. Elevated serum muscle enzymes
                    1. Myopathic changes on EMG
                      1. Muscle biopsy
                        1. humorally–mediated disorder (CD4 cells), cellular infiltrate, located principally in perifascicular regions, focused around blood vessels
                        2. Rash
                          1. skin findings
                            1. Gottron’s sign. Heliotrope rash. Shaw sign & V sign. Mechanic’s hands. Psoriasiform changes in scalp
                            2. treatment
                              1. DM, 87% responded to steroids but 92% flared when therapy was tapered
                            3. Inclusion body myositis
                              1. insidious onset
                                1. more prominent distal muscle weakness & atrophy (wrists, fingers, anterior tibial)
                                  1. Asymmetric muscle involvement
                                    1. On average, serum muscle enzyme levels are lower in IBM than in PM
                                      1. presence of typical inclusion bodies on muscle biopsy
                                      2. Overlap Myositis
                                        1. Myopathy associated with the other connective tissue diseases scleroderma, systemic lupus erythematosus, mixed connective tissue disease
                                          1. varies from clinically insignificant to typically severe PM or DM in which myopathy dominates the clinical picture
                                            1. anti-Ro, anti-La, anti-Sm, or anti-ribonucleoprotein (RNP) antibodies.
                                              1. treatment
                                                1. 89 to 100% responded to steroids, depended on specific antibodies.
                                              2. Work-up
                                                1. Serology: ESR, CK, CK-MB, AST, ALT, adolase, ANA, anti-Jo1, anti-SRP, anti-Sm, anti-Ro
                                                  1. Utox, HIV
                                                    1. Muscle biopsy
                                                      1. +/- EMGs
                                                        1. Age appropriate cancer screening for those with dermatomyositis
                                                        2. Treatment
                                                          1. Steroids, PT/rehab
                                                            1. Minimal data of long-term follow-up of pts
                                                              1. Methotrexate or Azathioprine
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