sjogren's syndrome by Dr.Aakanksha singh,Resident SKIN & VD

1. SJOGREN’S SYNDROME
Dr.Aakanksha Singh

2.  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 Sjögren who first
described it (1933) ;

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

4. 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
 Antibodies to the Ro antigen occur in excess in relatives of
pts with sjogren’s syndrome.

5. Pathogenesis of Sjögren’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

7.  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)
 Secondary changes – oedema of conjuctiva

8. C/F
 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

9.  Teeth – multilpe carries and early loss

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

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

12. SKIN MANIFESTATIONS (50%)
Xeroderma, pruritus and scaling
Annular erythema, Papular Erythema including
Sweet’s-like lesions.
Raynaud’s syndrome
Hyperglobulinemic Purpura
Vitiligo
Sweating abnormalities
Cutaneous Amyloidosis
Alopecia—diffuse and generalized

13. OTHER MANIFESTATIONS
Joint symptoms - Arthralgia and arthritis
Myalgia and myositis
ENT : Sinusitis / Hearing Loss
GI : GERD
Resp : Interstitial pneumonitis, pulmonary fibrosis and pulmonary hypertension
Nephro : Interstitial nephritis, Renal Tubular Acidosis
Neuro : migraine, neuropathies, cerebral vasculitis

14. Sjogren’s Syndrome – Associations

15. Diagnosis
 SS patients of both primary and secondary Sjögren’s
syndrome have marked 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 Sjögren's syndrome
 Histolgy of skin shows an absence of sebaceous
glands and decrease in the sweat glands.

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

17. 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

18. SCHIRMER’S TEST
 Schirmer's test places a small strip of filter paper inside the lower eyelids
(conjunctival sac). The eyes are closed for 5 minutes. The paper is then
removed and the amount of moisture is measured. This technique
measures basic tear function.
 A young person normally moistens 15 mm of each paper strip. Because
hypolacrimation occurs with aging, 33% of normal elderly persons may wet
only 10 mm in 5 minutes. Persons with Sjögren's syndrome moisten less
than 5 mm in 5 minutes.
 INTERPRETATION
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.

20. ROSE BENGAL DYE

21. Revised classification criteria for Sjogren’s Syndrome
 Ocular symptoms : at least one of -
 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
 Oral Symptoms : at least one of –
 Dry mouth for more than 3 months
 Recurrently or Persistently swollen salivary glands
 Need liquids to swallow dry food
 Ocular Signs –at least one the following two tests positive
 Schirmer’s test
 Rose Bengal score

22. 4. Histopathology: in minor salivary glands, focal lymphocytic
sialoadenitis (focus score ≥1).
5. Salivary gland involvement: a positive result for at least
one of the following diagnostic tests:
1 Unstimulated whole salivary flow (≤1.5 ml in 15 min)
2 Parotid sialography showing punctate,
cavitary, or destructive pattern, without evidence of obstruction
in the major ducts
3 Salivary scintigraphy showing delayed uptake, reduced
concentration
6. Autoantibodies – Anti Ro and Anti La

23. Criteria
For primary SS
 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, 6)
For secondary SS
 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 SS

24. 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.

25.  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