1. Sjogren's Syndrome

2. Sjögren's syndrome was in 1933 by
Swedish eye specialist Henrik Sjögren
• The condition causes antibodies to
attack the body's moisture-producing
glands, leading to dryness due to lack
of secretions.
• The illness most commonly affects the
eyes, mouth, salivary glands, lungs,
kidneys, skin and nervous system but
all organs of the body can be affected.
• In rare cases, complications from
Sjogren's syndrome can cause salivary
gland infections, kidney problems,
ulcers or pancreatitis.

4. • Venus Williams, 31, has spoken out about the autoimmune disease
that caused her to drop out of the U.S. Open yesterday minutes
before she was due to go on court.
• Miss Williams was diagnosed with Sjogren's syndrome two weeks ago
- after years of being misdiagnosed with other
conditions.
• 'For years I felt that I didn't have enough stamina and then,
four years ago, I felt like I was not getting enough air but I
was diagnosed with exercise-induced asthma,' she said.
•
'The medicine for asthma never worked.'
• Eventually, as her symptoms progressed doctors got to the bottom of
the problem. The athlete now suffers from fatigue, swollen joints, dry
eyes, dry mouth and heavy limbs.
By Daily Mail Reporter
UPDATED: 11:55 GMT, 2 September 2011

5. Sjögren's Syndrome
• A chronic, slowly progressive autoimmune
disease characterized by lymphocytic
infiltration of the exocrine glands resulting in
xerostomia and dry eyes.
• 1/3 have systemic manifestations
• Few develop lymphoma
• female-to-male ratio, 9:1

6. 2 Forms
• Primary Sjögren's
syndrome:
The disease presents
alone
• Secondary Sjögren's :
Asso. with other
autoimmune diseases
–RA
–SLE
–Scleroderma
–Mixed CT disease
–Primary biliary cirrhosis
–Vasculitis
–Chronic active hepatitis

7. Sjögren syndrome: Etiopathology
• Etiology -not well understood
• Findings suggest an ongoing interaction between
the innate and acquired immune systems
• Lymphocytic (T,B) infiltration of exocrine glands
+
• B lymphocyte hyper-reactivity

8. Sjögren syndrome: Etiopathology
Glandular epith cells express MHC class II mols
• Inherited susceptibility+ exo /endogenous antigens
• Trigger a self-perpetuating inflammatory response
• Continuing presence of active interferon pathways
suggest ongoing activation of the innate immune
system

9. Sjogren’s: Clinical Manifestation
The majority have symptoms related to
diminished lacrimal and salivary gland
function.
In most, the primary syndrome runs a slow and
benign course over 8 to 10 yrs.

10. Sjogren’s: Oral Manifestations
• Dryness (xerostomia), burning sensation
• Difficulty in swallowing dry food
• Inability to speak continuously
• Dental caries
• Problems in wearing dentures
Physical examination:
• Dry, reddened, sticky mucosa
• Atrophy of tongue
• Saliva not expressible/ cloudy
• Enlargement of salivary glands - 2/3 of primary SS
- Uncommon in secondary

11. Dryness of the mouth - xerostomia
Deep red tongue
Dental caries common

12. bilateral parotid enlargement- primary Sjögren
syndrome.

13. Sjögren’s syndrome and a L parotid mass.
Biopsy: B-cell lymphoma of mucosal lymphoid tissue

14. Sjogren’s: Ocular manifestations
Due to the destruction of corneal and bulbar
conjunctival epithelium, defined as
keratoconjunctivitis sicca
• A sandy/ gritty feeling under eyelids
• Burning
• Accumulation of thick secretions at inner canthi
• Decreased tearing, redness, itching,
• eye fatigue, increased photosensitivity

15. Sjogren’s: Clinical Manifestations
Other exocrine glands
• Resp : secretions dry nose, throat, and
xerotrachea
• GIT: Esophageal mucosal atrophy, atrophic
gastritis, and subclinical pancreatitis
• GUT: Dyspareunia and dry skin

16. Sjogren’s: Clinical Manifestation
Medications that cause similar symptoms
• Antidepressants
• Anticholinergics
• Beta blockers
• Diuretics
• Antihistamines
• Women on HRT
• Anxiety

17. Sjogren’s: Clinical Manifestations
Extra Glandular:
• Easy fatigability, low-grade fever, Raynaud‘s,
myalgias, and arthralgias
• Joints: Non-erosive arthritis
• Pulmonary : Dry cough (small airway disease)
• Renal: interstitial nephritis, acidosis and
nephrocalcinosis. Glomerulonephritis rare (SLE
overlaps).
• Vasculitis -purpura, recurrent urticaria, skin
ulcerations, GLN, and mononeuritis multiplex.
• SN hearing loss, any focal deficits

18. Sjogren’s: Clinical Manifestations
Glandular:
• Xerophthalmia
• Xerostomia
• Bilateral parotid swelling
Extraglandular:
• Arthralgias/arthritis
• Raynaud's phenomenon
• Lymphadenopathy
• Lung involvement
• Vasculitis
• Kidney involvement
• Liver involvement
• Lymphoma
• Splenomegaly
• Peripheral neuropathy
• Myositis

19. Sjogren’s: Investigations
Routine:
• Mild normochromic, normocytic anemia
• ESR rise- in 70%
Mouth:
• Sialometry
• sialography
• Imaging: Ultrasound, MRI or MR sialography of
salivary glands.
• salivary gland biopsy- focal lymphocytic
infiltrates.

20. Sjogren’s: Investigations
Eyes:
• Measurement of tear flow by schirmer‘s test
• Tear composition
• tear breakup time
• tear lysozyme content
• Slit-lamp examination after rose Bengal staining
• punctuate corneal ulcerations
• attached filaments of corneal epithelium

21. Schirmer test: Detects deficient tear
production in Sjögren syndrome

22. Sjogren’s Investigations: Must exclude
• Hepatitis C virus infection should be ruled out
since, apart from serologic tests, the
clinicopathologic picture is almost identical to
that of Sjögren's syndrome.
• HIV infection
• Sarcoidosis

23. Sjogren’s: Treatment
• Artificial tears, Rx -corneal ulcerations
• Avoid drugs that secretions (diuretics, anti HTs,
anticholinergics & antidepressants)
• Xerostomia: Best replacement - water
• Vaginal dryness: Propionic acid gels
• Secretagogues: Oral Pilocarpine / Cevimeline
• Arthralgias : HCQ
• RTA: Oral Soda bicarb
• Systemic vasculitis: Steroids,
immunosuppressives, M Abs
• High-grade lymphomas: Chemo (CHOP) + M Abs
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