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42 yoWF with hx of hypothyroidism, depression has a 2 week hx myalgia, weakness and a CPK of 3000. Adm to PHD CPK of 13379 and AST 225, ALT 107, LDH 1380 and CRP 2.6 mg/dl. EMG was classic for DM. She was d/c on Prednisone 80 mg qd but worsened w/ difficulty with stairs, and combing her hair, proximal weakness and muscle pain. The patient also complains of subjective fever, fatigue, myalgia, indigestion, and depression. CPK worsened at home to 11000 and was re-admitted. Rx with steroids, IVIG and MTX 21 yoBF with SLE x 2 yrs Rx with Plaquenil and Imuran. She developed progresive weakness in the arms over 2-3mos and was hospitalized with 2 pillow orthopnea and some RUQ pain and CPK of 12,112 and a Aldoase of 79. Started on prednisone 80 mg/day and Arava 100/wk and discharged with CPK 2000. She complains of difficulty with stairs, and combing her hair, and proximal weakness. She denies shortness of breath, dysphagia, and skin rash. Repeat CPK up to 5000. Steroid increased and pt put on MTX 56 yoWF wih RA since 2003, On Plaquenil, MTX, failed Enbrel. 2/04, she reports progressive worsening of weakness to arms and legs. She has difficulty with stairs getting off the toilet, chairs, upper pharyngeal dysphagia, mainly to pills and water, food. Also c/o Weight loss of 15 lbs, SOB cough, dry eyes/moutn. found to have m CPK elevation 458, mild LFTs elevation, with normal aldolase. EMG mixed myopathic/neuropathic. Bx showed autophagic vacuoloar myopathy Antimalarial Neuromyopathy Not dose or duration dependant Insidious painless LE/UE weakness Clinical myopathy 6.7%; Chemical myopathy 18.8% Bx: classic vacuolar myopathy and EM curvilinear bodies/complex lysosomes Rx: improvement within 2 mos of d/c Casado E. Ann Rheum Disease 2005 Polymyositis Dermatomyositis F:M = 2.5:1 Acute onset; all ages (bimodal) Incidence 2-7/million/year Weakness (+ myalgia): Proximal > Distal Skeletal muscle: dysphagia, dysphonia Sx: Rash, Raynauds, dyspnea 65% elevated CPK, aldolase 50% ANA (+) 90% +EMG; 85% + muscle biopsy Proposed Criteria for Myositis 1. 2. 3. 4. 5. Symmetric proximal muscle weakness Elevated Muscle Enzymes (CPK, aldolase, AST, ALT, LDH) Myopathic EMG abnormalities Typical changes on muscle biopsy Typical rash of dermatomyositis PM Dx is Definite w/ 4/5 criteria and Probable w/3/5 criteria DM Dx Definite w/ rash and 3/4 criteria and Probable w/ rash and 2/4 criteria Polymyositis Classification Bohan & Peter 1. 2. 3. 4. Primary idiopathic dermatomyositis Primary idiopathic polymyositis Adult PM/DM associated with neoplasia Childhood Dermatomyositis (or PM) often associated with vasculitis 5. Myositis associated with collagen vascular disease MYOPATHY: HISTORICAL CONSIDERATIONS Age/Sex/Race Acute vs. Insidious Onset Distribution: Proximal vs. Distal Pain? Drugs/Pre-existing Conditions Neuropathy Systemic Features MYOPATHIES Toxic/Drugs Infectious Muscular dystrophies, hereditary myopathies Neuropathic/Motor Neuron Disorders Coxackie A9, HBV, HIV, Stept., Staph, Clostridial, Toxoplasma, Trichinella Congenital neuromuscular disorders Etoh, Cocaine, Steroids, Plaquenil, Penicilamine, Colchicine, AZT, Lovastatin, Clofibrate, Tryptophan Myasthenia gravis, amyotrophic lateral sclerosis Endocrine/Metabolic Glycogen storage diseases, mitochondrial Drug-induced myopathy Amiodarone Amphetamines Chloroquine Cimetadine Cocaine Colchicine Corticosteroids Cyclosporin Danazol Emetine Ethanol Fibric-acid Rx Heroin Hydralzaine Hydroxychloroqui ne Hydroxyurea Levodopa Nicotinic acid Pancuronium Penicillamine Pentazocine Phenylbutazone Phenytoin Procainamide Rifampin Statins Sulfonamides Tiopronin Vecruonium Vincristine Zidovudine NONMYOPATHIC CONSIDERATIONS Fibromyalgia Polymyalgia Rheumatica Caucasians, > 55 yrs, M=F, ESR > 100, normal strength, no synovitis RA SLE Adult Still's Disease Vasculitis INCLUSION BODY MYOSITIS Bimodal age distribution, maybe hereditary Slow onset, progressive weakness Painless, distal and proximal weakness Normal or mildly elevated CPK Poor response to corticosteroids Dx: light microscopy may be normal or show CD8+ lymphs. Tubulofilamentous inclusion bodies on electron microscopy Role for amyloid? Presentation of PM/DM Presentation Painless proximal weakness (over 3-6 mos) Acute/subacute proximal pain and weakness (wks-2 mos) Insidious proximal/distal weakness (< 10 yrs) Proximal myalgia alone Dermatomyositis sine myositis Frequency (%) 55% 30% 10% 5% <1% Skeletal Muscle Weakness Painless; proximal > distal Upper Extremity: combing hair, dressing Lower: Stairs, toilet, car, falling, gait Dysphagia, hoarseness, regurgitation Inability to raise head from the pillow DERMATOMYOSITIS 5 Skin Features 1. Heliotrope Rash: over eyelids 2. 3. 4. 5. Seldom seen in adults Gottrons Papules (60-80%): MCPs, PIPs, MTPs, knees, elbows V-Neck Rash (Shawl sign): violaceous erythema ant. chest w/ telangiectasias Periungual erythema, digital ulcerations Calcinosis Mechanics Hands Calcinosis DIAGNOSTIC TESTING Physical Examiniation: Motor Strength (Gowers sign), Neurologic Exam Acute phase reactants unreliable Muscle Enzymes CPK: elevated >65%; >10% MB fraction is possible Muscle specific- Aldolase, Troponin, Carb. anhydraseIII AST > LDH > ALT Beware of incr. creatinine (ATN) and myoglobinuria Electromyogram: increased insertional activity, low amplitude, polyphasics, positive sharp waves Beware of neuropathic changes, incremental/decremental MU changes DIAGNOSTIC TESTING Muscle Biopsy (an URGENT not elective procedure) Call the neuropathologist! 85% Sensitive. Biopsy involved muscle (MRI guided) Avoid EMG/injection sites or sites of trauma Magnetic Resonance Imaging - detects incr. water signal, fibrous tissue, infiltration, calcification Investigational: Tc-99m Scans, PET Scans Serologic Tests: ANA (+) 60%, Abs against t-RNA synthetases INFLAMMATORY MYOSITIS Biopsy Findings Inflammatory cells Edema and/or fibrosis Atrophy/ necrosis/ degeneration Centralization of nuclei Variation in muscle fiber size Rarely, calcification Anti-synthetase syndrome: ILD, fever, arthritis, Raynauds, Mechanics hands PM/DM Complications PULMONARY Intercostal, diagphragm involvement Aspiration pneumonitis Infectious pneumonitis Drug induced pneumonitis Fibrosing alveolitis RARE: Pulmonary vasculitis Pulmonary neoplasia Pulmonary hypertension CARDIAC Elev. CPK-MB Mitral Valve prolapse AV conduction disturbances Cardiomyopathy Myocarditis MALIGNANCY & MYOSITIS Controversial Reports range from 10-25% If real, men over age 50 yrs at greatest risk Common tumors: Breast, lung, ovary, stomach, uterus, colon 60% the myositis appears 1st, 30% neoplasm 1st, and 10% contemporaneously Avoid invasive, expensive searches for occult neoplasia RHABDOMYOLYSIS Injury to the sarcolemma of skeletal muscle with systemic release of muscle macromolecules such as CPK, aldolase, actin, myoglobin, etc Maybe LIFE-THREATENING: from hyperkalemia, met. acidosis, ATN from myoglobinuria Common causes: EtOH, Cocaine, K+ deficiency, infection, PM/DM, infection (clostridial, staph, strept), exertion/exercise, cytokines PM/DM Diagnosis Symmetric progressive proximal weakness Elevated muscle enzymes (CPK, LFTs) Muscle biopsy evidence of myositis EMG: inflammatory myositis Characteristic dermatologic findings INFLAMMATORY MYOSITIS Treatment Early Dx, physical therapy, respiratory Rx Corticosteroids : 60-80 mg/day 80% respond within 12 weeks Steroid resistant Methotrexate Azathioprine IVIG, Cyclosporin, Chlorambucil: unproven No response to apheresis PROGNOSIS Poor in pts. with delayed Dx, low CPK, early lung or cardiac findings, malignancy Neoplasia in 10% of adults PT for muscle atrophy, contractures, disability Kids:50% remission, 35% chr active disease Adult < 20 yrs. do better than >55 yrs. Adults: Mortality rates betw. 28-47% @ 7 yrs. Relapses & functional disability are common Death: due to malignancy, sepsis, pulm. or cardiac failure, and complications of therapy Inflammatory Myositis Polymyositis (PM) and dermatomyositis (DM) are types of idiopathic inflammatory myopathy (IIM). IIM are characterized clinically by proximal muscle weakness Etiology: There is now known etiology. Demographics: PM is more common than DM in adults. Peak incidence occurs between 40 and 60 yrs. F:M 2:1 Muscles: Proximal muscle weakness, dysphagia, aspiration. respiratory failure or death. Skin: Gottron's papules, heliotrope rash, "V" neck rash, periungual erythema, "Mechanic's hands", calcinosis Dx: Muscle enzymes (CPK, aldolase), EMG, Biopsy Rx: Steroids, MTX, Azathioprine, IVIG