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Sjogren’s SyndromeBy
Farshid MokhberiShahid Beheshti University Of Medical Sciences & Health
services
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
Refrences WWW.UPTODATE.COMWWW.MEDSCAPE.COMWWW.EMEDICINE.COMWWW.PUBMED.COMWWW.WEBMD.COMWWW.MYOCLINIC.COM
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