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's syndrome:

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: EtiopathologyGlandular 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 - xerostomia Deep red tongue Dental caries common

bilateral parotid enlargement- primary Sjögren syndrome.

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: InvestigationsRoutine: • 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