Sjogren's Syndrome Flashcards
Definition of Sjögren’s Syndrome (SS)
A chronic, systemic autoimmune inflammatory disorder characterized by lymphocytic infiltration of exocrine glands, especially the lacrimal and salivary glands.
What is xerostomia and keratoconjunctivitis sicca
Xerostomia
Complaint of a dry mouth
Keratoconjunctivitis sicca
Dry eyes
This is due to lymphocytic infiltrates and destruction of salivary and lacrimal glands and systemic production of autoantibodies
What are organ systems that may be effected by SS
Lung (Pneumonitis)
- Kidney (Interstitial Nephritis) - Heart - Skin (Vasculitis) - Neural (Central And Peripheral) -Hematopoietic system.
Primary SS is what?
keratoconjunctivitis sicca and xerostomia usually accompanied by hyposalivation
Secondary SS is what?
Primary SS plus:
- Rheumatoid Arthritis (RA), - Systemic Lupus Erythematosus (SLE), - Scleroderma.
Keratoconjunctivitis Sicca Symptoms
Description:
-Reduction in the aqueous component of tears
Symptoms:
-Insidious onset over a period of years
-Vary in severity, but include dryness and foreign body sensation that may be worse in the evening
-Described as gritty or sandy feeling –more so than only a feeling of dryness
Keratoconjunctivitis Sicca clinical exam
Clinical Exam:
Irritation, itching, photophobia Thick rope-like strands at the inner canthus (mucous filaments) especially on awakening -Dilated vessels of the bulbar conjunctiva -Dullness of conjunctiva and cornea -Lacrimal gland enlargement sometimes
Complications of ocular dryness:
-corneal ulceration and infection of eyelids
Tests for Keratoconjunctivits Sicca
Rose-Bengal Test-detects damage of tissue from dryness
- Schirmer’s Test-measures tear
production - Minor salivary gland biopsy-lymphoid
germinal center and interstitial plasma cells
Epidemiology and Etiology of SS
World-wide disease
Incidence 4/100,000
Prevalence 0.5 –3% of population, depending on criteria used
Predominately women (female/male ratio 9:1)
Median onset at 35-50 years of age
Etiology (unknown)
Combination of genetic predisposition and environmental factors or trigger
Prognosis for SS
Risk of developing lymphoma 3-5% with primary SS
Standardized mortality ratio for primary SS is not significantly increased when compared to gender and age matched segments of normal population
Hypocomplementemia appears to be an independent risk factor for premature death.
Adverse prognosticators: glomerulonephritis, purpura, low C4 complement
DDx for SS
SLE (in younger women)
Sarcoidosis
Fibromyalgia with or without SS
Fatigue and Depression
Conditions characterized by parotid enlargement
Clinical Features for SS
Non-erosive arthralgia 60-70%
Raynaud’s 35-40%
Lymphadenopathy 15-20%
Vasculitis 5-10%
Pulmonary involvement 10-20%
Renal 10-15 %
Hepatic 5-10%
Neuropathy 2-5%
Myositis 1-2%
Lymphoma 3-4%
parotid gland swelling
Pharmacologic management for ocular dryness
Ocular dryness:
OTC: Eye drops such as Systane and Refresh™ Tears Rx: 0.05% cyclosporine eye emulsion (Restasis™)
Pharmacologic management for oral dryness
Oral dryness:
OTC:
Biotene products™
sugar-free chewing gums,
sugar-free lozenges (Xylitol sweetened products are preferred)
What is SS associated with
Association with autoantibodies SSA, and SSB (Ro/La) autoantibodies
Association HLA-DR 4 haplotype
Can be associated with other autoimmune conditions. Lupus RA PSS PM/DM