Etiology:

• Immunologic disease – Lymphocyte mediated destruction of exocrine glands – Xerostomia and keratoconjunctivitis sicca
• Lymphocytic infiltration and acinar destruction of lacrimal and salivary glands
• Autoimmune destruction in patients with the following HLA types:
  • Primary: HLA-B8, HLA-DR3
  • Secondary: HLA-DR4

(HLA – Human leukocyte antigen)

• This induces production of numerous autoantibodies:
  • Rheumatoid factor
  • Antinuclear antibodies (ANA)
  • Anti-Sjogren syndrome A (SS-A)
  • Anti-Sjogren syndrome B(SS-B)

Epidemiology:

• 90% women
• Swedish women
• Onset at 50 years

Classification:

• Primary:
  • Keratoconjunctivitis sicca (dry eyes)
  • Xerostomia (dry mouth)
• Exocrine glands only
• 80% unilateral/ bilateral salivary gland swelling

• Secondary:
  • Keratoconjunctivitis sicca (dry eyes)
  • Xerostomia (dry mouth)
  • Autoimmune condition, eg.:
    • Rheumatoid arthritis
    • SLE
    • Systemic sclerosis
    • 1ry biliary cirrhosis
• Exocrine glands + systemic/autoimmune disease
• 30-40% unilateral/ bilateral salivary gland swelling
• Rule out lymphoma

Clinical presentation:

Extreme tiredness due to multisystem effects

1. Glandular manifestations:

• Salivary – Xerostomia
• Lacrimal – Xerophthalmia
• Skin – Xeroderma
• Respiratory tract – Nasal dryness, sinusitis, tracheitis
• Pharynx and GIT – Dysphagia, atrophic gastritis, pancreatitis
• Oral cavity – Salivary gland enlargement, glossitis, mucositis
• Reproductive – Mucosal dryness

2. Extraglandular manifestation:

• Joints – Arthritis
• Skin – Purpura, Raynaud’s phenomenon
• Liver – 1ry biliary cirrhosis
• Renal – Renal tubular defects
• Endocrine – Thyroiditis
• Neurological – Neuropathy
• Hematological – Decreased RBC, WBC, Platelets
• Immunological – Autoantibodies,

Complications:

1. Candidiasis
2. Ascending sialadenitis
3. Pseudolymphoma, B-cell lymphoma
4. Dental caries

Diagnosis:

1. Schirmer’s test:

• 5 x 35mm strips of red litmus paper – place inside lower eyelid in inferior fornix
• Leave for 5 minutes – assess how far the tears have travelled
• +ve = lacrimation of ≤ 5mm
• 85% specific and sensitive

2. Sjogren’s lip biopsy:

• Shallow horizontal incisions made of 1.5 to 2cm on either side of inner lip (numb with LA)
• Approx 5-7 glands removed with sterile tweezers
• Close incisions with resorbable suture
• Assess number of foci of lymphocytes/4mm²/gland

3. Sialography:

• Snow storm or cherry blossom appearance

4. Scintiscanning:

• Technetium-99m pertechnetate

5. Serology:

• SS-A
• SS-B
• ANA
• Anti-dsDNA

6. FBC:

• Anemia
• Leukoplakia
• Eosinophilia

Histology:

• Replacement of gland parenchyma by inflammatory infiltrate with remnants of epimyoepithelial cells
• Therefore there is:
  • Lymphocytic infiltration
  • Acinar atrophy
  • Proliferation of duct epithelium
  • Epimyoepithelial islands

Management:

• Salivary substitutes/ sprays
• Sugar free gum
• Sialagogue eg. pilocarpine
• No alcohol/ tobacco
• Avoid xerostomic meds
• Palliative care + artificial saliva/tears

NB: Normal saliva flow 1-2 ml per min. Sjogren’s: 0.5 ml/min or less

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