Sjogren’s syndrome

Sjogren’s  syndrome 

  Definition

  Sjogren’s syndrome is a chronic, systemic autoimmune inflammatory disease that principally involves salivary and lacrimal glands resulting in xerostomia (dry mouth) and xerophthalmia (dry eyes).

  Presentation of both xerostomia and xerophthalmia is known as  sicca syndrome  (Sicca means dry).

  Forms of the disease 

A) Primary Sjogren’s syndrome  (sicca syndrome alone, no other autoimmune disorder is present).

B)  Secondary Sjogren’s  syndrome
The patient manifests sicca syndrome  in addition to another autoimmune disease as rheumatoid arthritis.

  Clinically 

  Mostly affects  middle-aged adult femals. 

A)  Eye manifestations: 

Dryness, redness, burning sensation of eyes and sensation of presence of a
foreign body in the eye  (The effects on the eye often are called
keratoconjunctivitis sicca).

B)  Oral manifestations: 

1. Xerostomia.
2. Difficulty in swallowing.
3. Altered taste.
4. The tongue often becomes fissured and exhibits atrophy of the papillae.
5. The oral mucosa is dry and may be red and tender (usually as a result of  candidiasis).
6. Difficulty in wearing dentures.
7. Increased risk for dental caries (especially cervical caries).

C)  Enlargement of the major salivary glands
: 
Swelling is usually diffuse, firm and  bilateral.  It may be non painful or slightly tender.

  Radiograghic examination (Sialography) 

  Punctate sialectasia demonstrating a "fruit-laden, branchless tree" .    
  This is due to leakage of contrast material through the duct wall reflecting significant ductal and acinar damage.

  Histopathologic features 

  Lymphocytic infiltration of the salivary gland with disrtuction of the acini.
  proliferation of some duct tissue forming epimyoepithelial islands.
  There is a great risk of malignant  transformation  to lymphoma (more common in primary Sjogren).

Xerostomia 

  Xerostomia means dry mouth

  Causes  
1.  Developmental (salivary gland aplasia).
2.  Loss of body fluids as:
-  Impaired fluid intake.
-  Hemorrhage.
-  Vomiting and diarrhea.
3.  Medications as antihistamines and antidepressants.
4.  Radiation therapy to the head and neck.
5.  Systemic diseases as Sjogren’s syndrome and diabetes.
6.  Local factors as smoking and mouth breathing.

  Clinical features 

1.  Reduction in salivary secretion.
2.  Difficulty with mastication and swallowing.
3.  The mucosa appears dry.
4.  Poor retention of dentures.
5.  The dorsal tongue often is fissured with atrophy of the filiform papillae.
6.  Predisposition to infection as  oral candidiasis  (because of the reduction in the cleansing and antimicrobial activity of the saliva).
7.   Increased risk for dental caries (especially cervical caries).