- •Preface
- •Contents
- •Contributors
- •1 Introduction
- •1.1 Historical Background
- •1.2 Pitfalls in Diagnosis and Methodology
- •1.3 Methods to Assess Disease Activity
- •1.4 Summary
- •References
- •2.1 Introduction
- •2.4 Pearls of Wisdom
- •References
- •3.1 Background: Overall Approach to Patient Care
- •3.1.1 Pearl
- •3.1.2 Pearl
- •3.2 Diagnosis Criteria and Laboratory Tests
- •3.2.1 Myth
- •3.2.2 Pearl
- •3.2.3 Myth
- •3.2.4 Myth
- •3.2.5 Pearl
- •3.2.6 Pearl
- •3.2.7 Pearl
- •3.3 Myths and Pearls About Clinical Presentations
- •3.3.1 Pearl
- •3.3.2 Myth
- •3.3.3 Pearl
- •3.3.4 Pearl
- •3.3.5 Pearl
- •3.3.6 Pearl
- •3.3.7 Pearl
- •3.3.8 Pearl
- •3.3.9 Pearl
- •3.3.10 Pearl
- •3.3.11 Pearl
- •3.3.12 Pearl
- •3.3.13 Myth
- •3.3.14 Pearl
- •3.3.15 Pearl
- •3.3.16 Myth
- •3.4 Myths and Pearls About Pathogenesis
- •3.4.1 Myth
- •3.4.2 Pearl
- •3.4.3 Pearl
- •3.4.4 Myth
- •3.4.5 Pearl
- •3.5 Myths and Pearls About Treatment
- •3.5.1 Myth
- •3.5.2 Pearl
- •3.5.3 Pearl
- •3.5.4 Pearl
- •3.5.5 Pearl
- •3.5.6 Pearl
- •3.5.7 Pearl
- •References
- •4.1 Background and Overview
- •4.1.1 Need for Written Information
- •4.1.2 Use of Internet as a Method to Provide Information
- •4.1.3 Patient Access to Computers
- •4.1.4 Types of Information Supplied to Patients and Referring Physicians
- •4.2.1 Background: The Confusion Surrounding Criteria for Autoimmune Disorders
- •4.2.5 Criteria for Fibromyalgia
- •4.3 Laboratory Results for ANA Often Drive Clinical Diagnosis
- •4.5 Status of Biologic Drugs in SS Patients
- •4.6 Ocular Treatment
- •4.6.2 Blepharitis
- •4.7 Therapy of Oral Manifestations
- •4.7.1 Prevention of Dental Caries
- •4.7.2 Oral Candida Prevention and Treatment
- •4.8 Summary
- •References
- •5.1 Introduction
- •5.4 Outcome Measures in SS
- •5.4.1 Outcome Measures in SS: A Brief History
- •5.6 Outcome Measures in SS: The Italian Study
- •References
- •6.1 Introduction
- •6.2 Benign Lymphoepithelial Lesion in Salivary Glands
- •6.3.1.2 Ectopic Germinal Center Formation
- •6.3.1.3 Clinical Implications of Ectopic Germinal Center Formation
- •6.4 Late Breaking Update
- •References
- •7.1 Conventional Radiographs
- •7.1.1 Sialography
- •7.2 Computer Tomography
- •7.3 Ultrasound
- •7.4 Magnetic Resonance Imaging
- •7.5 Nuclear Medicine
- •7.5.1 Scintigraphy
- •7.6 Comparison of Nuclear Medicine, Ultrasound, and MRI
- •References
- •8.1 Introduction
- •8.2 Evidence Supporting a Genetic Component in SS
- •8.4 Lessons from SLE and Other Autoimmune Diseases
- •8.5 Genes Implicated in SS
- •8.7 Insights from Genomic and Proteomic Studies
- •8.8 Conclusion
- •References
- •9.1 Introduction
- •9.2.4 Antibodies to Nuclear Protein NA14
- •9.3.1 Initiation Phase
- •9.3.2 Recognition Phase
- •9.3.3 Establishment Phase: Autoreactive T and B Lymphocytes Dysregulation and Aberrant Cytokines Production
- •9.3.5 Effector Phase
- •References
- •10.1 Introduction
- •10.2.1 Ro/La RNP Particles
- •10.2.3 The Ro60 Autoantigen
- •10.2.4 The Ro52 Autoantigen
- •10.2.5 The Multifunctional Chaperone Calreticulin
- •10.4.2 Early Epitope Recognition in Autoimmune Diseases and Epitope Spreading
- •References
- •11.2 Acinar Cell
- •11.3 Neuropeptides
- •11.3.1 Acinotrophic Neurogenic Stimuli
- •11.4 Sex Steroids
- •11.4.1 Steroidogenesis in Adrenal Glands
- •11.4.2 Regulation of the Adrenal Steroidogenesis
- •11.4.4 Peripheral Intracrine Synthesis of Sex Steroids
- •11.4.5 Intracrine Sex Steroids Production in pSS and sSS
- •11.4.7 Putative Mechanism of Action of the Intracrine Processing Defect
- •11.5.1 General Histopathology
- •11.5.2 T Lymphocytes
- •11.5.3 B Lymphocytes
- •11.5.4 Chemokines
- •11.5.5 Adhesion Molecules
- •11.5.6 Cytokines
- •References
- •12.1 Background
- •12.2 Incidence, Symptomatic Presentation, and Impact on Quality of Life
- •12.3 Diagnostic Screening Examination
- •12.4 Overview of Dry Eye Management
- •12.4.1 Dry Eyes Deserve Respect and Careful Monitoring
- •12.4.2 Four Levels of Severity Differentiation
- •12.4.2.1 Level 1
- •12.4.2.2 Level 2
- •12.4.2.3 Level 3
- •12.4.2.4 Level 4
- •12.5.2 General Guidelines for the Dry Eye Patient
- •12.6 Additional Types of Therapy
- •12.7 Moisture Preservation and Oral Medications
- •12.7.2 Punctal Plugs
- •12.8 Oral Medications and Supplements
- •12.8.1 Dietary Fatty acids (Flaxseed Oil) and Dry Eyes
- •12.8.2 Oral Medications
- •12.9 Complications Associated with Ophthalmologic Cosmetic Procedures
- •12.10 Summary
- •References
- •13.1 Introduction
- •13.2 The Lacrimal Functional Unit (LFU)
- •13.3 The General Role of the LFU in Normal and Pathological Situation
- •13.4 Innervation of the Lacrimal Functional Unit
- •13.5 Efferent Structures
- •13.5.1 Lacrimal Glands
- •13.5.2 Goblet Cells
- •13.5.3 Meibomian Glands
- •13.6 Maintenance of the Lacrimal Functional Unit
- •13.6.1 Hormonal
- •13.6.2 Immunological
- •13.8 The Normal Ocular Surface Environment
- •13.9 The Makeup of the Tear Film
- •13.9.1 Hydrated Mucin Gel
- •13.9.3 Aqueous Components
- •13.10 The Pathophysiology of Dry Eye
- •13.10.1 Loss of Hormonal Support
- •13.10.2.1 Afferent Arm
- •13.10.2.2 Efferent Arm
- •13.11 Loss of Ocular Surface Homeostasis
- •13.11.1 Alterations of the Mucin, Lipid, and Aqueous Composition
- •13.11.2 Mucins
- •13.11.3 Lipids
- •13.12 The Ocular Surface Immunosuppressive Environment
- •13.14 Late-Breaking Additions
- •References
- •14.1 Saliva in Oral Health and Disease
- •14.1.1 Saliva in Dental and Mucosal Defense
- •14.1.2 Assessment of Oral Dryness
- •14.1.2.2 Objective Measurements of Hyposalivation
- •14.2 Saliva as a Diagnostic Fluid
- •14.2.1 Biomarker Analyses in Saliva
- •14.3 Complications of Oral Dryness
- •14.3.1 Management of Xerostomia
- •14.3.2 Caries Preventive Measures
- •14.3.2.3 Dietary Advice
- •14.3.2.4 The Time Factor
- •References
- •15.1.1 Endothelial Cells
- •15.1.2 Epithelial Cells
- •15.1.3 T cells
- •15.1.4 B cells
- •15.2 Mechanisms Mediating Salivary Gland Dysfunction
- •15.2.1 Acinar Cell Innervation and Humoral Immunity
- •15.2.3 Fluid Movement in the Salivary Glands and Aquaporins
- •15.3.1 Environmental Factors
- •15.3.2 Secondary Signals
- •15.3.3 Apoptosis, Autoantigens, and Potential Danger Signals in the Salivary Glands
- •15.3.4 Immunoregulation
- •15.3.5 B-cell-Activating Factor
- •15.3.6 Hormones
- •15.3.7 Microchimerism
- •References
- •16.1 Introduction
- •16.2 Diagnosis
- •16.3 Head and Neck Manifestations
- •16.3.1 Ophthalmic
- •16.3.2 Oral
- •16.3.3 Otologic
- •16.3.4 Rhinologic
- •16.3.5 Laryngeal
- •16.3.6 Esophageal
- •16.3.7 Thyroid
- •16.3.8 Neurological
- •16.4 Treatment
- •16.5 Conclusion
- •16.6 Patient Handout
- •References
- •17.1 Introduction
- •17.2 Cutaneous/Dermatologic Manifestations
- •17.4 Endocrinopathic/Pancreatic Manifestations
- •17.4.1 Hypothyroidism
- •17.4.2 Adrenal
- •17.4.3 Pancreas
- •17.5 Pulmonary Manifestations
- •17.5.1 Interstitial Pneumonitis
- •17.6.1 Pericarditis
- •17.6.2 Autonomic Manifestations
- •17.6.3 Congenital Heart Block
- •17.6.4 Accelerated Atherosclerosis
- •17.7 Gastrointestinal Manifestations
- •17.8 Hepatic and Pancreatic Manifestations
- •17.9 Renal/Urological Manifestations
- •17.10 Hematologic Manifestations
- •17.11 Obstetrical/Gynecological Manifestations
- •17.12 Vasculitis
- •17.12.1 CNS Arteritis in the SS Patient
- •17.13 Differential Diagnosis of Extraglandular Manifestations of SS
- •17.13.1 Medications and Other Metabolic Disorders
- •17.14 Manifestations and Differential Diagnosis in the Pediatric Population
- •17.15 Summary
- •17.16 Late-Breaking Updates
- •References
- •18.1 Introduction
- •18.2 Treatment and Management of Cutaneous Manifestations
- •18.2.1 Treatment of Dry Skin
- •18.3 Arthralgia/Arthritis
- •18.4.1 Chronic Cough
- •18.5 Renal Manifestations
- •18.5.1 Interstitial Nephritis
- •18.5.1.1 Glomerular Disease
- •18.6 Gastrointestinal Manifestations
- •18.6.1 Mesenteric Vasculitis
- •18.6.2 Primary Biliary Cirrhosis
- •18.7 Urologic
- •18.8 Therapeutic Management of Obstetrical/Gynecological Manifestations
- •18.9 Special Precautions at the Time of Surgery
- •18.10 Vaccinations in the SS Patient
- •18.11 Summary
- •18.12 Late-Breaking Updates
- •References
- •19.1 Introduction
- •19.3.1 Fatigue
- •19.3.2 Musculoskeletal
- •19.3.4 Gastrointestinal Manifestations
- •19.3.5 Liver Involvement
- •19.3.6 Lung Involvement
- •19.3.7 Kidney Involvement
- •19.3.8 Neurologic Involvement
- •19.3.9 Hematologic Involvement
- •19.4 Conclusions
- •References
- •20.1 Introduction
- •20.2 Diagnosis
- •20.3 Staging and Evaluation of Treatment Response
- •20.4 Treatment
- •20.5 Summary/Pearls
- •References
- •21.1 Introduction
- •21.2 What Is Fatigue?
- •21.3 Potential Causes of Fatigue in pSS
- •21.3.1 Biological
- •21.3.1.1 Cytokines
- •21.3.1.2 Neuroendocrine
- •21.3.1.3 Sleep
- •21.3.2 Psychosocial
- •21.3.2.1 Depression
- •21.3.2.2 Fibromyalgia
- •21.4 Measurement of Fatigue and Other Extraglandular Symptoms
- •21.6 Potential Approaches to Treatment of Fatigue and Other Extraglandular Symptoms
- •21.7 Measurement of Dryness (Sicca) Symptoms
- •21.8 Data from Existing Clinical Studies Addressing Dryness in pSS
- •21.9 Conclusion: Clinical Trial Outcomes
- •References
- •22.1 Introduction
- •22.2 Clinical Evaluation of Neurological Findings in SS
- •22.3.1 Role of Cell-Mediated Immunity
- •22.3.2 The Role of Antibodies Associated with Neurological Manifestations of SS
- •22.4 Investigations
- •22.4.1 Neurophysiology
- •22.4.2 Autonomic Studies
- •22.4.3 MR Imaging of the Spinal Cord
- •22.5 Peripheral Clinical Manifestations
- •22.6 Painful Sensory Neuropathies
- •22.6.1 Differential Diagnosis
- •22.7 Sensory Ataxic Neuropathy
- •22.7.1 Differential Diagnosis
- •22.8 Neuromuscular Weakness
- •22.8.1 Differential Diagnosis
- •22.9 Neuromuscular Pain
- •22.9.1 Differential Diagnosis
- •22.10 Autonomic Neuropathy
- •22.10.1 Differential Diagnosis
- •22.11 Trigeminal Neuropathy and Other Cranial Neuropathies
- •22.12 Central Nervous System Manifestations
- •22.12.2 Cognitive Impairment
- •22.12.3 Movement Disorders
- •22.12.4 Aseptic meningitis and Meningoencephalitis
- •22.12.5 Other Neurological Disorders
- •22.13 Investigations of Central Nervous System Manifestations
- •22.13.1 Serology
- •22.13.2 Spinal Fluid
- •22.13.4 Nuclear Brain Imaging Studies
- •22.13.5 Cerebral Angiography
- •22.14 The Puzzling Neurological Manifestations of Fibromyalgia
- •22.15 Interpretation of ANA in the Patient with Neurological Symptoms
- •22.16 Treatment
- •22.16.1 Peripheral Nervous System Treatment: Overview
- •22.16.2 Painful Sensory Neuropathies
- •22.16.3 Ataxic Neuropathy
- •22.16.4 Motor and Sensory Neuropathies
- •22.16.5 Central Nervous System Treatment
- •22.16.6 Side Effects of Immunosuppressive Therapy
- •22.17 Summary of Special Points to Neurologists
- •22.17.3 Relationship of Neurological Symptoms to Sicca Manifestations
- •22.18 Summary for Rheumatologists
- •References
- •23.1 Introduction
- •23.3.1 Labial Minor Salivary Gland Biopsy
- •23.3.2 Sialography
- •23.4 The Application of a Bite Guard
- •References
- •24.1 Introduction
- •24.2 How to Provide the Essential Tear Components to the Ocular Surface
- •24.3 Use of Autologous Serum Eye Drops for the Treatment of Dry Eye
- •24.4 Ongoing Research with Autologous Serum Eye Drops
- •24.5 Preparation of Autologous Serum Eye Drops
- •24.8 Conclusion
- •References
- •References
- •27.1 A Disease of Antiquity in Ancient China
- •References
- •References
- •References
- •30.1 Introduction
- •30.2 Evaluation of Systemic Features of Primary SS
- •30.2.4 Comparisons of Systemic Disease Activity Scores
- •30.3.1 The SSI: Sicca Symptoms Inventory
- •30.4 Conclusion
- •References
- •31.1 Clinical Practice Guidelines
- •31.2 Clinical Trials Consortium
- •31.3 Professional Education and Awareness
- •31.4.1 Rheumatology Working Group
- •31.4.2 Ocular Working Group
- •31.4.3 Oral Working Group
- •31.4.5 Facilitator for Both Initiatives
- •32.1 Introduction
- •32.2 For Which Patients Should Biological Therapy Be Considered?
- •32.7 BAFF Inhibition
- •32.8 Interferon Inhibition
- •32.9 Gene Therapy
- •32.10 Other Targets for Biologic Therapy
- •32.11 Conclusions and Future Directions
- •References
- •33.1 Overview of the Pathogenesis of pSS
- •33.1.1 Initial Steps
- •33.1.1.1 Breach of Self-tolerance
- •33.1.1.2 Activation of Innate Immunity and Interferon Pathways
- •33.1.1.4 Regulation of BAFF Secretion
- •33.1.1.6 Other Cytokines, Chemokines, and Adhesion Molecules Are Involved in the Pathogenesis of the Disease
- •33.1.3 Glandular Hypofunction Rather Than Glandular Destruction
- •33.2 Emerging Therapies
- •33.2.1 Prerequisite for the Development of New Drugs in pSS
- •33.2.1.1 Disease Activity Score
- •33.2.1.2 Selection of Patients
- •33.2.3.1 Inhibition of the Triggering Factors of IFN Activation
- •33.2.3.2 IFN Blockade
- •33.2.3.3 Antagonists of BAFF and APRIL
- •33.2.3.4 B-cell Depletion
- •33.2.3.5 Other B-cell-Targeted Therapy: Other Anti-CD20 and Anti-CD22
- •33.3 Other Therapeutic Perspectives
- •33.3.1 Inhibition of Other Cytokines and Chemokines
- •33.3.3 Gene Therapy
- •33.4 Conclusion
- •References
- •34.1 Introduction
- •34.5 Conclusion
- •References
- •Index
20 Lymphoproliferation and Lymphoma in Sjögren’s Syndrome |
353 |
|
|
¥In patients with asymptomatic MALT lymphoma and low SS disease activity treatment can be deferred. In these patients watchful waiting is indicated.
¥Response evaluation of treatment should include both lymphoma and SS parameters.
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Fatigue, Dryness, and Quality |
21 |
of Life as Clinical Trial Outcomes |
in Primary Sjögren’s Syndrome
Simon J. Bowman
Abstract
Although dryness symptoms are regarded as the deÞning features of SjšgrenÕs syndrome (SS), fatigue is also a common and often disabling symptom for many patients. The biologic mechanisms of fatigue in SS or in other rheumatic diseases are poorly understood. Low mood associated with having a chronic disease plays a part. Attempts to show correlations between fatigue levels and the levels of cytokines or other biologic parameters or with clinical measures of Òdisease activityÓ have not generated a clear answer. Much fatigue research has focused on the measurement and classiÞcation of fatigue features and a number of fatigue questionnaires have been developed. Although we can now, therefore, ÒmeasureÓ fatigue levels, there is no proven therapy for fatigue in SS, and considerable debate as to whether a biological or a psychosocial approach is more appropriate. Our understanding of dryness symptoms has been complicated by the lack of correlation between the level of symptoms and the degree of objective dryness measured by standard tests in groups of patients. This has generated a lack of certainty as to what is the goal of therapy in SSÑimprovement in symptoms or in physical measures of tear and/or salivary ßow or histological features in the exocrine glands, or some combination of all of these. What is clear from a number of studies is that patients with SS have reduced levels of health-related quality of life comparable to that of rheumatoid arthritis or systemic lupus erythematosus. Despite these limitations, however, there has been considerable progress in symptom assessment, and this should lay the groundwork for clinical trials of therapeutic agents over the next few years.
Keywords
SjšgrenÕs syndrome ¥ Fatigue ¥ Patient symptoms ¥ Clinical trials
S.J. Bowman ( )
Department of Rheumatology, University Hospitals Birmingham, Birmingham, England
e-mail: simon.bowman@uhb.nhs.uk
R.I. Fox, C.M. Fox (eds.), Sjögren’s Syndrome, DOI 10.1007/978-1-60327-957-4_21, |
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