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Sjogren's Syndrome
Sjögren's syndrome was in 1933 by Swedish eye specialist Henrik Sjögren
• The condition causes antibodies to attack the body's moisture-producing glands, leading to dryness due to lack of secretions.
• The illness most commonly affects the eyes, mouth, salivary glands, lungs, kidneys, skin and nervous system but all organs of the body can be affected.
• In rare cases, complications from Sjogren's syndrome can cause salivary gland infections, kidney problems, ulcers or pancreatitis.
• Venus Williams, 31, has spoken out about the autoimmune disease that caused her to drop out of the U.S. Open yesterday minutes before she was due to go on court.
• Miss Williams was diagnosed with Sjogren's syndrome two weeks ago - after years of being misdiagnosed with other conditions.
• 'For years I felt that I didn't have enough stamina and then, four years ago, I felt like I was not getting enough air but I was diagnosed with exercise-induced asthma,' she said.
•'The medicine for asthma never worked.'
• Eventually, as her symptoms progressed doctors got to the bottom of the problem. The athlete now suffers from fatigue, swollen joints, dry eyes, dry mouth and heavy limbs.
By Daily Mail ReporterUPDATED: 11:55 GMT, 2 September 2011
Sjögren's Syndrome
• A chronic, slowly progressive autoimmune disease characterized by lymphocytic infiltration of the exocrine glands resulting in xerostomia and dry eyes.
• 1/3 have systemic manifestations
• Few develop lymphoma
• female-to-male ratio, 9:1
2 Forms
• Primary Sjögren'ssyndrome:
The disease presents alone
• Secondary Sjögren's :
Asso. with other autoimmune diseases
–RA
– SLE
– Scleroderma
–Mixed CT disease
–Primary biliary cirrhosis
–Vasculitis
–Chronic active hepatitis
Sjögren syndrome: Etiopathology
• Etiology -not well understood
• Findings suggest an ongoing interaction between the innate and acquired immune systems
• Lymphocytic (T,B) infiltration of exocrine glands
+
• B lymphocyte hyper-reactivity
Sjögren syndrome: Etiopathology
Glandular epith cells express MHC class II mols
• Inherited susceptibility+ exo /endogenous antigens
• Trigger a self-perpetuating inflammatory response
• Continuing presence of active interferon pathways suggest ongoing activation of the innate immune system
Sjogren’s: Clinical Manifestation
The majority have symptoms related to diminished lacrimal and salivary gland function.
In most, the primary syndrome runs a slow and benign course over 8 to 10 yrs.
Sjogren’s: Oral Manifestations• Dryness (xerostomia), burning sensation
• Difficulty in swallowing dry food
• Inability to speak continuously
• Dental caries
• Problems in wearing dentures
Physical examination:
• Dry, reddened, sticky mucosa
• Atrophy of tongue
• Saliva not expressible/ cloudy
• Enlargement of salivary glands - 2/3 of primary SS
- Uncommon in secondary
Dryness of the mouth - xerostomiaDeep red tongue Dental caries common
bilateral parotid enlargement- primary Sjögrensyndrome.
Sjögren’s syndrome and a L parotid mass.Biopsy: B-cell lymphoma of mucosal lymphoid tissue
Sjogren’s: Ocular manifestations
Due to the destruction of corneal and bulbar conjunctival epithelium, defined as keratoconjunctivitis sicca
• A sandy/ gritty feeling under eyelids • Burning • Accumulation of thick secretions at inner canthi• Decreased tearing, redness, itching, • eye fatigue, increased photosensitivity
Sjogren’s: Clinical Manifestations
Other exocrine glands
• Resp : secretions dry nose, throat, and xerotrachea
• GIT: Esophageal mucosal atrophy, atrophic gastritis, and subclinical pancreatitis
• GUT: Dyspareunia and dry skin
Sjogren’s: Clinical Manifestation
Medications that cause similar symptoms
• Antidepressants
• Anticholinergics
• Beta blockers
• Diuretics
• Antihistamines
• Women on HRT
• Anxiety
Sjogren’s: Clinical Manifestations
Extra Glandular:
• Easy fatigability, low-grade fever, Raynaud‘s, myalgias, and arthralgias
• Joints: Non-erosive arthritis
• Pulmonary : Dry cough (small airway disease)
• Renal: interstitial nephritis, acidosis and nephrocalcinosis. Glomerulonephritis rare (SLE overlaps).
• Vasculitis -purpura, recurrent urticaria, skin ulcerations, GLN, and mononeuritis multiplex.
• SN hearing loss, any focal deficits
Sjogren’s: Clinical Manifestations
Glandular:
• Xerophthalmia
• Xerostomia
• Bilateral parotid swelling
Extraglandular:
• Arthralgias/arthritis
• Raynaud's phenomenon
• Lymphadenopathy
• Lung involvement
• Vasculitis
• Kidney involvement
• Liver involvement
• Lymphoma
• Splenomegaly
• Peripheral neuropathy
• Myositis
Sjogren’s: Investigations
Routine:
• Mild normochromic, normocytic anemia
• ESR rise- in 70%
Mouth:
• Sialometry
• sialography
• Imaging: Ultrasound, MRI or MR sialography of salivary glands.
• salivary gland biopsy- focal lymphocytic infiltrates.
Sjogren’s: Investigations
Eyes:
• Measurement of tear flow by schirmer‘s test
• Tear composition
• tear breakup time
• tear lysozyme content
• Slit-lamp examination after rose Bengal staining
• punctuate corneal ulcerations
• attached filaments of corneal epithelium
Schirmer test: Detects deficient tear production in Sjögren syndrome
Sjogren’s Investigations: Must exclude
• Hepatitis C virus infection should be ruled out since, apart from serologic tests, the clinicopathologic picture is almost identical to that of Sjögren's syndrome.
• HIV infection
• Sarcoidosis
Sjogren’s: Treatment• Artificial tears, Rx -corneal ulcerations
• Avoid drugs that secretions (diuretics, anti HTs, anticholinergics & antidepressants)
• Xerostomia: Best replacement - water
• Vaginal dryness: Propionic acid gels
• Secretagogues: Oral Pilocarpine / Cevimeline
• Arthralgias : HCQ
• RTA: Oral Soda bicarb
• Systemic vasculitis: Steroids, immunosuppressives, M Abs
• High-grade lymphomas: Chemo (CHOP) + M Abs
vaginal dryness