Sjogren's Syndrome (Told) Flashcards
SJÖGREN SYNDROME =
KERATOCONJUNCTIVITIS SECCA (KCS)
dry eyes (decreased tear production, damages to eye surface)
Dry mouth (increased tooth decay; periodontal disease)
Primary Sjogrens
OCCURS IN ISOLATION PREDOMINENTLY IN WOMEN 40-60 YEARS DRY EYES, MOUTH AND RESPIRATORY TRACT. EXOCRINE GLAND DYSFUNCTION. RA AND OTHER AUTOANTIBODIES COMMON
Secondary Sjogrens
ASSOCIATION WITH OTHER RHEUMATIC DISEASE RA SLE PRIMARY BILIARY CIRRHOSIS SCLERADERMA POLYMYOCITIS HASHIMOTOS THYROIDITIS POLYARTERITIS INTERSTITIAL PULMONARY FIBROSIS
Preliminary Criteria of the Classification of Sjögren’s Syndrome
4/6 criteria: sensitivity of 93.5% & specificity of 94%.
Autoimmune exocrinopathy Ocular symptoms Oral symptoms Ocular signs (Schirmer’s or Rose Bengal) Characteristic histopathologic features Salivary gland involvement by testing Autoantibodies (RF, SS-A, SS-B)
SOLID PHASE ASSAYS OF ANTI-SSA AND ANTI-LA SSB
AUTO ANTIBODY PATTERN PRESENT IN 49%
OF PATIENTS WITH SLE AND 75% OF PATIENTS
WITH SJÖGRENS
Examples:
Anti –Ro60 SSA
Nucleolar and Nuclear
Speckled pattern
Anti-Ro52 SSA
Cytoplasmic staining
Pattern
Anti-LA SSB finely
Speckled pattern
OTHER LABS
CBC= ANEMIA, LEUKOPENIA, EOCINOPHELIA
HYPERGAMMAGLOBULINEMIA
(Polyclonal)
RA POSITIVE 70% OF THE TIME
ANA POSITIVE 95% OF THE TIME
Extraglandular manifestations in primary Sjogren’s Syndrome
constitutional: fever, fatigue, lymphadenopathy
Joints/ muscles- articular involvement, tendomyalgia, myositis
skin- Raynaud’s phenomenon, cutaneous vasculitis, skin involvement other than cutaneous vasculitis
Endocrine- autoimmune thyroiditis
Respiratory tract- pulmonary involvemnt, serositis
GI tract: esophageal involvemnt, autoimmune hepatitis, acute pancreatitis
Nervous system- peripheral neuropathy, CNS involvement
Urogenital ract- renal involvement, bladder involvement
Hematology: thrombocytopenia, lymphoproliferative disease
Clinical presentations
parotid gland enlargement
Infected stenson’s dut
Dental caries
Salivary duct stone
SICCA SYMPTOMS OF SJÖGRENS SYNDROME
ORAL MUCOSA
VAGINAL MUCOSA
CONJUNCTIVA
Schirmer’s test
stick paper in eyes
Wetting of less than 5mm/5min of the filter paper strip is abnormal & indicates reduced lacrimal secretions.
Rose bengal staining
Rose Bengal staining + indicates inflammation & irritation of the conjunctival layer.
lung involvement
LOW TRACHIOBRONCHIAL SECRETIONS LEAD TO
NONSPECIFIC INTERSTITIAL PNEUMONIA (NSIP) AND
XYEROTRACHEA.
Sjögren’s Syndrome Treatment
Sjogren’s Syndrome is treated symptomatically Artificial tears methyl cellulose Cyclosporin drops oral hygiene and hydration Pilocarpine 5mg QID Cevimeline 30 mg TID Careful watch for Lymphoma
