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Sjogren’s Syndrome

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Overview

Sjögren’s syndrome is an autoimmune condition of unknown aetiology affecting the exocrine glands leading to the main symptoms of dry eyes (xerophthalmia), dry mouth (xerostomia), and enlargement of the parotid glands. It can happen in isolation or be secondary to another autoimmune condition such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), or systemic sclerosis (SSc).

There is a significantly increased risk of developing lymphoid malignancy (around 60 times as high)

“Sicca” refers to the dryness of the eyes and mouth.

Epidemiology

  • Age of onset is usually 30-40 years of age
  • 20 times more common in women
  • Affects women more severely than men

Risk Factors & Associations

  • Female
  • History of rheumatological disease: RA, SLE, or SSc
  • Patients may develop primary biliary cirrhosis or chronic hepatitis

Presentation

  • Dry eyes
    • Patients often complain of the feeling of sand/gravel in their eyes, itch, or burning
    • They may also have red eyes and sensitivity to light and wind
  • Dry mouth
  • Fatigue
  • Vaginal dryness
  • Recurrent parotitis
  • Enlarged parotid glands
  • Chronic pain – myalgia and polyarthralgia
  • Raynaud’s phenomenon
  • Peripheral polyneuropathy (rare)
  • Cranial polyneuropathy (facial or trigeminal nerves) (rare)
  • Vasculitis causing mononeuritis multiplex (rare)

Signs on Examination

  • Eye dryness:
    • Lack of tear pool
    • Blepharitis
    • Dilated conjunctival vessels
  • Dry mouth:
    • Wooden tongue depressor may stick to the tongue
  • Bilateral enlargement of the parotid glands
    • A unilateral and hard salivary gland should prompt immediate referral
  • Features of other autoimmune diseases

Differential Diagnoses

Rheumatoid arthritis (RA)

  • RA has joint pains and morning stiffness, typically in the hands and feet
  • Dry eyes, dry mouth, and enlarged parotid glands are not generally seen in RA

Systemic lupus erythematosus (SLE)

  • Photosensitivity present
  • Malar rash present
  • Other features of SLE present

Drug-induced sicca

  • Drugs are: oral contraceptives, antihistamines, beta-blockers, phenothiazine, or atropine

Idiopathic sicca

  • Atrophic glands and fibrosis on salivary gland biopsy

Investigations

  • Schirmer’s test:
    • Filter paper is placed in the lower eyelid
    • The test is positive if <5mm of paper is wet after 5 minutes
  • Anti-60kD (SS-A) Ro and anti-La (SS-B) antibodies:
    • These are found in up to 90% of patients
    • A good way of remembering these is ORAL – La and Ro backwards
  • Salivary gland biopsy should be taken if patients have a unilaterally enlarged salivary gland
    • This is to rule out malignancy

Management

Management primarily involves treating the symptoms:

  • Artificial saliva for dry mouth
  • Artificial tears for dry eyes
  • NSAIDs for musculoskeletal symptoms

Corticosteroids are given in vasculitis

Monitoring

Patient Advice

  • Patients should be educated about using artificial tears and saliva for not only symptomatic relief but preventing damage from being done to the eyes and mouth
  • Anticholinergics, antihistamines, and diuretics can worsen eye dryness
  • Patients should use emollients on dry and cracking skin
  • Patients should have adequate dental care

Complications

  • Blepharitis
  • Dry eyes and blurry vision
  • Raynaud’s phenomenon
  • Lymphoma – up to 60 times increased risk
  • Parotid gland tumours
  • Anaemia of chronic disease
  • Corneal ulceration
  • Infection of the eye secondary to dryness
  • Oral candidiasis
  • Neurological involvement – autonomic dysfunction
  • Pulmonary fibrosis

Prognosis

  • Prognosis is generally good unless there’s an associated disorder. There is still significant morbidity
  • Patients with a low C4 count have a higher risk of developing non-Hodgkin’s lymphoma and have a worse prognosis with higher mortality rates

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