Sjögren’s Syndrome – At A Glance

Sjögren’s Syndrome is an autoimmune disease affecting the exocrine glands resulting in dryness of the mucous membranes.

It is a chronic disease that will reduce the quality of life. 

As there is no cure the main aim is to reduce the troublesome symptoms to improve the quality of life.

There is a significant increase in risk of developing lymphoma
Incidence : 4 in 100 000 individuals 

Females are commonly affected – M : F ratio is 9 : 1

Types Of Sjögren’s Syndrome

  • Primary Sjögren’s Syndrome
    • More severe than secondary Sjögren’s syndrome
  • Secondary Sjögren’s Syndrome
    • Secondary to rheumatoid arthritis, SLE
    • Has a mild presentation

Clinical Presentation Of Sjögren’s Syndrome

  • Presentation is around 40 – 60 years
  • Sicca symptoms
    • Dry eyes -Keratoconjuntivitis sicca
    • Dry mouth
  • Dry skin
  • Vaginal dryness
  • Bilateral pituitary swelling -Seen in children
  • Extra-glandular involvement (Late manifestations)
    • Vasculitis – Rash after exposure to sun
    • Athralgia, myalgia
    • Renal tubular acidosis – Subclinical presentation
    • Peripheral neuropathy
    • Can affect the lungs. liver and GI tract as well
  • Schirmer test
    • Less than 5 mm after 5 minutes is considered as a positive test 

Investigations Of Sjögren’s Syndrome

  • Complete blood count
    • Anaemia
    • Leukopenia
  • Rheumatoid factor -Present in almost all cases
  • ANA – Present in 70% of cases
    • Anti-SSA/Ro 
    • Anti-SSB/La
  • Hypergammaglobulinemia
  • C4 – Reduced
  • Histology – Salivary gland biopsy
    • Focal lymphocytic infiltration
Management Of Sjögren’s Syndrome
There is no cure for Sjögren’s syndrome, aim is to reduce the symptoms and improve the quality of life
  • Artificial tears – To prevent keratopathy
  • Artificial saliva
  • Pilocarpine – Stimulate saliva production 
  • Skin lotions 
  • Vaginal lubricants
  • NSAIDs – Athralgia and myalgia
  • Severe extra-glandular involvement 
    • Treated with immunossupressive agents (Cyclophosphamide), monoclonal antibodies, immunoglobulins 
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