Sjogren’s SyndromeBy

Farshid MokhberiShahid Beheshti University Of Medical Sciences & Health

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DefinitionSjogren’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)

ETIOLOGYFemale : Male = 9 : 14/5/6th decadeAutoimmune ; HLA-B8 / DR3 ,although DR4 is

more closely associated with syndrome occuring with raynaud’s phenomenonPathogenesis 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

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)

Clinical TypesPRIMARY : 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.

Clinical FeaturesGlandular 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

Teeth :multilpe carries and early loss

Chronic oral candidiasis is frequent.Parotid Gland Enlargement

Lacrimal Gland Involvement – Dryness of eyes causes keratoconjuctivitis sicca.

Burning itchy sensation in the eyes.

Skin manifestationXeroderma, pruritus and scaling Annular erythema, Papular Erythema Raynaud’s syndrome Hyperglobulinemic PurpuraVitiligoSweating abnormalities Cutaneous AmyloidosisAlopecia—diffuse and generalized

OTHER MANIFESTATIONSJoint 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

Sjogren’s Syndrome Associations

Diagnosishypergammaglobulinemia

(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.

Biopsy of labial salivary glands

Ly

lymphocytic and plasma cells infiltrateTwo excretory ducts and 3 mucous salivary gland acini are seen

SCHIRMER’S TESTGerman 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

SCHIRMER’S TEST1. Normal which is ≥15 mm wetting of the paper after 5 minutes2. Mild which is 14-9 mm wetting of the paper after 5 minutes3. Moderate which is 8-4 mm wetting of the paper after 5 minutes4. Severe which is <4 mm wetting of the paper after 5 minutes.

Rose Bengal

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

CriteriaFor primaryIn 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 secondaryIn 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

TreatmentSymptomatic treatment for dryness of eyes –

by lubricating agents such as 0.5% methylcellulose eye drops for 4-5 times daily.

Cyclosporine ocular dropsBromhexine 16 mg TDS has been found to

increase the lacrimal secretion.Artificial saliva and cyclosporin(2.5-5mg/kg),

for xerostomia.Routine dental careSteam inhalation may help dryness of the

respiratory tract.

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

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