Sjögren Syndrome Sjögren Syndrome A chronic autoimmune disease. A chronic autoimmune disease. Characterized by lymphocytic infiltration and Characterized by lymphocytic infiltration and acinar destruction of lacrimal and salivary acinar destruction of lacrimal and salivary glands. glands. Classified into: Classified into: 1. Primary Sjögren or sicca syndrome : dry mouth 1. Primary Sjögren or sicca syndrome : dry mouth (xerostomia) and dry eyes (xerophthalmia or (xerostomia) and dry eyes (xerophthalmia or keratoconjunctivitis sicca) keratoconjunctivitis sicca) 2. Secondary Sjögren syndrome : xerostomia, 2. Secondary Sjögren syndrome : xerostomia, xerophthalmia, & an autoimmune CT disease, xerophthalmia, & an autoimmune CT disease, usually rheumatoid arthritis. usually rheumatoid arthritis.
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Sjögren SyndromeSjögren Syndrome
A chronic autoimmune disease.A chronic autoimmune disease.
Characterized by lymphocytic infiltration and acinar Characterized by lymphocytic infiltration and acinar destruction of lacrimal and salivary glands.destruction of lacrimal and salivary glands.
Classified into:Classified into:1. Primary Sjögren or sicca syndrome : dry mouth 1. Primary Sjögren or sicca syndrome : dry mouth
(xerostomia) and dry eyes (xerophthalmia or (xerostomia) and dry eyes (xerophthalmia or keratoconjunctivitis sicca)keratoconjunctivitis sicca)
2. Secondary Sjögren syndrome : xerostomia, 2. Secondary Sjögren syndrome : xerostomia, xerophthalmia, & an autoimmune CT disease, usually xerophthalmia, & an autoimmune CT disease, usually rheumatoid arthritis.rheumatoid arthritis.
Sjögren Syndrome: Clinical FeaturesSjögren Syndrome: Clinical Features
Rheumatoid arthritis is the most common CT disease Rheumatoid arthritis is the most common CT disease associated with 2ry SS.associated with 2ry SS.
Other autoimmune diseases that may be associated Other autoimmune diseases that may be associated include:*include:*
Sjögren Syndrome: Clinical FeaturesSjögren Syndrome: Clinical Features
Unless stated otherwise, the general term Unless stated otherwise, the general term Sjögren syndrome (SS) is used to Sjögren syndrome (SS) is used to encompass both types.encompass both types.
Both 1ry & 2ry SS exhibit a wide Both 1ry & 2ry SS exhibit a wide spectrum of clinical features associated spectrum of clinical features associated with widespread involvement of other with widespread involvement of other glands and other tissues.glands and other tissues.
Sjögren Syndrome: Clinical FeaturesSjögren Syndrome: Clinical Features
Sjögren Syndrome: Clinical FeaturesSjögren Syndrome: Clinical Features
European criteria for diagnosis of SS relate to:European criteria for diagnosis of SS relate to:i.i. Ocular symptoms.Ocular symptoms.ii.ii. Ocular signs.Ocular signs.iii.iii. Oral symptoms.Oral symptoms.iv.iv. Salivary gland function.Salivary gland function.v.v. Labial salivary gland histology.Labial salivary gland histology.vi.vi. Ro and La autoantibodies.Ro and La autoantibodies.
4 of the 6 criteria need to be fulfilled.4 of the 6 criteria need to be fulfilled.
Sjögren Syndrome: Clinical FeaturesSjögren Syndrome: Clinical Features
SS predominantly affects middle-aged SS predominantly affects middle-aged females (9:1 F:M ratio).females (9:1 F:M ratio).
The most common symptoms are related The most common symptoms are related to xerostomia and xerophthalmia.to xerostomia and xerophthalmia.
In general, ocular & oral manifestations In general, ocular & oral manifestations are more severe in 1ry SS.are more severe in 1ry SS.
Sjögren Syndrome: Clinical FeaturesSjögren Syndrome: Clinical Features
Xerostomia may be Xerostomia may be associated with:associated with:
1.1. difficulty in swallowing & difficulty in swallowing & speakingspeaking
2.2. increased fluid intakeincreased fluid intake3.3. disturbances of tastedisturbances of taste4.4. soreness & redness of soreness & redness of
mucosa associated with mucosa associated with candidosiscandidosis
Sjögren Syndrome: Clinical FeaturesSjögren Syndrome: Clinical Features
Oral mucosa appears Oral mucosa appears dry, smooth, and dry, smooth, and glazed.glazed.
Dorsum of tongue Dorsum of tongue often appears red and often appears red and atrophic with variable atrophic with variable degrees of fissuring degrees of fissuring and lobulation.and lobulation.
Sjögren Syndrome: Clinical FeaturesSjögren Syndrome: Clinical Features
Sjögren Syndrome: Clinical FeaturesSjögren Syndrome: Clinical Features
Lacrimal gland Lacrimal gland enlargement is enlargement is uncommon.uncommon.
Although clinical Although clinical involvement of minor involvement of minor salivary glands is salivary glands is uncommon, they are uncommon, they are often involved often involved microscopically.microscopically.
Sjögren Syndrome: Histopathological FeaturesSjögren Syndrome: Histopathological Features
Major glands:Major glands:
1.1. Lymphocytic infiltration, Lymphocytic infiltration, initially around initially around intralobular ducts, intralobular ducts, eventually replacing the eventually replacing the whole affected lobules.whole affected lobules.
2.2. 20% B cells, 80% T 20% B cells, 80% T cells, mostly T-helper.cells, mostly T-helper.
3.3. Acinar atrophy.Acinar atrophy.
Sjögren Syndrome: Histopathological FeaturesSjögren Syndrome: Histopathological Features
Major glands:Major glands:
4.4. Proliferation of duct epithelium Proliferation of duct epithelium to form epimyoepithelial to form epimyoepithelial islands.islands.
5.5. The appearance is described The appearance is described as as myoepithelial sialadenitismyoepithelial sialadenitis or or benign lymphoepithelial lesionbenign lymphoepithelial lesion..
6.6. Unlike lymphoma, the infiltrate Unlike lymphoma, the infiltrate does not cross interlobular CT does not cross interlobular CT septa.septa.
Sjögren Syndrome: Histopathological FeaturesSjögren Syndrome: Histopathological Features
Minor glands:Minor glands:
1.1. Focal collections of lymphoid Focal collections of lymphoid cells, initially around intralobular cells, initially around intralobular ducts.ducts.
2.2. The number of foci reflects the The number of foci reflects the severity of the disease.severity of the disease.
Sjögren Syndrome: Histopathological FeaturesSjögren Syndrome: Histopathological Features
Minor glands:Minor glands:
3.3. The semi-quantitative assessment The semi-quantitative assessment of this focal lymphocytic of this focal lymphocytic sialadenitis in labial minor salivay sialadenitis in labial minor salivay gland biopsies is an important gland biopsies is an important investigation in establishing a investigation in establishing a diagnosis, and is one of the diagnosis, and is one of the diagnostic criteria.diagnostic criteria.
4.4. However, since the appearance is However, since the appearance is non-specific, it must be interpreted non-specific, it must be interpreted in the presence of clinical features in the presence of clinical features and serological investigations.and serological investigations.
Sjögren Syndrome: InvestigationsSjögren Syndrome: InvestigationsApproximate frequencies of serological abnormalities & autoantibodies in Approximate frequencies of serological abnormalities & autoantibodies in
Although neither anti-Ro or anti-La is Although neither anti-Ro or anti-La is specific for SS, they are diagnostically specific for SS, they are diagnostically helpful since they may be detected some helpful since they may be detected some time before clinical picture develops.time before clinical picture develops.
Strong evidence that it is autoimmune.Strong evidence that it is autoimmune.
Genetic factors thought to be important in increasing susceptibility Genetic factors thought to be important in increasing susceptibility to external factors which trigger the disease.to external factors which trigger the disease.
Occurs with increased frequency in patients with certain HLA class Occurs with increased frequency in patients with certain HLA class II MHC genes.II MHC genes.
Several viruses, especially EBV have been suggested as potential Several viruses, especially EBV have been suggested as potential trigger factors.trigger factors.
Immunological mechanisms leading to destruction of glandular Immunological mechanisms leading to destruction of glandular tissue probably involve mainly T cells and their cytokines.tissue probably involve mainly T cells and their cytokines.
Pathogenic significance of the range of circulating autoantibodies is Pathogenic significance of the range of circulating autoantibodies is uncertain.uncertain.
Risk of B cell lymphoma Risk of B cell lymphoma developing in affected gland developing in affected gland 44 times that of general 44 times that of general population.population.
Risk varies from <1%-6% of Risk varies from <1%-6% of SS patients.SS patients.
Risk may be slightly greater in Risk may be slightly greater in 1ry SS than 2ry SS.1ry SS than 2ry SS.
Malignancy usually occurs late Malignancy usually occurs late in the course of disease.in the course of disease.
May be associated with May be associated with increased swelling of gland.increased swelling of gland.
Associated lymphomas share Associated lymphomas share many similarities with MALT many similarities with MALT lymphomas; they tend to lymphomas; they tend to pursue indolent course & pursue indolent course & remain localized until late.remain localized until late.
