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Sjogrens syndrome

Sjogrens syndrome is an autoimmune disorder characterized by triad of dry eyes (keratoconjunctivitis sicca), dry month (xerostomia) and arthritis. It has been classified into primary where the disease occurs by itself and secondary when it is associated with other autoimmune disorders like systemic lupus erythematosis, polymyositis, scleroderma and Rheumatid arthritis etc. In the primary form, the involvement is mainly of exocrine system and in the secondary form, besides glands other tissues are also involved.

Aetiopathology

Sjogrens syndrome  is a disease which primarily affects women in the age group of forty and fifty years. The main defect is lymphocytic proliferation and immune complex deposition.

Patients with primary form have increased frequency of HLA-B8, HLA DRW 3 and MT-2 antigens. They also develop an immune complex vasculitis.

Activation of mono nuclear cells results in involvement of glandular structures while oligoclonal B cell activation produces increased levels of circulating immune complexes which produce auto antibodies like rheumatoid factor, SSA (anti RO) and SSB (antiLa). These are associated with a more serious profile of the disease.

Clinical features

The most common presentation of Sjogrens syndrome is dry eyes and dry mouth. The former are due to atrophy of lachrymal glands while the latter is due to atrophy of the salivary glands.

This dryness may extend to other mucosal areas like respiratory tract (Presistent dry cough) and female genital tract. Other manifestations include immune complex glomerulonephritis. Raynauds syndrome, nonthrombocytopenic purpura, vasculitis, sensory polyneuropathy, pseudolymphoma etc.

Cases of Sjogren syndrome who have only dry eyes and dry mouth are known as ‘Sicca syndrome’ and these patients do not have rheumatid arthritis but are associated with other auto immune disorder like SLE, polymyositis, Hashimoto’s thyroiditis, poly arteritis nodosa.

Primary form of the disease has mainly kerato conjunctivitis sicca and has presence of anti-RO and anti-LA/SSB with frequency of HLA-B8-DR3 antigens while in secondary form of disease there is evidence of under lying connective tissue disease with HLA-DR 4 positivity. Rheumatid arthritis may be associated with this form.

Diagnosis

Sjogrens syndrome is based on the presence of thy eyes, dry mouth and presence of anti nuclear antibodies. Schirmer‘s test is an important and simple test. Here a standard strip of filter paper is placed on the inside of lower eye lid.

If it does not get wet in 5 minutes, the test is positive. Further staining of conjunctiva by Rose Bengal dye may show a punctuate or filamentary keratitis.

A lip biopsy may be done to study antinuclear antibodies which are found in 70% of cases. Besides this anti RO (SSA) antibodies are also found in equally higher percentage of cases of sjogrens syndrome.

Treatment

There is no satisfactory treatment for Sjogrens syndrome available. Treatment mainly is directed towards symptomatic relief. For dry eyes artificial tears (0.5% hydroxymethyl cellulose) for dry mouth, oral soothing gels and vaginal gels for dryness in vagina.

Salivary secretions are stimulated by the use of lemon drops. Besides this special precautions should be taken about oral hygiene as these people are likely to suffer from dental caries.

Systemic medications include glucocorticoids which are successful in the management of severe form of disease (glomerulonephritis, vasculitis, pseudolymphoma, pneumonitis).

Other drugs which are of benefit include drugs like methotrexate, azathioprine, cyclophosphamide and chloroq,uine. Gold salts which were used earlier are now discarded. Main aim of treatment is directed towards amelioration of symptoms.

Prognosis

Cases of primary sjogrens syndrome carry good prognosis and but for some disability due to dryness, otherwise life expectancy is not affected. Prognosis in secondary form of disease depends on the type of complications they are suffering from.

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