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.

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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