Sjogren's syndrome is an autoimmune disorder characterized by dry eyes and dry mouth due to reduced functioning of the salivary and lacrimal glands. It can occur as a primary condition or secondary to other autoimmune diseases like rheumatoid arthritis. The disease is more common in women over age 40 and is associated with genetic and immunological factors. Symptoms include dryness, eye and mouth pain, and enlarged salivary glands. Histological examination shows lymphocytic infiltration of the glands. Treatment focuses on symptom relief; prognosis depends on the extent of systemic involvement.

1. Sjogren’s syndrome
(Sicca syndrome, Gougerot-Sjögren syndrome)

2. • Description
• Etiology
• Location sa body (if indicated)
• Types if may ara
• Clinical feature/s
• Occurrence
• Common sites
• Histologic feature (Picture if available)
• Treatment and Prognosis

3. Sjogren’s syndrome
• Described as a triad consisting of
keratoconjunctivitis sicca,
xerostomia and rheumatoid
arthritis
• There are some patients present
only with dry eyes and dry mouth
(sicca complex or primary
Sjögren’s syndrome), while others
also develop systemic lupus
erythematosus, polyarteritis
nodosa, polymyositis or
scleroderma, as well as
rheumatoid arthritis (secondary
Sjögren’s syndrome).

4. Etiology.
• May be genetic, hormonal, infectious and immunologic, among
others. It may well be that a combination of factors, both extrinsic
and intrinsic, play a role in the etiology of this condition
• Most authorities consider an altered immunologic response to be
the main intrinsic factor which is responsible for the disease.
• The sicca complex and Sjogren’s syndrome have been found to be
associated with the HLA system, specifically HLA-DR3 and HLA-B8
which are associated with primary form of the disease and HLA-
DRw52 seen to be associated with both the forms of Sjögren’s
syndrome.

5. Clinical Features
• Occurs predominantly in women over 40 years of age; children or young adults
may be affected.
• Female: male ratio is approximately 10 : 1.
• The typical features of the disease are dryness of the mouth and eyes as a result
of hypofunction of the salivary and lacrimal glands which results in painful,
burning sensations of the oral mucosa.
• Various secretory glands of the nose, larynx, pharynx and tracheobronchial tree
(buccopharyngolaryngitis sicca), as well as of the vagina, are also involved with
this dryness.
• Lymphadenopathy is more than twice as common in the primary form of the
disease

6. Clinical Features
• Rheumatoid arthritis, as mentioned, is an integral part of secondary Sjögren’s
syndrome.
• It has been shown that patients with Sjögren’s syndrome with rheumatoid
arthritis have certain different clinical manifestations than patients with sicca
complex, despite similar histologic findings and some laboratory findings.
• In this regard, patients without rheumatoid arthritis, that is, sicca complex or
primary Sjögren’s syndrome, more frequently manifest parotid gland
enlargement, lymphadenopathy, purpura, Raynaud’s phenomenon, kidney
involvement and myositis.

7. Histologic Features
• Three types of histologic alterations in the
major salivary glands have been described.
• Intense lymphocytic infiltration of the gland
replacing all acinar structures although the lobular
architecture is preserved.
• Proliferation of ductal epithelium and
myoepithelium to form ‘epimyoepithelial islands’.
• Atrophy of the glands sequential to the lymphocytic
infiltration.
• Patients also exhibited alterations in the
accessory salivary glands of the lip
characteristically similar to those in the major
glands

8. Radiographic Features
• Sialography may be of diagnostic
value in Sjögren’s syndrome.
• Sialographs demonstrate the
formation of punctate, cavitary
defects which are filled with
radiopaque contrast media. These
filling defects have been said to
produce a ‘cherry blossom’ or
‘branchless fruit-laden tree’ effect
radiographically.

9. Treatment and Prognosis
• There is no satisfactory treatment for Sjögren’s syndrome.
• Most patients are treated symptomatically.
• Keratocon junctivitis is treated by instillation of ocular lubricants such as
artificial tears containing methylcellulose, and xerostomia is treated by saliva
substitutes such as those used in the treatment of person with xerostomia
secondary to radiation therapy
• Extensive dental caries is a complication which is quite common, and
scrupulous oral hygiene and frequent fluoride application is indicated to
reduce this problem.
• There is no specific treatment for enlargement of the salivary glands.
• Surgery has been employed but is generally recommended only in patients
with discomfort

10. Treatment and Prognosis
• Sjögren syndrome is chronic. Overall health and life expectancy are
mainly unaffected in patients whose only symptoms are dry eyes and
mouth. However, prognosis is less favorable in those with systemic
organ involvement. In those with severe disease, death may
occasionally result from pulmonary infection and, rarely, from renal
failure or lymphoma. Associated systemic autoimmune disorders may
dictate prognosis.