Sjögren’s syndrome

Inflammation and fibrosis of exocrine glands with Dry eyes, dry mouth and dry vagina and systemic symptoms

About

• Autoimmune destruction of lacrimal and salivary glands
• Dry eyes and dry mouth are called the sicca syndrome.
• Dry mouth xerostomia, dry eyes xerophthalmia.

Types

• Primary Sjögren’s syndrome (PSS): Autoimmune disease seen in middle-aged older lady and 50% are RF positive
• Secondary Sjögren’s syndrome: symptoms come on with
  • Rheumatoid arthritis
  • SLE, Scleroderma
  • Primary Biliary Cirrhosis. 95% RF positive

Aetiology

• Chronic Lymphocytic infiltration and fibrosis of exocrine glands
• Mainly lacrimal and salivary: dry mouth and dry eyes
• Autoimmune disease which presents with dry mouth, eyes, skin and vagina
• Association with HLA B8/DR3

Clinical

• Females x 9 and onset age 30-40s
• Dry Mouth: dental caries, parotitis, bad breath
• Keratoconjunctivitis sicca: dry gritty eyes + corneal + conjunctival irritation. Positive Schirmer test
• Vaginal dryness : dyspareunia
• Arthritis/Arthralgia: which can be erosive
• Vasculitis, Raynaud's phenomenon
• Fatigue, Myalgia, Myositis, Arthralgia, Lymphadenopathy
• Vasculitis purpura, neuropathies, Pancreatitis
• Renal tubular acidosis

Associations

• Thyroid disease
• Autoimmune hepatitis
• Primary biliary cirrhosis
• Non-Hodgkin's B-cell lymphoma.

Investigations

• FBC: anaemia, low WCC can be seen
• Elevated ESR/CRP reflext disease activity
• Typically rheumatoid factor is +ve in 38%.
• ANA positive in 80% cases
• Anti-Ro (SSA in 60%) & anti-La (SSB in 26%) antibodies may be present (in pregnancy, these cross the placenta and cause fetal congenital heart block in 5%)
• Polyclonal hypergammaglobulinaemia
• Schirmer's test: measures conjunctival dryness. A strip of filter paper placed on lower lid with wetting. Less than <5mm in 5min is +ve and confirms defective tear production.
• Rose Bengal score or other ocular dye score shows punctate or filamentary keratitis (use a slit-lamp).
• Labial gland biopsy can aid diagnosis. May show lymphocytic infiltration of the exocrine glands

Management

• Treatment symptomatic with artificial tears/saliva replacement solutions
• Symptomatic: Dry mouth so drinking plenty and moistening mouth sprays
• Xerophthalmia: Artificial tears (e.g. Hypromellose), eye lubricant ointments. Ophthalmic review.
• Ciclosporin eyedrops recently approved in the US
• NSAIDS and Hydroxychloroquine for arthritis
• Systemic disease - Hydroxychloroquine, Methotrexate and steroids for systemic problems

References