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