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Flashcards in Sjogren's Syndrome Deck (16):
1

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.

2

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

3

What are organ systems that may be effected by SS

Lung (Pneumonitis)
-Kidney (Interstitial Nephritis)
-Heart
-Skin (Vasculitis)
-Neural (Central And Peripheral) -Hematopoietic system.

4

Primary SS is what?

keratoconjunctivitis sicca and xerostomia usually accompanied by hyposalivation

5

Secondary SS is what?

Primary SS plus:

-Rheumatoid Arthritis (RA),
-Systemic Lupus Erythematosus (SLE),
-Scleroderma.

6

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

7

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

8

Tests for Keratoconjunctivits Sicca

Rose-Bengal Test-detects damage of tissue from dryness

2. Schirmer’s Test-measures tear
production

3. Minor salivary gland biopsy-lymphoid
germinal center and interstitial plasma cells

9

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

10

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

11

DDx for SS

SLE (in younger women)

Sarcoidosis

Fibromyalgia with or without SS

Fatigue and Depression

Conditions characterized by parotid enlargement

12

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

13

Pharmacologic management for ocular dryness

Ocular dryness:

OTC: Eye drops such as Systane and Refresh™ Tears

Rx: 0.05% cyclosporine eye emulsion (Restasis™)

14

Pharmacologic management for oral dryness

Oral dryness:

OTC:
Biotene products™
sugar-free chewing gums,
sugar-free lozenges (Xylitol sweetened products are preferred)

15

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

16

Pharmacologic management of SS

Rx:
Cevimeline (Evoxac™) tid
Pilocarpine (Salagen™) 3-4 times/day

Both are muscarinic cholinergic agonists
Contraindications:

Affect cardiac conduction rate/heart rate
Avoid in patients with angina; Beta blockers
Avoid with glaucoma, severe asthma
Side effects:
-Sweating, Flushing, Polyuria
-Cause visual blurring and decreased night vision