A Clinician’s Pearls & Myths in Rheumatology

Description: This book builds upon the first edition with new and improved chapters. The book explores the rich assemblage of clinical wisdom from expert rheumatologists from a wide range of specialties including Vasculitis and Sjogren’s syndrome. It examines the nuggets or ‘pearls’ of wisdom gained from collective clinical experience about the diagnosis or treatment of various diseases whilst also aiming to debunk myths that have influenced the practice of rheumatology by many clinicians. The second edition of A Clinician’s Pearls and Myths in Rheumatology is a critical resource for both practitioners and students of rheumatology. This book provides a clear guide for those wishing to take a “deep dive” into the diagnostic and therapeutic elements of rheumatology. See more medical ebooks at here: Osteoporosis in Clinical Practice Case Files: Internal Medicine 6th Edition 2021 The Role of the Human Gutome on Chronic Disease Immune-Mediated Myopathies and Neuropathies Malignant peritoneal mesothelioma How to request a test: A clinician’s guide to the interpretation and evaluation of medical tests Preface When I was a child in the second grade, my mother developed an acutely painful index finger on her right hand. After several sleepless nights with unrelenting pain that was disguised from me, she was admitted to the hospital. The surgeon suspected a glomus tumor—a benign growth that sometimes develops in the nailbed. But no tumor was found at surgery. Instead, the surgeon shared an astonishing observation with my father: “When I removed her fingernail, she didn’t bleed.” And at that point, the nature of her disease began to dawn on her doctors and on my father, who was himself a physician. My mother had developed Raynaud’s phenomenon as a teenager. When she and my father were newlyweds and he was a medical student, they had mused together at the oddity of her intermittently pale white fingers, which developed sometimes even in the summer. Around the time I was born, she suffered from a skin problem that I learned—years later—was dermatitis herpetiformis. As a young boy, I remember her scratching her intensely itchy legs. As I grew up, I became aware of the subtle scars left on her face, usually well-hidden with makeup. The active skin inflammation faded after a few years, but other aspects of the autoimmune kaleidoscope came into sharper focus. The ischemic finger in the context of her preceding issues led to the diagnosis of scleroderma. In that light, most of the medical events in my mother’s life make sense now. Her finger hadn’t bled at surgery because of the severity of vasoconstriction. The shapes of her fingers that I can still see and remember dearly, including the curved fingernail that never grew back normally, were the result of her disease. The calcinoses and digital pitting that developed in her fingers were also consequences of her slowmoving but relentless illness, as were the intermittent bouts of dysphagia caused by esophageal dysmotility. For a time, during my teens, my mother often had to excuse herself from the dinner table because she just couldn’t swallow her food. A painful memory for me. Despite that, Mom led a full life and it seemed to most that nothing slowed her down: raising two rambunctious boys; being a loving wife and returning to graduate school; becoming a devoted first-grade teacher; using her hands constantly in spite of sensitive fingers on sewing, knitting, cooking, and playing the piano; enjoying lifelong friends; attending our soccer games with mittens on to counter Raynaud’s attacks; and delighting in family travel. It all came to a halt far too early. When I was a medical intern, the hospital operator paged me to return a call from my father. Mom had developed an acute bowel obstruction because of scleroderma gut and had been taken to surgery that morning. I flew home, to her bedside in the intensive care unit. There, far more fragile that anyone had realized, she died twelve days later of adult respiratory distress syndrome. The real cause, of course, was scleroderma. She was 54 years old. It is little consolation that traumatic health events in one’s family make a physician a better doctor. Yet it is true. My mother’s nearly lifelong struggle with scleroderma—ironically regarded as “limited”—helps me understand the uncertainties, frustrations, and tears of my patients and their families. Mom’s illness, of course, contributed to my becoming a rheumatologist and dedicating my career to helping patients address diseases like hers. It is only fitting, then, that this book is dedicated to her. So much has happened since the First Edition was published in 2009. New rheumatic diseases have been identified. (IgG4-related disease was scarcely mentioned in the First Edition!) Biomedical science has witnessed important advances in assessment and diagnosis, some of which have already altered approaches to patient care. Creative new therapies have been conceived and studied. Some in fact have worked, been approved, and are already improving patients’ lives. The world has endured (and still persists in) a viral pandemic that has underscored the importance of vaccines and public health and forced us to re-think how we apply many of our existing treatments. Though many challenges remain, my inherent optimism – a trait inherited from my mother – inspires confidence that the coming years will mark growing progress for the patients we treat. The judgment of inspired clinicians will remain fundamental to inspiring advances and maintaining safe speeds on the paths ahead. It is in this spirit that this book is written: by clinicians, for clinicians. I am grateful to the more than 200 contributors who offer guidance here from their own approaches to our art, sharing their hard-earned and frequently elegant clinical wisdom. Boston, MA, USA John H. Stone Table of contents : Preface Contents Contributors 1: Rheumatoid Arthritis Early Rheumatoid Arthritis Clinical Features Serological Features and Radiology Risk Factors Disease Assessment Treatment Glucocorticoids, Methotrexate, and Conventional DMARDs Biologic Agents Adverse Effects Comorbidities Immunizations in RA References 2: Rheumatoid Vasculitis References 3: Adult-Onset Still’s Disease References 4: Juvenile Idiopathic Arthritis Diagnosis Clinical Manifestations Systemic JIA Management References 5: Monogenic Autoinflammatory Syndromes Vacuoles, E1 Activating Enzyme, X-Linked Autoinflammatory Somatic Syndrome (VEXAS) Familial Mediterranean Fever Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS) Hyper Immunoglobulin D and Periodic Fever Syndrome Cryopyrin-Associated Periodic Syndromes Chronic Recurrent Multifocal Osteomyelitis/Chronic Nonbacterial Osteomyelitis Cherubism Haploinsufficiency of A20 (HA20) References 6: Juvenile Spondyloarthritis References 7: Axial Spondyloarthritis References 8: Psoriatic Arthritis References 9: Reactive Arthritis References 10: Systemic Sclerosis (Scleroderma) and Raynaud’s Phenomenon Raynaud’s Phenomenon Diagnosis Autoantibodies SSc Overlaps Gastrointestinal Involvement Lung Disease in SSc Pulmonary Arterial Hypertension Cardiac Disease in SSc Renal Disease in SSc Arthritis Peripheral Neuropathy in SSc Stem Cell Transplantation References 11: Sjögren’s Disease Nomenclature Diagnosis and Classification Epidemiology Pathogenesis Sicca Features Ocular Manifestations Oral Manifestations Evaluation Major Salivary and Lacrimal Gland Involvement Extraglandular Involvement Laboratory Findings Immunological Assays Differential Diagnosis Prognosis and Outcome Treatment General Measures Systemic Manifestations References 12: Systemic Lupus Erythematosus Diagnosis Clinical Features Serology Cutaneous Lupus Lupus Nephritis Neuropsychiatric Lupus General Management Points Treatment References 13: Childhood-Onset SLE and Neonatal Lupus Erythematosus Neonatal Lupus: Pearls and Myths References 14: The Antiphospholipid Syndrome Prevalence of aPL and Classification Criteria Classification Criteria Risk Factors for Thrombosis in APS Morbidity and Mortality Conditions Associated with APS Clinical Manifestations of APS Cutaneous Manifestations Cerebral Manifestations Catastrophic Antiphospholipid Syndrome Novel Clinical Features Treatment Risk Factor Reduction Asymptomatic Patients Thrombotic Events Pregnancy References 15: Reproductive Health in the Rheumatic Diseases General Considerations Medications Systemic Lupus Erythematosus Antiphospholipid Syndrome (APS) Neonatal Lupus Syndromes Rheumatoid Arthritis References 16: Inflammatory Myopathies Dermatomyositis Versus Polymyositis Versus Other Idiopathic Inflammatory Myopathies (IIM Skin Disease Muscle Enzymes Myositis-Specific Auto-antibodies Imaging Inclusion Body Myositis Toxic Myopathies Treatment References 17: Juvenile Dermatomyositis References 18: Vasculitic Neuropathy Presentation Diagnosis: Electrodiagnostic Studies and Biopsy Pathophysiology Treatment References 19: Pediatric Vasculitis Kawasaki Disease IgA Vasculitis (Formerly Henoch-Schönlein Purpura) Polyarteritis Nodosa References 20: Behçet Syndrome Pearls and Myths: Behçet Syndrome References 21: Eosinophilic Granulomatosis with Polyangiitis References 22: Granulomatosis with Polyangiitis Epidemiology Nose, Sinuses, and Ears Subglottic Stenosis Mouth Eyes Lungs Kidneys Bladder Skin Joints Peripheral and Cranial Nerves Central Nervous System Spleen ANCA Treatment and Course References 23: Microscopic Polyangiitis Lungs Kidneys Skin Joints Peripheral and Cranial Nerves ANCA Treatment and Course References 24: Oral Manifestations Associated with Rheumatic Diseases Oral Manifestations of the Rheumatic Diseases Introduction Aphthous and Aphthous-Like Ulcerations Lichenoid Mucosal Lesions Pigmented Mucosal Lesions Gingival Enlargement Salivary Gland Hypofunction Burning Mouth Coated Tongue Medication-Related Osteonecrosis of the Jaw Chronic Nonbacterial Osteomyelitis Osteolysis in Systemic Sclerosis Temporomandibular Joint Disorder Dysphagia Microstomia Giant Cell Arteritis and Jaw Claudication Periodontal Disease Bibliography 25: Cryoglobulinemia References 26: Polyarteritis Nodosa References 27: Giant Cell Arteritis and Polymyalgia Rheumatica GCA: Epidemiology GCA: Signs and Symptoms PMR: Signs and Symptoms GCA: Ophthalmic Symptoms and Findings Laboratory Testing GCA: Imaging Temporal Artery Biopsy Pathology Treatment Glucocorticoids Tocilizumab GCA: Methotrexate PMR Outcomes References 28: Takayasu’s Arteritis References 29: Central Nervous System Vasculitis and Reversible Cerebral Vasoconstriction Syndrome References 30: Thromboangiitis Obliterans References 31: Less Common Vasculitides Cogan’s Syndrome Erythema Elevatum Diutinum Urticarial Vasculitis References 32: Relapsing Polychondritis Ear Involvement Nasal Involvement Respiratory Involvement Other Manifestations Treatments References 33: Fibromyalgia References 34: Clinical Features of Gout References 35: Epidemiology of Gout References 36: Treatment of Gout References 37: CPPD and Other Microcrystalline Disorders Symptoms and Signs Radiology Diagnosis Epidemiology and Genetics Pathophysiology Treatment and Outcomes References 38: Inflammatory Eye Disease Retina Susac Syndrome Central Serous Retinopathy Optic Neuritis Scleritis and Peripheral Ulcerative Keratitis Giant Cell Arteritis Uveitis Orbital Inflammatory Disease Histiocytoses IgG4-Related Disease References 39: Immune-Mediated Inner Ear Disease Definition The Clinical History Physical Findings Auditory and Vestibular Tests Differential Diagnosis Treatment References 40: Osteoporosis: Epidemiology and Assessment Osteoporosis Epidemiology Osteoporosis Diagnosis: Bone Mass Measurement References 41: Osteoporosis Prevention and Treatment Osteoporosis Prevention Osteoporosis Treatment References 42: Paget’s Disease of Bone References 43: Lyme Epidemiology/Prevention Diagnosis Lyme Arthritis Post-Lyme Disease Symptoms References 44: Osteoarthritis Myths Pearls References 45: Regional Musculoskeletal Problems Introductory Pearl General Points on Performing Arthrocentesis and Joint Injection Tendinitis: General Points Shoulder Elbow Wrist Hand Coccyx Leg Cramps Hip Knee Ankle References 46: Low Back and Neck Pain Low Back Pain Epidemiology History and Physical Examination Electrodiagnostic and Laboratory Studies Imaging Lumbar Spondylosis Disk Herniation Cauda Equina Syndrome Spondylolisthesis Spinal Stenosis Diffuse Idiopathic Skeletal Hyperostosis (DISH) Nonspecific LBP (Idiopathic LBP) Neoplasm Infection Miscellaneous Treatment General Principles Treatment of Acute LBP Treatment of Chronic LBP Treatment of Disk Herniation Treatment of Spinal Stenosis Neck Pain References 47: The Ehlers-Danlos Syndromes References 48: Sarcoidosis References 49: Amyloidosis References 50: IgG4-Related Disease Clinical Findings Pathology Laboratory Studies Imaging Treatment Prognosis References 51: Castleman Disease References 52: Erdheim-Chester Disease References 53: Kikuchi-Fujimoto Disease References 54: Whipple’s Disease Etiology and Epidemiology Immunopathogenesis of Tropheryma whipplei Pathology Clinical Presentation Gastrointestinal Manifestations Rheumatologic Manifestations Neurologic Manifestations Neuromuscular Manifestations Cardiac Manifestations Other Organ Manifestations Diagnosis Treatment Complications References Index