Sjogren's Syndrome: A Clinical Handbook

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Sjögren\'s Syndrome: A Clinical Handbook
Contributors
Dedication
Preface
1. Sjögren\'s syndrome: past, present, and future
	Perspective
	Brief review of the past—as “we only stand on the shoulders of past giants”
		Early history
		The pioneers
		Looking backward at the SS-A and SS-B autoantigens
	Questions for the present
		Classification and diagnosis
		Clinical trials
		Etiology and pathogenesis
		Clinical challenges
	Looking forward to to the future
		Early diagnosis
		Precision medicine and unmet needs
		Clinical trials
		Interdisciplinary care and future directions
	Conclusions
	References
2. Diagnosis and evaluation of Sjögren\'s syndrome
	Challenge of diagnosis
	Clinical presentations
	Approach to diagnosis in the clinical setting
	Objective assessment of dry eyes
	Objective assessment of salivary gland involvement
	Proof of autoimmunity
	Evolution of the classification criteria
	Practical diagnosis
	Differential diagnosis
	Further evaluation
	Conclusions
	References
3. Oral manifestations and management in Sjögren\'s syndrome
	Importance of saliva
		Causes of salivary hypofunction
	Major complications and management
		Treatment of xerostomia
		Caries and the loss of noncarious tooth surfaces
		Overview of caries prevention
			Fluoride
			Remineralizing strategies
			Antimicrobial agents
			Salivary stimulation
		Tooth surface loss
	Salivary gland swelling
	Salivary gland obstruction
		Mucus plugs
		Sialolithiasis
	Infections
		Bacterial sialadenitis
		Oral candidiasis
			Prevention and treatment of oral candidiasis
	Mucosal lesions
		Associated autoimmune oral lesions
		Glossodynia
	Conclusions
	References
4. Diagnosis and management of Sjögren\'s syndrome related dry eye
	Introduction
	Diagnosis
		Symptoms
		Ocular examination
	Treatment of dry eye and meibomian gland dysfunction
		Overview
		Environmental/lifestyle modifications
		Lubrication
		Oral secretagogues
		Topical anti-inflammatory medications
		Conservation of tears
		Management of severe dry eye
		Treatment of eyelid disease
	Special considerations
		Contact lenses
		Corneal neuropathy, neuropathic pain, and dry eye
		Ocular refractive surgery
		Cataract surgery
	Conclusions
	References
5. Approach to children with Sjögren’s syndrome
	Introduction
	Clinical manifestations
		Glandular manifestations
			Parotitis
			Oral/ocular sicca symptoms
			Gynecologic manifestations
		Extraglandular manifestations
			Constitutional symptoms
			Neurologic manifestations
			Renal manifestations
			Cutaneous manifestations
			Gastrointestinal manifestations
			Musculoskeletal manifestations
			Pulmonary manifestations
			Hematologic manifestations
	Evaluation
		Challenge of diagnosis
		Adult classification criteria in pediatric Sjögren\'s syndrome
		Diagnostic algorithm
		Differential diagnosis of childhood Sjögren\'s syndrome
		Salivary gland histopathologic examination in children
		Making the diagnosis
	Management
		Recurrent parotitis
		Dry mouth
		Keratoconjunctivitis sicca
		Other organs
		Preventive therapy
		Long-term prognosis
	Conclusions
	References
6. Extraglandular abnormalities in Sjögren\'s syndrome
	Fatigue and fever
	Musculoskeletal manifestations
	Pulmonary manifestations
	Renal manifestations
		Tubulointerstitial nephritis
		Renal tubular acidosis
		Other renal manifestations
	Gastrointestinal manifestations
		Esophageal/gastric
		Intestinal
		Hepatic
	Hematologic abnormalities
		Cytopenias
		Immunoglobulin abnormalities
	Dermatologic manifestations
	Conclusions
	References
7. Vasculitis in Sjögren\'s syndrome
	Definition and classification
	Clinical manifestations
		Cutaneous vasculitis
			Cutaneous vasculitis as a prognostic indicator
			Cutaneous vasculitis patient subsets
		Peripheral nervous system involvement
		Central nervous system involvement
		Renal involvement
		Other organs
		Antineutrophil cytoplasmic antibody-associated vasculitis and Sjögren syndrome overlap
		European Sjögren\'s syndrome disease activity index score and vasculitis
	Treatment
	Conclusions
	References
8. Lymphoproliferative disease in Sjögren\'s syndrome
	Introduction
	Epidemiology
	Lymphoma in secondary Sjögren\'s syndrome
	Lymphoma subtypes
	Multiple myeloma
	Risk factors and prediction models
	Pathology and pathogenesis
	Clinical presentation
	Imaging
	Diagnostic evaluation
	Lymphoma staging
	Treatment of lymphoma
	Prognosis
	Screening protocol for lymphoma
	Conclusions
	References
9. Sjögren\'s syndrome: peripheral and autonomic nervous system involvement
	Epidemiology
		Peripheral neuropathy
		Autonomic neuropathies
	Clinical presentation
		Small fiber neuropathy
		Sensory ataxic neuronopathy
		Axonal sensory and sensorimotor polyneuropathy
		Cranial neuropathy
		Mononeuritis multiplex
		Other neuropathies in Sjögren\'s syndrome
			Chronic inflammatory demyelinating polyneuropathy
			Radiculoneuropathy
			Motor neuron disease
	Diagnostic evaluation
		Nerve conduction studies and electromyography
			General considerations
			Sensory nerve integrity
			Motor neuron integrity
			Nerve injury
			Electromyography
			Electrophysiologic patterns of peripheral neuropathy
		Autonomic testing
			Background
			Autonomic diagnosis
			Head-up tilt table test
			Postural orthostatic tachycardia syndrome
			Sweat testing
		Biopsies
			Intraepidermal nerve fiber analysis
			Cutaneous nerve biopsy
	Lessons from clinicopathologic studies
		Microscopic vasculitis in peripheral nerve biopsies
		Pathophysiologic role of the sensory neuron
	Management and prognosis
		Treatment of peripheral neuropathies
			Conservative measures
			Corticosteroids
			Cyclophosphamide
			Other immunosuppressants
			Plasma exchange
			Immunoglobulin therapy
			Biological therapy
			Autonomic involvement
	Conclusions
	References
10. Diagnosis and management of central nervous system Sjögren\'s syndrome
	Prevalence
	Pathophysiology
	Major clinical syndromes
		Focal/multifocal disease
		Demyelinating syndromes
			Neuromyelitis optica
			Multiple sclerosis
		Diffuse disease
	Neurodiagnostic studies
	Treatment
	Conclusions
	References
11. Treatment of Sjögren\'s syndrome internal organ manifestations and constitutional symptoms
	Constitutional symptoms
	Rheumatologic manifestations
		Inflammatory musculoskeletal pain
		Management
		Muscular involvement
		Fibromyalgia
	Respiratory manifestations
		Upper respiratory tract and airway disease
		Interstitial lung disease
		Treatment of nonspecific interstitial pneumonitis
		Treatment of other Sjögren\'s syndrome interstitial lung disease
		Airway disease
		Pulmonary lymphoma
		Pulmonary amyloidosis
		Pleural involvement
		Pulmonary arterial hypertension
	Management of gastrointestinal manifestations
		Mouth
		Esophagus
		Gastric involvement
		Liver
		Pancreas
		Small and large intestines
	Management of renal manifestations
		Renal tubular dysfunction
		Glomerulonephritis
	Conclusions
	References
12. Integrative rheumatology: complementary and alternative medicine for the management of Sjögren\'s syndrome
	Introduction
	Dietary modifications
	Nutritional supplements
		Vitamin A
		Vitamin C
		Iron, vitamin B12, and folic acid
		Vitamin D
		Coenzyme Q10
		Polyunsaturated fatty acids
		Omega-6 fatty acids: linoleic acid and γ-linolenic acid
		Green tea catechins
		Curcumin
		Ginger
	Acupuncture
	Guided imagery
	Psychodynamic group therapy
	Cognitive behavior therapy
	Sleep disorders
	Nonpharmacologic therapies for management of fibromyalgia
	Conclusions
	References
13. Perioperative management of patients with Sjögren\'s syndrome
	Perioperative management specific to Sjögren\'s syndrome
	Managing sicca preoperatively
	Managing extraglandular manifestations
	Postoperative care
	Risk of infection after surgery
	Management of immunosuppression
	Conventional disease-modifying antirheumatic drugs
	Biologic and targeted therapies
	Glucocorticoids
	Key messages for immunosuppression
	Conclusions
	References
14. Utility and safety of vaccines in Sjögren\'s syndrome
	Vaccination: general considerations
	History
	Childhood vaccinations
	Adult vaccinations
		Influenza vaccination
		Pneumococcal vaccination
		Zoster vaccination
		Papillomavirus vaccination
		Hepatitis B vaccination
		Other vaccines
	Travel
	Effect of immunosuppressive medications on vaccine effectiveness
	Vaccination as a potential trigger of disease
	Vaccines as treatment
	Conclusions
	References
15. The cost of Sjögren\'s syndrome and the value of patient resources and support in management
	Introduction
	Burden of illness
	Disability
	Stress and Sjögren\'s syndrome
	Support and availability of resources linked to better health
	The Sjögren\'s Foundation: creating a community of support
		Support and communication
		Patient education
		Engaging the professional community
	Conclusions
	References
Index
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