Managing Myositis. A Practical Guide

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Foreword Myositis Through the Ages Bibliography Preface Contents Contributors 1: Introduction to Myositis Introduction What Is Myositis? Historical Perspective The Classification of Myositis Conclusion References 2: Myositis Basics/Who Gets Myositis Introduction Prevalence and Incidence of Myositis and Its Subtypes Age, Gender, Racial/Ethnic and Geographical Differences Risk of Myositis in Family Members of IIM Patients The Role of Environmental and Genetic Factors in the Development of Myositis Environmental Risks: The Role of Noninfectious Risk Factors Environmental Risks: The Role of Infectious Agents Genetic Risk Factors in Idiopathic Inflammatory Myopathies Conclusion References 3: Evaluating the Patient with Suspected Myositis Introduction History Demographic Considerations Pattern and Progression of Muscle Weakness Extra-Muscular Features of Myositis Social, Family, and Medication Assessment Physical Examination Laboratory Evaluation Imaging Studies Specialized Tests Electromyography and Nerve Conduction Study Muscle Biopsy Conclusion References 4: Asymptomatic HyperCKemia Introduction Etiology of Asymptomatic HyperCKemia Problems with the “Normal” Laboratory CK Physical Activity, Exercise, and CK Levels Non-neuromuscular Etiologies of Asymptomatic HyperCKemia Macro CK Statins Endocrine Disorders Neuromuscular Etiologies of Asymptomatic HyperCKemia Idiopathic HyperCKemia Diagnostic Yield of Muscle Biopsy and EMG/NCS in Asymptomatic HyperCKemia Conclusion: Diagnostic Approach to Asymptomatic HyperCKemia References 5: Clinical Features of Myositis: Muscular Manifestations Introduction Patterns of Muscle Weakness Myalgia Versus Weakness Muscle Examination Dysphagia and Dysphonia Muscle Damage and Steroid Myopathy Red Flags for Myositis Diagnosis Disease Course Conclusion References 6: Clinical Features of Myositis: Skin Manifestations Introduction Description of Individual DM Rashes Calcinosis Cutis and Other Uncommon Rashes Rashes Associated with Clinically Amyopathic Dermatomyositis Autoantibody Association of the Dermatomyositis Rashes Differential Diagnosis References 7: Clinical Features of Myositis: Lung Manifestations Introduction Asymptomatic Lung Involvement Chronic Cough Rapidly Progressive ILD Conclusion References 8: Clinical Features of Myositis: Cardiac Manifestations Introduction Pathophysiology Clinical Manifestations Subclinical Heart Involvement Diagnostic Modalities Management Prognosis Conclusions and Recommendations References 9: Clinical Features of Myositis: Arthritis, Raynaud Phenomenon, Constitutional Introduction Arthritis and Arthralgia Rheumatoid Factor (RF) and Anti-CCP Positivity in Idiopathic Inflammatory Myopathies Radiographic Findings of Arthritis Raynaud Phenomenon (RP) Constitutional Symptoms Fever Fatigue Myalgia Conclusion References 10: Clinical Features of Myositis: Juvenile Dermatomyositis Introduction Muscle Disease Skin Disease Extra-muscular Disease Features Myositis-Specific Autoantibodies Diagnosis of Juvenile Dermatomyositis References 11: Making the Diagnosis of Myositis: Definition and Classification of Myositis Introduction When to Suspect Myositis Classification Criteria The EULAR/ACR Myositis Classification Criteria IBM Diagnostic/Classification Criteria Conclusion References 12: Making the Diagnosis of Myositis: Electrodiagnostic Testing Introduction Planning the Study Nerve Conduction Study Needle Electromyography Voluntary Contraction: Motor Unit Potential (MUP) Assessment Recruitment EMG Findings in Myositis The Needle Examination Report Caveats and Correlations Differential Diagnosis References 13: Making the Diagnosis of Myositis: Muscle Biopsy and Interpretation Introduction Selecting a Muscle Biopsy Site Processing the Specimen Interpretation of Muscle Biopsy Findings How to Read the Muscle Biopsy Report Dermatomyositis (DM) Polymyositis (PM) Inclusion Body Myositis (IBM) Necrotizing Autoimmune Myopathy Antisynthetase Syndrome Steroid and Other Toxic Myopathies Metabolic Myopathies and PM Mimics References 14: Making the Diagnosis of Myositis: Skin Biopsy and Interpretation Introduction Histologic Findings in Cutaneous Dermatomyositis Where to Biopsy and When to Biopsy? Additional Stains Used in the Workup of Suspected Cutaneous Dermatomyositis Regional/Anatomic and Clinical Variations in Histologic Findings in Cutaneous Dermatomyositis Histologic Differential Diagnosis Ancillary Techniques Conclusion References 15: Making the Diagnosis of Myositis: Lung Biopsy and Interpretation Introduction Histopathologic Patterns of Myositis-Associated Lung Disease Distinguishing Myositis-Associated Lung Disease from Other Lung Diseases Association of Myositis Subtypes and Autoantibodies with Histopathologic Features Prognosis of Myositis-Related Lung Disease Should I Obtain a Lung Biopsy? What Kind of Biopsy Should I Ask for? Conclusion References 16: Making the Diagnosis of Myositis: Muscle MRI Introduction MRI Protocols in IIM Types of Muscle Magnetic Resonance Imagings Diagnostic Yield of Magnetic Resonance Imaging Distribution of Magnetic Resonance Imaging Involvement in IIM Subtypes Magnetic Resonance Imaging in Guiding Muscle Biopsies MRI Pattern in Myositis Mimics Magnetic Resonance Imaging for Disease Activity Versus Damage Magnetic Resonance Imaging for Treatment Response Other Advantages of Magnetic Resonance Imaging in Myositis Functional Magnetic Resonance Imaging Techniques Muscle Ultrasound FDG-PET 99mTechnetium Pyrophosphate Scintigraphy Conclusion References 17: Making the Diagnosis of Myositis: Laboratory Testing in Myositis Introduction Muscle Enzymes: CK, Aldolase, LDH, AST, and ALT Inflammatory Markers: Ferritin, ESR, CRP, etc Anti-nuclear Antibodies and Anti-cytoplasmic Pattern in Myositis Anti-cytoplasmic Pattern in Anti-synthetase Syndrome Myositis Autoantibodies: When to Order and How to Interpret References 18: Role of ANA and Myositis Autoantibodies in Diagnosis Introduction Autoantibody Detection Diagnosis Conclusion References 19: Role of Myositis Autoantibodies in Management and Prognosis Introduction Malignancy Survey Risk Stratification and Treatment of Interstitial Lung Disease Refractory Myopathy References 20: Traditional Myositis Autoantibodies: Synthetase, Mi-2, SRP, Ku, PM-Scl, Ro, U1RNP Introduction Antisynthetase Antibodies (Anti-ARS) Anti-Mi-2 Anti-Signal Recognition Particle Antibodies (Anti-SRP) Anti-PM-Scl and Anti-Ku Anti-Ro/SSA, Anti-U1RNP References 21: Dermatomyositis-Associated Autoantibodies: TIF1-γ, NXP2, and MDA5 Introduction Transcription Intermediary Factor 1-γ (TIF1-γ) Nuclear Matrix Protein 2 (NXP2) Melanoma Differentiation-Associated Gene 5 Protein (MDA5) References 22: Newly Described Myositis Autoantibodies: HMGCR, NT5C1A, SAE, PUF60 Introduction Anti-HMGCR Anti-NT5C1A (Anti-CN1A) Anti-PUF60 Anti-SAE Other Emerging MSA/MAAs References 23: Myositis Mimics: The Differential Diagnosis of Myositis Introduction Myositis Mimics: The Differential Diagnosis of Myositis Identifying and Diagnosing the Metabolic Myopathies McArdle Disease Acid Maltase Deficiency Mitochondrial Myopathies Identifying and Diagnosing the Muscular Dystrophies Facioscapulohumeral Muscular Dystrophy Dysferlinopathy Calpainopathy Identifying the Inclusion Body Myositis Patient Endocrinopathies and Muscle Symptoms Toxic Myopathies Amphiphilic Drugs Antimicrotubular Myopathy Antiretroviral Agents Alcohol Self-Limited Statin-Associated Myopathic Symptoms Steroid Myopathies Other Myositis Mimics Funding References 24: Immune-Mediated Necrotizing Myopathy (IMNM) Introduction Anti-HMGCR Autoantibody-Positive Myopathy, Also Called Statin-Associated Immune-Mediated Necrotizing Myopathy Anti-SRP Autoantibody-Positive Myopathy Autoantibody-Negative Immune-Mediated Necrotizing Myopathy Immune-Mediated Necrotizing Myopathies Associated with Anti-Synthetase Syndrome and Other Connective Tissue Diseases Cancer-Associated Necrotizing Myopathy Selected Management Features Management of Anti-HMGCR Ab+ Myopathy Anti-SRP+ Myopathy Conclusion References 25: Cancer-Associated Myositis Introduction Epidemiology Risk Factors Autoantibody Associations Cancer Screening Role of Tumor Markers in Cancer Screening Role of Positron Emission Tomography/Computed Tomography (PET/CT) in Cancer Screening Follow-Up of Cancer-Associated Myositis Management References 26: Myositis-Associated Interstitial Lung Disease Introduction Case No. 1 Case No. 2 Symptoms Physical Examination Signs and Symptoms of Possible Underlying Myositis Autoantibodies Associated with ILD (See Chaps. 20–21) Demographics and Clinical Features Associated with ILD Natural History of ILD Radiographic Patterns in Myositis-Associated ILD Strategies for Treating ILD Pharmacologic Therapy Nonpharmacologic Therapy Prognosis of Myositis-Associated ILD Outcomes and Selection for Lung Transplantation Other Pulmonary Complications Conclusion References 27: Pregnancy in Myositis Introduction Pregnancy Effect on the Disease Disease Effect on Pregnancy (Maternal and Fetal Outcomes) Anesthetic Management for Cesarean Delivery in Myositis Patients Clinical and Pathological Correlations Treatment of Myositis During Pregnancy Concomitant Medication Conclusion References 28: Pathogenesis of Myositis Introduction Genetics and Environment Adaptive Immunity Role of B and T Cells Role of Innate Immunity Non-immune Mechanisms Vascular Pathology and the Role of Ischemia Conclusions References 29: Management Considerations: Pharmacologic Intervention Introduction Immunosuppressive Medications Intravenous Immunoglobulin (IVIg) Biologic Agents Other Treatment Considerations Conclusion References 30: Management Considerations: Juvenile Dermatomyositis Introduction General Approach Pharmacologic Therapy Glucocorticoids Hydroxychloroquine (Plaquenil) Methotrexate Cyclosporine Mycophenolate Mofetil (MMF) Azathioprine (Imuran) Cyclophosphamide (Cytoxan) Intravenous Immunoglobulin (IVIG, Gamunex, Gammagard) Biological Therapies B-Cell Depletion Rituximab (Rituxan) Anti-T Cell Abatacept (Orencia) Anti-cytokines Tocilizumab (Actemra) Anti-tumor Necrosis Factor (Anti-TNF) Infliximab (Remicade) Etanercept (Enbrel) Future Therapeutic Targets Additional Treatments Stem Cell Transplantation Physical Therapy and Exercise Skin Involvement Calcinosis Conclusion References 31: Management Considerations: Refractory Skin Rash and Calcinosis Introduction Principles of Management of Cutaneous Dermatomyositis Differentiate Active Skin Disease from Damage Target all Organs Involved, Not Just the Skin Topical Therapy Is Mainly Adjunctive Therapy Discuss the Importance of Photoprotection Knowing the Patient’s Dermatomyositis-Specific Antibody May Be Useful Most Treatment Guidelines for Cutaneous Dermatomyositis Are Expert Opinions Treatment Ladder for the Inflammatory Erythema of Cutaneous Dermatomyositis Biologics and Other Agents Specific Scenarios Calcinosis Procedural Options Calcium Modulators and Vasoactive Medications Anti-inflammatory Medications References 32: Management Considerations: Interstitial Lung Disease Introduction Specific Agents, Dosage, and Side Effects Treatment Consideration for Distinct Clinical Scenarios Monitoring of Therapy Conclusion References 33: Role of Exercise in the Management of Myositis Introduction The Role of Physical and Occupational Therapy Physical Therapy The Anti-inflammatory Effects of Exercise Intervention Exercise as a Treatment Modality in PM and DM: A Randomized Controlled Trial Effects of Exercise in PM and DM Exercise Programs in Inclusion Body Myositis Exercise Programs in Juvenile Dermatomyositis (JDM) Introducing Exercise to a Patient Occupational Therapy Activities of Daily Living in Myositis Polymyositis and Dermatomyositis Inclusion Body Myositis Occupational Therapy Treatment Conclusion References 34: Dietary Considerations in Myositis Introduction Gluten Sensitivity and Gluten-Free Diet Vitamin D Deficiency Creatine Supplementation Protein Supplementation Retinoid Acid Dietary Statin Anti-inflammatory Diet and Gut Microbiome Conclusion References 35: Distinguishing Disease Activity and Damage in Myositis Introduction Muscle Weakness: Activity vs. Damage Differentiating Activity from Damage Based on Muscle Testing Dyspnea: Activity vs. Damage Assessment of the Skin Assessment of Joints Conclusions References Index