Sjorgen Syndrome

• Chronic disease characterised by;
  • Dry eyes (keratoconjunctivitis sicca)
  • Dry mouth (xerostomia)
• Due to immunological mediated destruction of the lacrimal and salivary glands
• In its primary form it is an isolated disorder called Sicca syndrome
• May exist in a secondary form when it is associated with other autoimmune diseases , RA being the most common

 

Pathology

T and B cell infiltration of the glands resulting in local antibody production and fibrosis

• Often patients are RF positive (whether they have arthritis or not)
• The majority have ANA’s
• Particularly disease is associated with the autoantibodies anti-Ro (SS-A) and anti-La (SS-B)
• Associated with MHC-Class II alleles
• T cell mediated. It is possible that the cytoskeletal protein a-fodrin
• Other exocrine glands such as those lining the respiratory and GI tract as well as the vagina may be affected
• Lymphocytic infiltration can form lymphoid follicles
• The corneal epithelium may become dry, inflamed and ulcerated
• Dryness and crusting of the nose may lead to nasal ulceration and perforation of the nasal septum
• In around 25% of patients, extraglandular tissue is involved, kidneys, lungs, skin, CNS and muscles. This is more common in patients with high titres of SS-A antibody
• Glomerular lesions are extremely uncommon in comparison with SLE. However tubular defects are often seen such as renal tubular acidosis and phosphaturia

 

Clinical manifestations

• More common in women aged 50 to 60
• The keratoconjunctivis can result in blurring of vision, burning and itching and the production of thick secretions
• Xerostomia results in difficulty swallowing food, decreased ability to taste and cracks and fissures occurring in the mouth and dryness of the buccal mucosa
• Parotid gland enlargement occurs in 50% of patients
• Other symptoms are nasal dryness, epistaxis, recurrent bronchitis and pneumonitis
• Manifestations of extraglandular disease are synovitis, pulmonary fibrosis and peripheral neuropathy
• About 60% of patients have another recurrent autoimmune disease such as RA
• Mikulicz syndrome is the term used to described enlargement of the lacrimal and salivary gland. Causes include sarcoidosis, leukaemia and lymphoma
• Biopsy of the lip to look at minor salivary glands is required for the diagnosis of Sjorgen’s syndrome
• Patients have a 40-fold increased risk in developing lymphoid malignancy