Polymyositis Inflammatory myopathy T cell attack on individual muscle fibers Assoc.

oc. w/ autoimmune dz, Cardiomyopathy Females > Males Onset around 35 y/o

Dermatomyositis Inflammatory myopathy Autoimmune mediated attack on capillaries B Cell mediated Childhood type assoc. post virus Assoc. risk of malignancy AdenoCA of cervix, lung, ovarian, bladder, stomach CA

S/S:

Prox > distal weakness Symmetric Bulbar Myalgia (33%) Cardiac Interstitial lung dz, arthritis, Raynauds, thick skin (assoc. w/ anti-Jo-1)

Dx: EMG w/ fibs and small, polyphasic MUAP CK Antimyosin Ab Jo-1 Ab (20-25%) Bx showing endomysial and perivascular monocyte inflammation Tx: Steroids Immunosuppression IVIG PLEX Prog: Variable, most improve w/ 4-6 wks of tx

S/S: Rash (malar, eyelids, extensors, chest) Shawl sign (shoulder rash) Heliotrope rash in sun exposed region Gottrons papules-extensor joints (pink knuckles) Periorbital edema Cutaneous calcinosis Prox > Distal weakness Myalgias DTRs nl Dysphagia (1/3) Priapism Interstitial Lung Dz, cardiac dz Contractures Dx: CK EMG w/ fibs and small, polyphasic MUAP Bx w/ perifascicular atrophy and sparing of central fascicle Mi-2 Ab (assoc. w/ ovarian and other CA) Mi-2 Ab neg more likely vasculitis assoc. w/ +Jo-1 +ANA Tx: Steroids Immunosuppression IVIG Hydroxychlorquine for skin lesions

Inclusion body myositis Inflammatory myopathy T-cell mediated attack Men, >50 y/o Assoc. HLA-DR 20% assoc. w/ monoclonal gammopathy Slowly progressive Variants IBM2 (Nonaka) (Adult-onset distal) Hereditary , AR, Ch 9p IBM3 Missense mutation- myosin IBM w/dementia & Pagets of bone AD, Chr. 9p Diffuse weak, CN abn, Pagets dz, FTLD, cardiomyopathy S/S: Prox = distal weakness LE > UE Asymmetric No myalgia Dysphagia Finger flexors & quads Spares face, deltoid, FDI Muscle atrophy

Med induced Statins Colchicine Zidovudine (AZT) Fibric acids (gemfibrozil)

Dx: CK elevated(not as high) Bx: endomysial inflammation Ubiquitin inclusion bodies NCS w/ subclinical sensorimotor neuropathy in 10-30% EMG may be classic w/ small, polyphasic MUAP or large (chronic reinnervation) MUAPs & small, complex (myopathic type) MUAPs in same muscle Tx: No good therapy Steroids/ immunesuppression no known benefit + potential s/e