Sjogrens Syndrome

Sjögren’s syndrome is an autoimmune condition that results in abnormal dryness of the mouth, eyes and/or other tissues as well as swelling of glands around the face & neck.

(Keratoconjunctivitis characterized by xerophthalmia, xerostomia, rheumatoid arthritis.)

Primary sjogrens syndrome	Dry mouth, dry eyes, associated with HLA system, HLA D3 & HLA-B8 re associated with primary form and HLA-DRW52 is associated with both primary and secondary form. Extra glandular manifestations Arthritis, Raynaud’s phenomenon, Lymphoma, Pulmonary involvement, GIT and hepatobiliary features Renal involvement Vasculitis Neuromuscular involvement Autoimmune thyroid disease.
Secondary sjogrens syndrome	Dry mouth, Dry eyes, Connective tissue disorders like SLE , polyarteritis nodosa, polymyositis or scleroderma

Etiology

Genetic in origin
Hormonal induced
Extraneous factor like EBV has been implicated.
Immunological bases

Clinical features

Female predilection 10: 1
Less than 40 years
Painful burning sensation consistently present in the mucosa
Less or reduced sensation of the tracheobronchial of larynx, pharynx & nose.
Female genital tract
Patient will be having reduced salivary flow & elevated levels of immunoglobulin Ig A(IgG)
80% of the patient shows parotid gland enlargement
Presence of lymphadenopathy
Enlargement of nodes
Purpura in some patients

Diagnosis include:

Blood Tests
Marker antibodies for Sjögren’s- The antibodies associated with Sjogren's include anti-Ro (SS-A) and anti-La (SS-B) antibodies, rheumatoid factor, and antinuclear antibodies.
Eye Tests
Schirmer test

Rose Bengal dye test

Dental Tests

Salivary Flow: Measures the amount of saliva produced over a certain period of time.
Salivary Gland Biopsy(usually in the lower lip): Confirms lymphocytic infiltration of the minor salivary glands.

Histopathology of normal appearing mucosa containing minor salivary gland

• Focal lymphocytic sialadentitis
• Acinar cells are destroyed.
• Focus score of less than 1 defined as no of lymphocytic foci per 4 mm2 of glandular tissue less than 50 lymphocytes.
• Proliferation of ductal & myoepithelial forming epi-myoepithelial islands.
• Atrophy of the glands sequential to lymphocytic infiltration.

Sialography
Normal sialogram of a salivary gland gives a leafless tree pattern. Sjogrens syndrome shows cherry blossom appearance, space occupying lesion manifest as ball in hand appearance.