Sjogren's syndrome is an autoimmune disease characterized by lymphocytic infiltration and destruction of the salivary and lacrimal glands, resulting in dry mouth and eyes. It was first described in 1933 by Swedish ophthalmologist Henrik Sjogren. The disease has a 9:1 female predominance and usually occurs in the 4th-6th decades of life. It is believed to have genetic, environmental, and hormonal components. Diagnosis involves tests for dry mouth and eyes like Schirmer's test, as well as blood tests for autoantibodies. Treatment focuses on managing dryness symptoms with artificial tears and saliva, along with medications to reduce inflammation and treat complications.

1. By
Farshid Mokhberi
Shahid Beheshti University Of Medical Sciences & Health services

2. Definition
 Sjogren’s syndrome is a late onset chronic systemic
autoimmune disease characterized by lymphocytic
infiltration and destruction of the salivary and
lacrimal glands resulting in dry mouth(xerostomia)
and dryness and atrophy of conjunctiva and cornea
(keratoconjunctivits sicca).
 Swedish Ophthalmologist Henrik Sjogren who first
described it (1933)

3. ETIOLOGY
 Female : Male = 9 : 1
 4/5/6th decade
 Autoimmune ; HLA-B8 / DR3 ,although DR4 is more
closely associated with syndrome occuring with raynaud’s
phenomenon
Pathogenesis of Sjogren’s syndrome is believed to be
multifactorial.
Known to be autoimmune, but studies suggest that the
disease process has genetic, environmental(EBV/HCV) and
hormonal(associated with high prevalence in women,esp
estrogen) components

5.  Lymphocyte and plasma cell infiltration  Auto-
antibody production (to ‘Ro’)
 Connective tissue proliferation
 Glandular cell apoptosis  atrophy of glandular
structures in affected tissues (salivary glands,
sebaceous glands, sweat glands)

6. Clinical Types
 PRIMARY : Alone
 SECONDARY : associated underlying connective
tissue diseases (RA / SLE / Scleroderma )
 SICCA SYNDROME : Xerophthalmia/ Xerostomia
/Internal Organ / Bone Inv
 This disease is caused by an immune-mediated
inflammation of salivary,lacrimal and sweat glands as
Sicca Syndrome or with internal organ involvement.

7. Clinical Features
 Glandular manifestation
 Dry mouth (Xerostomia) due to decreased production of
saliva by salivary glands
Cracked, peeling and atrophic appearance of the lips. Dry and fissured tongue

8. Teeth :
 multilpe carries and early loss

9.  Chronic oral candidiasis is frequent.
 Parotid Gland Enlargement

10.  Lacrimal Gland Involvement – Dryness of eyes causes
keratoconjuctivitis sicca.
 Burning itchy sensation in the eyes.

11. Skin manifestation
 Xeroderma, pruritus and scaling
 Annular erythema, Papular Erythema
 Raynaud’s syndrome
 Hyperglobulinemic Purpura
 Vitiligo
 Sweating abnormalities
 Cutaneous Amyloidosis
 Alopecia—diffuse and generalized

12. OTHER MANIFESTATIONS
Joint symptoms: Arthralgia and arthritis
Myalgia and myositis
ENT : Sinusitis / Hearing Loss
GI : GERD
Respiratory: Interstitial pneumonitis, pulmonary
fibrosis and pulmonary hypertension
Nephrology : Interstitial nephritis, Renal Tubular
Acidosis
Neurology : migraine, neuropathies, cerebral vasculitis

13. Sjogren’s Syndrome Associations

14. Diagnosis
 hypergammaglobulinemia
(IgG>IgA>IgM),ANA(>50%) elevated total protein
and sedimentation rate.
 Anti-Ro and Anti-La Antibodies occur in
approximately 60% of patients with Sjogren's
syndrome
 Histolgy of skin shows an absence of sebaceous
glands and decrease in the sweat glands.

15. Biopsy of labial salivary glands
Ly
lymphocytic and plasma cells infiltrate
Two excretory ducts and 3 mucous
salivary gland acini are seen

16. SCHIRMER’S TEST
German Ophthalmologist Otto Schirmer
 determines whether the eye produces enough tears
to keep it moist
 This test is used when a person experiences very
dry eyes or excessive watering of the eyes

17. SCHIRMER’S TEST
1. Normal which is ≥15 mm wetting of the paper after
5 minutes
2. Mild which is 14-9 mm wetting of the paper after 5
minutes
3. Moderate which is 8-4 mm wetting of the paper
after 5 minutes
4. Severe which is <4 mm wetting of the paper after 5
minutes.

19. Rose Bengal

20. Revised classification criteria for Sjogren’s Syndrome
1-Ocular symptoms
• Dry eyes for more than 3 months
• Sensation of sand or gravel in the eyes
• Need for tear substitutes more than 3 times a day
2-Oral Symptoms
• Dry mouth for more than 3 months
• Recurrently or Persistently swollen salivary glands
• Need liquids to swallow dry food
3-Ocular Signs
• Schirmer’s test
• Rose bangal test
4-Salivary gland involvement
5- Autoantibodies
Anti Ro and Anti La

21. Criteria
For primary
 In patients without any potentially associated disease,
primary SS may be defined as follows:
 a. The presence of any four of the six items is
indicative of primary SS, as long as either item 4
(Histopathology) or 6 (Serology) is positive.
 b. The presence of any three of the four objective
criteria items (that is, items 3, 4, 5)
For secondary
 In patients with a potentially associated disease, the
presence of item 1 or item 2 plus any two from among
items 3, 4, and 5 may be considered as indicative of
secondary

22. Treatment
 Symptomatic treatment for dryness of eyes – by
lubricating agents such as 0.5% methylcellulose eye
drops for 4-5 times daily.
 Cyclosporine ocular drops
 Bromhexine 16 mg TDS has been found to increase the
lacrimal secretion.
 Artificial saliva and cyclosporin(2.5-5mg/kg), for
xerostomia.
 Routine dental care
 Steam inhalation may help dryness of the respiratory
tract.

23.  Candidiasis – topical nystatin 3times/day for a week
 Systemic – ketoconazole 200-400mg/day or fluconazole
50-100 mg/day or itraconazole -100mg/day for 2 weeks
 Systemic steroids are effective in reducing parotid
swelling
 hydroxychloroquine 200 mg daily is useful

24. Refrences
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25. Thanks For Your Attention