sjogren's syndrome clinical features,diagnosis,management

1. SJOGREN’S SYNDROME

2. Swedish
Ophthalmologist
described it (1933) ;
Henri
k
Sjögre
n
wh
o
Sjogren’ssyndrome is a late onset chronic
systemic autoimmune disease characterized by
lymphocytic infiltration and destruction of the
salivary and lacrimal
atrophy of conjunctiva and cornea
(keratoconjunctivits sicca).
firs
t
glands resulting in dry mouth(xerostomia) and
dryness and

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

4. ETIOLOGY
Female : Male = 9 :
1 4/5/6th decade
associated with syndrome occuringwith raynaud’s
phenomenon
Antibodies to the Ro antigen occur in excess in
relatives of
ptswith
sjogren’ssyndrome.
Autoimmune ; HLA-B8 / DR3 ,although DR4 is more
closely

5. Pathogenesis of Sjögren’ssyndrome 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,espestrogen) components

7. Connective tissue proliferation
Lymphocyte and plasma cell infiltration
Secondary changes –oedemaof conjuctiva
Auto
-
Glandular cell apoptosis atrophy of glandular
structures in affected tissues (salivary glands,
antibody production (to
‘Ro’)
sebaceous glands, sweat glands)

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 –multilpecarries 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%)
Vitilig
o
Cutaneous Amyloidosis Alopecia—
diffuse and generalized
Xeroderma, pruritus and scaling
Annular erythema, PapularErythema including
Sweet’s-like lesions.
Raynaud’s syndrome
Sweating abnormalities
HyperglobulinemicPurpura

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

14. Sjogren’sSyndrome –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'ssyndrome
Histolgyof 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 gland aciniare seen
Two excretory ducts and 3 mucous salivary

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
INTERPRETATION
1. Normal which is ≥15mm wetting of the paper after 5 minutes
2. Mild which is 14-9mm wetting of the paper after 5 minutes
3. Moderate which is 8-4mm wetting of the paper after 5 minutes
4. Severe which is <4mm wetting of the paper after 5 minutes.
Schirmer'stest places a small strip of filter paper inside the lower eyelids
(conjunctivalsac). 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 15mm of each paper strip. Because
hypolacrimationoccurs with aging, 33% of normal elderly persons may wet
only 10mm in 5 minutes. Persons with Sjögren's syndromemoisten less
than 5mm in 5 minutes.

20. ROSE BENGAL DYE

21. Revised classification criteria for Sjogren’sSyndrome
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).
6. Autoantibodies –Anti Ro and Anti La
5. Salivary gland involvement: a positive result for at least
one of the following diagnostic tests:
1 Unstimulatedwhole salivary flow (≤1.5 ml in 15 min)
2 Parotid sialographyshowing punctate,
cavitary, or destructive pattern, without evidence of
obstruction
in the major ducts
3 Salivary scintigraphyshowing delayed uptake, reduced
concentration

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

24. Treatmen
t
Symptomatic treatment for dryness of eyes–by
lubricating agents such as 0.5% methylcellulose eye
drops for 4-5 times daily.
Bromhexine16 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.
Cyclosporine ocular drops

25. Systemic steroids are effective in reducing parotid
swelling
Candidiasis –topical nystatin3times/day for a week
Systemic –ketoconazole 200-400mg/day or fluconazole
50-100 mg/day or itraconazole-100mg/day for 2
weeks
hydroxychloroquine200 mg daily is useful