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Inflammatory My Opa Thies

Inflammatory My Opa Thies

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Published by sridhar
NEUROLOGY MYOPATHIES AND ITS CLASSIFICATION
NEUROLOGY MYOPATHIES AND ITS CLASSIFICATION

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Published by: sridhar on Mar 14, 2013
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07/12/2013

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The Physician's Guide to Laboratory Test Selection and Interpretation
ARUP LABORATORIES | 500 Chipeta Way | Salt Lake City, Utah 84108-1221 | (800) 522-2787 |
www.arupconsult.com
| www.aruplab.com
 © 2006–2013 ARUP Laboratories. All Rights Reserved.Inflammatory Myopathies - p. 1 of 10
Inflammatory Myopathies
Diagnosis
Indications for Testing
Symmetrical proximal muscle weakness
Criteria for Diagnosis
Bohan and Peter criteria for diagnosis of PM and DMBohan and Peter Criteria for Diagnosis of PM and DMProximal muscle weakness, usually symmetricalElevated serum muscle enzymes (CK, aldolase)Electromyographic abnormalitiesCommon – myopathic potential (low amplitude, short duration and polyphasic action potentials)Characteristic triad – myopathic potentials, fibrillations, positive sharp waves, increased insertionalactivity, complex repetitive dischargesMuscle biopsy findings typical of PM or DM – necrosis, phagocytosis, regeneration, inflammationDermatological features of DM, Gottron’s sign or papules, or heliotrope rash
Definite diagnosis requires four criteria with rash for DM and without rash for PMProbable diagnosis requires three criteria with rash for DM and without rash for PM
Laboratory Testing
Initial screening testsCreatine kinase (CK) – elevated, but consider other causes for elevations >100-foldESR or CRP – elevated during active phases of diseaseCalcium – should be normalTSH – rule out thyroid diseaseAntibody testingANA – positive in 50-80% of patientsAnti-histidyl-tRNA synthetase (Jo-1) testingFound in 18-36% of patients satisfying myositis criteria – not useful in confirming diagnosis; morecommon in PMNearly all Jo-1-positive patients have lung involvement (antisynthetase syndrome) and moderateto severe diseaseRelationship between Jo-1 antibody titer and disease activity reported but not confirmedJo-1 antibodies rarely found in any other disease statesOverlap syndromesOther myositis-specific antibodiesAnti-synthetase antibodies – predictive of lung involvement, not usually found in IBMIn addition to Jo-1, less common markers include the followingAnti-PL7 (threonyl-tRNA synthetase)Anti-PL-12 antibodies (anti-alanyl-tRNA synthetase)Anti-EJ (glycyl-ts RNA)Anti-OJ (anti-isoleucyl-tRNA synthetase)Anti-KS (asparaginyl tRNA synthetase)Anti-HA (tyrosyl tRNA synthetase)Anti-Zo (phenylalanyl tRNA synthetase)Non-synthetase antibodies
 
The Physician's Guide to Laboratory Test Selection and Interpretation
ARUP LABORATORIES | 500 Chipeta Way | Salt Lake City, Utah 84108-1221 | (800) 522-2787 |
www.arupconsult.com
| www.aruplab.com
 © 2006–2013 ARUP Laboratories. All Rights Reserved.Inflammatory Myopathies - p. 2 of 10
Anti-signal recognition particle (anti-SRP) – myocardial involvement frequent; severe, rapidlyprogressive myositis with high CKAnti-p155/140 – may be found in juvenile DMOther myositis-associated autoantibodies rare in juvenile diseaseAnti-HMGCRAnti-MDA5Anti-Mi-2Anti-140Anti-SA5Anti-MJ (NXP-2)Myositis-associated antibodiesAnti-PM-Scl –scleroderma-polymyositisAnti-U1 RNP/anti-U3 RNPAnti-KuAnti-pLAAnti-topoAnti-RO (SSA)Anti-5bKDaAnti-hPMS1Nonspecific testsAldolase, AST, ALT, LD, serum myoglobin – variably elevated
Histology
Muscle biopsy is gold standard for diagnosisUsually performed on proximal muscles of the leg but not in end-stage muscles – MRI may be helpfulin choosing muscleOpen biopsy preferred – larger sampleDMDemonstrate perivascular and perimysial inflammation (B-cells, macrophages and CD4+ cells)T-cells usually absentDistinct muscle pathologyPMDemonstrates CD8+ T-cell invasion of non-necrotic muscle; uniform expression of MHC-1 at surfaceof muscleIBMRimmed vacuoles with amyloid and filamentous masses; cytochrome oxidase negative fibersUniform expression of MHC-1 at surface of muscleImmunohistochemistry – SM1-31 antibody best detected antibody but not specific for IBM
Other Testing
EMG – changes consistent with myopathy, including increased spontaneous and insertional activity withfibrillation potential, complex repetitive discharges, positive repetitive discharges, positive sharp waves,early recruitment and small polyphasic motor unit potentialsAbnormal in 70-90% of patientsNot specific for inflammatory myopathiesAmyopathic DM may have only subtle myopathy on EMG
Imaging Studies
 
The Physician's Guide to Laboratory Test Selection and Interpretation
ARUP LABORATORIES | 500 Chipeta Way | Salt Lake City, Utah 84108-1221 | (800) 522-2787 |
www.arupconsult.com
| www.aruplab.com
 © 2006–2013 ARUP Laboratories. All Rights Reserved.Inflammatory Myopathies - p. 3 of 10
Ultrasound – muscle edema with alteration of normal architectureMay visualize subcutaneous calcificationsCT – fatty infiltration suggests chronic diseaseMRI – very sensitive for detection of muscle edema
Differential Diagnosis
Weakness aloneEndocrine myopathiesThyroid disease(hyper- and hypo-)Cushing syndromeDrug-related myopathiesStatinsSteroidsDiureticsColchicineHydroxychloroquine AlcoholGrowth hormoneAmyloidosisSarcoidosisNeuropathiesPolymyalgia rheumaticaMetabolic myopathiesLipid storage disordersMitochondrial diseasesNeuromuscular transmission disordersMyasthenia gravisSpinal stenosisParaneoplastic syndromesEaton-Lambert syndromeMuscular dystrophiesLimb-girdle dystrophyFacioscapulohumeral dystrophyDuchenne muscular dystrophyMotor neuron disordersSpinal muscular atrophyAmyotrophic lateral sclerosis – Lou Gehrig diseaseVasculitis/polyarteritis nodosa Weakness (with or without rash)ViralEnterovirus(eg, polio)InfluenzaParvovirus B19Human T-lymphotropic virus type 1Hepatitis B virus– HBVBacterial/parasiticToxoplasmosis

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