Pocket Primer on the Rheumatic Diseases (eBook)

John H. Klippel, MD - President and CEO of the Arthritis Foundation based in Atlanta, GA, Dr Klippel has more than 30 years experience in rheumatology, and since joining the Arthritis Foundation in 1999 as medical director, has provided overall direction to the Arthritis Foundation's research programs. He previously served as clinical director of the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) and as the scientific director of the Alliance for Lupus Research. His honours and awards include the Surgeon General's Exemplary Service Award, Distinguished Clinical Teacher Award (NIH Clinical Center), Directors Award (NIH Clinical Center) and the Burroughs-Wellcome Visiting Professor Award from the Royal Society of Medicine in London. John H. Stone, MD, MPH is Clinical Director of Rheumatology, Harvard Medical School, Boston, MA, USA and the Chairman of the International Network for the Study of the Systemic Vasculitides (INSSYS). Dr Stone's research interests relate to translational investigations in systemic vasculitis. He has directed two multicenter clinical trials in Wegener's granulomatosis and antineutrophil cytoplasmic antibody-associated vasculitis, the Wegener's Granulomatosis Etanercept Trial (WGET) and the Rituximab in ANCA-associated Vasculitis (RAVE) trial. His other research efforts relate to the identification of new biomarkers through proteomic approaches, and to the development of clinical trials' outcome measures in rheumatic autoimmune diseases. Leslie J Crofford MD - Gloria W. Singletary Professor of Internal Medicine, Chief of the Division of Rheumatology and Director of the Center for the Advancement of Women’s Health at the University of Kentucky, Lexington, KY. Dr Crofford specialises in auto-immune diseases, with research interests including rheumatoid arthritis, myositis and lupus treatments. Patience H White MD MA - Chief Public Health Officer at Arthritis Foundation, Professor of Medicine and Pediatrics at George Washington School of Medicine and Health Sciences (GWU) and also Executive Director of the Adolescent Employment Readiness Center, Atlanta, GA. Dr White has published over 60 articles and has been the principle investigator on 23 peer-reviewed grants and contracts in her specialized area of independent functioning in adulthood for youth with disabilities.

CONTRIBUTORS 4
Table of Contents 6
INTRODUCTION 7
1 HISTORY AND PHYSICAL EXAMINATION 8
History 8
GALS (Gait, Arms, Legs, Spine) Screening 8
Examination of Specific Joint Areas 10
Hand and Wrist 10
Elbow 11
Shoulder 11
Hip 12
Sacroiliac Joint 13
Spine 13
Knee 14
Ankle and Hindfoot 15
Midfoot and Forefoot 15
2 LABORATORY ASSESSMENT 16
Acute-Phase Reactants 16
Erythrocyte Sedimentation Rate (ESR) 16
C-Reactive Protein 16
Rheumatoid Factor 17
Antinuclear Antibody (ANA) 18
Specific Autoantibodies 18
Complement 18
3 ARTHROCENTESIS AND SYNOVIAL FLUID ANALYSIS 23
Indications 23
Technique 23
SF Analysis 26
4 IMAGING TECHNIQUES 28
Conventional Radiography 28
Computed Tomography 28
Magnetic Resonance Imaging 29
Bone Densitometry 29
Ultrasound 30
Scintigraphy 30
5 MONARTICULAR JOINT DISEASE 32
6 POLYARTICULAR JOINT DISEASE 34
7 REGIONAL RHEUMATIC PAIN SYNDROMES 36
Disorders of the Lower Back 37
8 FIBROMYALGIA AND DIFFUSE PAIN SYNDROMES 51
Clinical Features 51
Diagnosis 52
Treatment 54
9 RHEUMATOID ARTHRITIS 55
Clinical Features 55
Articular Manifestations 55
Two categories 55
Morning stiffness 55
Signs of synovitis 55
Structural damage 55
Extra-Articular Manifestations 56
Constitutional symptoms 56
Organs 56
Laboratory Features 57
Diagnosis 58
Treatment 61
Goals 61
Educate 61
Referral 61
Nonsteroidal anti-inflammatory drugs (NSAIDS) 61
Glucocorticoids 61
Disease-modifying antirheumatic drugs (DMARDs) and bio- logicresponse modifiers 61
10 JUVENILE IDIOPATHIC ARTHRITIS 63
Clinical Features 63
Laboratory Features 65
Diagnosis 65
Treatment 66
Special Considerations 68
Examination 68
Height and weight 68
Infants and toddlers 68
School-aged children 68
Adolescents 68
Growth 69
Multifactorial 69
Impact of JIA subtype 69
Local impact 69
Other factors 69
Nutrition 69
Adherence 69
Factors affecting adherence 69
Strategies for improving adherence 69
Child Development 69
Psychosocial 69
School and educational achievement 69
11 PSORIATIC ARTHRITIS 70
Clinical Features 70
Radiographic Features 71
Diagnosis 71
Treatment 72
12 ANKYLOSING SPONDYLITIS 73
Clinical Features 73
Laboratory Features 74
Imaging 74
Diagnosis 75
Treatment 77
13 REACTIVE AND ENTEROPATHIC ARTHRITIS 78
Reactive Arthritis 78
Clinical Features 78
Antecedent extra-articular infection 78
Joints affected 78
Signs and symptoms 78
Dactylitis (sausage digit) 78
Enthesitis 78
Lower back and buttock pain 78
Extra-articular features 78
Laboratory Features 78
Seronegativity 78
Diagnosis 79
Clinical diagnosis 79
Exclude septic arthritis 79
Exclude psoriatic arthritis 79
Treatment 79
Nonsteroidal anti-inflammatory drugs (NSAIDs) 79
Glucocorticoids 79
Disease-modifying antirheumatic drugs (DMARDs) 79
Tumor necrosis factor (TNF) inhibitors 79
Enteropathic Spondyloarthritis 79
Clinical Features 79
IBD 79
Arthritis 80
Peripheral arthritis 80
Axial arthritis 80
Skin manifestations of IBD 80
Ocular complications of IBD 80
Laboratory Features 80
Seronegativity 80
Anemia 80
Acute-phase reactants 80
Human leukocyte antigen (HLA)-B27 80
Diagnosis 81
Clinical diagnosis 81
Magnetic resonance imaging (MRI) 81
Radiographs 81
Treatment 81
NSAIDs 81
Glucocorticoids 81
Sulfasalazine 81
TNF inhibitors 81
14 OSTEOARTHRITIS 82
Clinical Features 82
Laboratory Features 84
Radiographic Features 84
Diagnosis 84
15 CALCIUM PYROPHOSPHATE DIHYDRATE, HYDROXYAPATITE, AND MISCELLANEOUS CRYSTALS 89
Calcium Pyrophosphate Dihydrate Crystals 89
Clinical Features 89
Crystal deposition 89
Pseudogout 89
Pseudo-osteoarthritis 89
Pseudo-rheumatoid arthritis 89
Pseudo-neuropathic anthropathy 90
Lanthanic 90
Laboratory Features 90
Pseudogout 90
Pseudo-rheumatoid arthritis 90
Diagnosis 90
Polarizing light microscopy 90
Radiographic Features 91
Punctate and linear densities 91
Screening views 91
Metacarpophalangeal joints 92
Treatment 92
Aspiration 92
Nonsteroidal anti-inflammatory drugs (NSAIDs) 92
Oral colchicine 92
Systemic glucocorticoids 92
Apatite/Basic Calcium Phosphates 92
Clinical Features 92
Osteoarthritis 92
Shoulder 92
Calcific periarthritis 93
Acute arthritis 93
Calcinosis 93
Diagnosis 93
Microscopy 93
Radiographic Features 93
Periarticular calcification 93
Destructive arthritis of the shoulder 93
Treatment 93
NSAIDs 93
Miscellaneous Crystals 93
16 INFECTIOUS DISORDERS 94
Septic Arthritis 94
Clinical Features 94
Risk factors 94
Joints affected 94
Suspect septic arthritis 94
Underlying inflammatory arthritis 94
Gonococcal arthritis 94
Hepatitis B virus 94
Hepatitis C virus 94
HIV 94
Laboratory Features 95
Synovial fluid 95
Gram stain 95
Culture 95
Diagnosis 95
Suspect septic arthritis 95
Pseudoseptic arthritis 95
Treatment 95
Prompt treatment 95
Begin antibiotics immediately 95
Antibiotic choice 96
Drain joint space 96
Immobilization and analgesics 96
Physical therapy 96
Lyme Disease 96
Clinical Features 96
Erythema migrans 96
Early localized disease 96
Early disseminated disease 96
Late disease 97
Chronic arthritis of Lyme disease 97
Chronic neurologic manifestations 97
Laboratory Features 97
Diagnosis 97
History and physical findings 97
Confirm 97
False positive 97
Seropositive 97
Exclude similar entities 97
Treatment 99
Antibiotic treatment 99
Refractory Lyme arthritis 99
Response to therapy 99
17 SYSTEMIC LUPUS ERYTHEMATOSUS 100
Clinical Features 100
Skin 100
Malar or “butterfly” rash 100
Discoid lupus (DLE) 100
Lupus panniculitis 100
Subacute cutaneous lupus erythematosus (SCLE) 100
Alopecia 100
Mucosal lesions 101
Vasculitis 101
Vasculopathy 101
Musculoskeletal System 101
Arthritis, arthralgia 101
Joints affected 101
Myalgia and muscle weakness 101
Renal 101
Asymptomatic 101
Abnormal urinalysis 101
Proteinuria 102
Renal function 102
Renal biopsy 102
Nervous System 102
Neuropsychiatric systemic lupus 102
Psychiatric disorders 102
Cognitive defects 102
Seizures 102
Meningitis 102
Cranial nerves 102
Cardiovascular System 102
Pericarditis 102
Accelerated atherosclerosis 102
Primary myocardial involve- ment 103
Pleura and Lungs 103
Pleuritis 103
Pulmonary involvement 103
Pulmonary hypertension 103
Laboratory Features 103
Hematologic Abnormalities 103
CBC 103
Anemia 103
Leukopenia 103
Thrombocytopenia 104
Autoantibodies and Complement 104
ANA 104
dsDNA 104
Anti-Sm antibodies 105
SSA/Ro and SSB/La 105
Complement 105
Diagnosis 105
Treatment 107
General Management 107
Patient education 107
Identify concomitant conditions 107
Photoprotection 107
Review prescription drugs 107
Infections 107
Pregnancies 107
Cardiovascular disease 107
Osteoporosis 107
Adverse effects of NSAIDs 108
Topical or intralesional preparations 108
Intra-articular glucocorticoids 108
Oral or parenteral therapy 108
Low dose 108
Higher dose 108
Uses 108
Combinations 108
18 ANTIPHOSPHOLIPID SYNDROME 111
Clinical Features 111
Laboratory Features 111
Diagnosis 112
Treatment 113
19 SYSTEMIC SCLEROSIS 114
Clinical Features 114
Raynaud’s Phenomenon 114
Reversible vasospasm of digital vessels 114
Tissue damage 114
Skin 114
Early changes 114
Late changes 114
Musculoskeletal 115
Early features 115
Nonspecific musculoskeletal complaints 116
Pain and stiffness 116
Late features 116
Pulmonary 116
Early features 116
Rales 116
Detection 116
Course 116
Mortality 116
Gastrointestinal 116
Mouth 116
Upper esophagus 116
Lower esophagus 116
Small intestine 116
Large intestine 117
Cardiac 117
Clinical manifestations 117
Pericardial effusion 117
Renal 117
Renal crisis 117
Outcome 117
Risk factors 117
Laboratory Features 117
Diagnosis 118
Treatment 118
Raynaud’s phenomenon 118
Prevent attacks 118
Vasodilator therapy and oral analgesics 118
Necrosis 119
Skin 119
Topical moisturizers 119
Pruritus 119
Skin ulcers 119
Subcutaneous calcinosis 119
Colchicine 119
Musculoskeletal 119
Goals 119
Physical therapy 119
Noninflammatory myopathy 119
NSAIDs 119
Pure analgesics 120
Glucocorticoids and methotrexate 120
Gastrointestinal 120
Nonpharmacologic treatments 120
Pharmacologic therapies 120
Promotility agents 120
Broad-spectrum antibiotics 120
Pseudo-obstruction 120
Kidneys 120
ACE inhibitors 120
Angiotensin-II receptor inhibitor 120
Early detection 120
20 IDIOPATHIC INFLAMMATORY MYOPATHIES 121
Disease Subsets 121
Clinical Features 122
Laboratory Features 122
Diagnosis 123
Treatment 123
21 METABOLIC MYOPATHIES 125
Diagnosis 125
Treatment 128
Muscle glycogenosis 128
Modified exercise regimens 128
Modified diet 128
Secondary Metabolic Myopathies 129
22 SJÖGREN’S SYNDROME 130
Clinical Features 130
Ocular Manifestations 130
Gritty eyes 130
Matter 130
Oral Manifestations 130
Common oral symptoms 130
Oral examination 130
Salivary glands 130
Cutaneous 130
Dry skin 130
Thyroid 131
Thyroid disease 131
Respiratory 131
Nonproductive cough 131
Progressive pulmonary disease 131
Musculoskeletal 131
Symmetrical polyarthritis 131
Neurologic 131
Peripheral neuropathy 131
Hematologic 131
Lymphoid malignancy 131
Reproductive 131
Vaginal dryness 131
Urinary Tract 131
Irritable bladder 131
Increased urinary frequency 131
Renal abnormalities 131
Laboratory Features 132
Tests 132
Minor salivary gland biopsy 132
Schirmer’s test 132
Fluorescein tear film 132
Staining 132
Diagnosis 132
Treatment 134
23 VASCULITIDES 136
Giant Cell Arteritis, Polymyalgia Rheumatica, and Takayasu’s Arteritis 136
Clinical Features 136
GCA 136
Onset 136
Cranial GCA 136
Generalized inflammatory syndrome 136
Large vessel GCA/aortitis 136
Isolated PMR 136
Muscle pain/stiffness 136
Constitutional features 136
TA 137
Onset 137
Generalized inflammatory syndrome 137
Carotid and vertebral artery involvement 137
Claudication 137
Pulselessness 137
Cardiac disease 137
Aortic regurgitation 137
Myocardial ischemia 137
Aneurysms 137
Gastrointestinal (GI) symptoms 137
Laboratory Features 137
GCA 137
Acute-phase reactants 137
Anemia 137
Platelets 138
Liver function tests 138
Diagnosis 138
Vaso-occlusive disease and systemic inflammation 138
Temporal artery biopsy 138
Bruits 138
Aortography 138
Differential diagnosis for PMR 138
Treatment 138
Glucocorticoids 138
Aspirin 138
Methotrexate 138
Polyarteritis Nodosa 138
Clinical Features 139
Fibrinoid necrosis 139
Microaneurysms 139
Constitutional symptoms 139
Kidneys 139
Cutaneous manifestations 139
Neuropathy 139
GI symptoms 139
Testicular pain or tenderness 139
Coronary involvement 139
Laboratory Features 139
Antineutrophil cytoplasmic antibodies (ANCA) 139
Diagnosis 139
Physical examination 139
Angiography 140
Biopsy 140
Differential diagnosis 140
Treatment 140
Glucocorticoids 140
Cyclophosphamide 140
The Antineutrophil Cytoplasmic Antibody-Associated Vasculitides: Wegener’s Granulomatosis, Microscopic Polyangiitis and the Churg–Strauss Syndrome 141
Clinical Features 142
Skin 142
Arthritis/arthralgias 142
GI involvement 142
Diagnosis 143
Histopathology 143
Exclude rheumatoid arthritis (RA) 143
Treatment 144
Rituximab 144
Cyclophosphamide 144
Azathioprine or methotrexate 144
Glucocorticoids 144
Immune Complex-Mediated Vasculitides 144
Clinical Features 144
Hypersensitivity Vasculitis (sometimes termed “cutaneous leukocytoclastic angiitis”) 144
ACR criteria 144
Cryoglobulinemic Vasculitis 145
Neurologic manifestations 145
Vascular stasis 145
Purpura, arthralgias, and myalgias 145
Systemic 145
HSP 145
IgA deposition 145
Antecedent infection 145
ACR criteria 145
Hypocomplementemic Urticarial Vasculitis 145
Urticaria 145
Arthralgias and myalgias 145
Hypocomplementemic urticarial vasculitis syndrome (HUVS) 145
Diagnosis 146
Biopsy 146
Light microscopy 146
Direct immunofluorescence 146
Serological testing 146
Exclude systemic lupus erythematosus 146
Treatment 146
Underlying cause 146
Glucocorticoids 146
Immunosuppressants 146
Biologic agents 147
Miscellaneous Vasculitis (Primary Angiitis of the Central Nervous System, Cogan’s Syndrome, and Erythema Elevatum Diutinum) 147
Clinical Features 147
Primary Angiitis of the Central Nervous System (PACNS) 147
Headache 147
CNS complications 147
Cognitive function 147
Differential diagnosis 147
Cogan’s Syndrome 147
Sensorineural hearing loss 147
Ocular 147
Systemic 148
Erythema Elevatum Diutinum (EED) 148
Cutaneous lesions 148
Comorbidity 148
Diagnosis 148
PACNS 148
Biopsy 148
Cerebral angiogram 148
Cerebral spinal fluid (CSF) 148
Magnetic resonance imaging 148
Exclude CNS infection 148
Progressive multifocal symptoms 148
Cogan’s Syndrome 149
Onset 149
EED 149
Vestibular testing 149
Exclude 149
Treatment 149
PACNS 149
Aggressive therapy 149
Cogan’s Syndrome 149
Glucocorticoids 149
EED 149
Comorbid conditions 149
Dapsone 149
Kawasaki’s Disease 149
Clinical Features 150
Principal criteria 150
Diagnosis 150
American Heart Association (AHA) guidance 150
Treatment 150
Salicylates 150
IVIG 150
Glucocorticoids 150
24 BEHÇET’S SYNDROME 151
Clinical Features 151
Laboratory Features 152
Diagnosis 152
Treatment 154
25 ADULT STILL’S DISEASE 155
Clinical Features 155
Laboratory Features 155
Radiographic Features 155
Diagnosis 155
Treatment 156
Acute Disease 156
NSAIDs 156
Liver function 156
Glucocorticoids 156
Chronic Disease 157
Arthritis control 157
Adjunct therapy 157
26 AUTOINFLAMMATORY DISORDERS /PERIODIC SYNDROMES 158
Clinical Features and Treatment 158
27 LESS COMMON ARTHROPATHIES 162
Hematologic and Malignant Disorders 162
Hemophilia and Musculoskeletal Symptoms 162
Recurrent hemarthrosis 162
Causes 162
Radiographs 162
Treatment of acute hemarthrosis 162
Treatment of chronic hemarthrosis 162
Sickle Cell Disease and Musculoskeletal Symptoms 162
Sickle cell arthropathy 162
Recurrent painful crises 162
Osteonecrosis 162
Dactylitis 162
Osteomyelitis 163
Painful crisis prevention 163
Painful crisis treatment 163
Thalassemia and Musculoskeletal Symptoms 163
Musculoskeletal manifestations 163
Malignant Disease and Musculoskeletal Symptoms 163
Mechanisms 163
Rheumatic Disease and Endocrinopathies 163
Hypolipoproteinemia and Arthritis 165
Neuropathic Arthropathy 166
Dermatologic Disorders 167
Neutrophilic Dermatoses 167
Sweet’s syndrome 167
Pyroderma gangrenosum 167
Other dermatoses 167
Panniculitides 167
Erythema nodosum 167
Weber–Christian disease 168
Lupus panniculitis 168
Lipodermatosclerosis 168
Calcifying panniculitis 168
Cytophagic histiocytic panniculitis 168
Sclerosing/Fibrosing Diseases 168
Localized scleroderma 168
Lichen sclerosus 168
Scleromyxedema 168
Nephrogenic systemic fibrosis 168
Pustular Conditions 169
Generalized pustular psoriasis 169
SAPHO syndrome 169
PAPA syndrome 169
Hidradenitis suppurativa 169
Acne fulminans 169
Other Dermatoses 169
Conditions with possible rheumatic consequences 169
Hypertrophic Osteoarthropathy 169
Clinical Features 169
Clubbing 169
Periostosis 169
Bone pain 170
Pachyderma 170
Diagnosis 170
Clubbing 170
Adjacent bone pain 170
Radiographs 170
Mild periostosis 170
Severe periostosis 170
Joint space 170
Radionuclide bone scanning 170
Treatment 170
Analgesics or NSAIDs 170
Bisphosphonates 170
28 COMPLEX REGIONAL PAIN SYNDROME 171
Clinical Features 171
Diagnosis 172
Treatment 172
29 OSTEONECROSIS 174
Clinical Features 174
Radiographic Features 174
Diagnosis 174
Treatment 175
30 PAGET’S DISEASE 177
Clinical Features 177
Laboratory Features 177
Diagnosis 178
Treatment 178
31 OSTEOPOROSIS 179
Clinical Features 179
Laboratory Features 179
Diagnosis 180
Treatment 180
32 THERAPEUTIC INJECTIONS 183
Injection Technique 183
Injections in Specific Disorders 185
Rheumatoid Arthritis 185
Inflamed, painful joints 185
Extra-articular injections 185
Osteoarthritis 186
Controversy 186
Pain 186
Crystalline Arthropathy 186
Diagnosis and treatment 186
33 NONSTEROIDAL ANTI-INFLAMMATORY DRUGS 187
Adverse Effects 187
34 GLUCOCORTICOIDS 194
Mechanism of Action 194
Clinical Use 196
Adverse Events 196
New Developments 197
35 OPERATIVE TREATMENT 198
Hip 198
Knee 198
Foot and Ankle 199
Hand and Wrist 199
Elbow 200
Shoulder 200
Cervical Spine 200
36 COMPLEMENTARY AND ALTERNATIVE THERAPIES 202
INDEX 204