SJOGREN’S SYNDROME

Dr.Aakanksha Singh

Sjogren’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 Sjögren who first

described it (1933) ;

This disease is caused by an immune-mediated

inflammation of salivary,lacrimal and sweat glands as

Sicca Syndrome or with internal organ involvement.

Clinical Types

PRIMARY SS - Alone.

SECONDARY SS - associated underlying connective

tissue diseases (RA / SLE / Scleroderma )

SICCA SYNDROME – Xerophthalmia + Xerostomia –

Internal Organ / Bone Inv

ETIOLOGY Female : Male = 9 : 1

4/5/6th decade

Autoimmune ; HLA-B8 / DR3 ,although DR4 is more closely

associated with syndrome occuring with raynaud’s

phenomenon

Antibodies to the Ro antigen occur in excess in relatives of

pts with sjogren’s syndrome.

Pathogenesis of Sjögren’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)

Secondary changes – oedema of conjuctiva

C/F Glandular 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 MANIFESTATIONS (50%)Xeroderma, pruritus and scaling

Annular erythema, Papular Erythema including Sweet’s-like lesions.

Raynaud’s syndrome

Hyperglobulinemic Purpura

Vitiligo

Sweating abnormalities

Cutaneous Amyloidosis Alopecia—diffuse and generalized

OTHER MANIFESTATIONS

Joint symptoms - Arthralgia and arthritis

Myalgia and myositis

ENT : Sinusitis / Hearing Loss

GI : GERD

Resp : Interstitial pneumonitis, pulmonary fibrosis and pulmonary hypertension

Nephro : Interstitial nephritis, Renal Tubular Acidosis

Neuro : migraine, neuropathies, cerebral vasculitis

Sjogren’s Syndrome – Associations

Diagnosis SS patients of both primary and secondary Sjögren’s

syndrome have marked hypergammaglobulinemia

(IgG>IgA>IgM),ANA(>50%) elevated total protein and

sedimentation rate.

Anti-Ro and Anti-La Antibodies occur in approximately

60% of patients with Sjögren'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 infiltrate

Two excretory ducts and 3 mucous salivary

gland acini are seen

SCHIRMER’S TEST

German 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 TEST Schirmer's test places a small strip of filter paper inside the lower eyelids

(conjunctival sac). The eyes are closed for 5 minutes. The paper is then removed and the amount of moisture is measured. This technique measures basic tear function.

A young person normally moistens 15 mm of each paper strip. Because hypolacrimation occurs with aging, 33% of normal elderly persons may wet only 10 mm in 5 minutes. Persons with Sjögren's syndrome moisten less than 5 mm in 5 minutes.

INTERPRETATION

1. Normal which is ≥15 mm wetting of the paper after 5 minutes

2. Mild which is 14-9 mm wetting of the paper after 5 minutes

3. Moderate which is 8-4 mm wetting of the paper after 5 minutes

4. Severe which is <4 mm wetting of the paper after 5 minutes.

ROSE BENGAL DYE

Revised classification criteria for Sjogren’s Syndrome

Ocular symptoms : at least one of -

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

Oral Symptoms : at least one of –

Dry mouth for more than 3 months

Recurrently or Persistently swollen salivary glands

Need liquids to swallow dry food

Ocular Signs –at least one the following two tests positive

Schirmer’s test

Rose Bengal score

4. Histopathology: in minor salivary glands, focal lymphocytic

sialoadenitis (focus score ≥1).

5. Salivary gland involvement: a positive result for at least

one of the following diagnostic tests:

1 Unstimulated whole salivary flow (≤1.5 ml in 15 min)

2 Parotid sialography showing punctate,

cavitary, or destructive pattern, without evidence of obstruction

in the major ducts

3 Salivary scintigraphy showing delayed uptake, reduced

concentration

6. Autoantibodies – Anti Ro and Anti La

Criteria

For primary SS

In 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, 6)

For secondary SS

In 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 SS

Treatment Symptomatic treatment for dryness of eyes – by

lubricating agents such as 0.5% methylcellulose eye

drops for 4-5 times daily.

Cyclosporine ocular drops

Bromhexine 16 mg TDS has been found to increase the

lacrimal secretion.

Artificial saliva and cyclosporin(2.5-5mg/kg), for

xerostomia.

Routine dental care

Steam 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