[7] Sjögren’s syndrome Flashcards
What is Sjögren’s syndrome?
A long-term autoimmune disease affecting the body’s moisture producing glands
What is the current thought of pathophysiology of Sjögren’s syndrome?
Specific, self-perpetuating, immune-system mediated loss of exocrine glands
What cells are particularly affected in Sjögren’s syndrome?
- Acinar cells
- Ductal cells
Why is the current understanding of Sjögren’s syndrome limited?
It can explain some symptoms but not the systemic effects of the disease
What is thought to contribute to Sjögren’s syndrome?
- Genetic factors
- Environmental factors
- Other factors
What are the risk factors for Sjögren’s syndrome?
- Female
- Other autoimmune diseases
- 20-30 years
- Post-menopausal
- Family history
- HLA class II markers
What other autoimmune disorders increase risk of Sjögren’s syndrome?
- SLE
- RA
- Scleroderma
What are the common presenting symptoms of Sjögren’s syndrome?
- Dry mouth
- Keratoconjunctivtis sicca
- Vaginal dryness
- Dry skin
- Dry nose
What are other symptoms suggestive of a dry mouth seen in Sjögren’s syndrome?
- Recurrent mouth infections
- Swollen parotid glands
- Dysphonia
- Difficulty swallowing and eating
What symptoms can suggest keratoconjunctivitis sicca in Sjögren’s syndrome?
- Blurred vision
- Constant eye discomfort
What other organs of the body can be affected by Sjögren’s syndrome?
- Kidneys
- Blood vessels
- Lungs
- Liver
- Pancreas
- Brain
What systemic symptoms can present in Sjögren’s syndrome?
- Debilitating fatigue
- Joint pain
- QoL impairment
What are the first line investigations for Sjögren’s syndrome?
- Schirmer’s test
- Anti-60 kD (SS-A) Ro and anti-La (SS-B)
What is Schirmer’s test for Sjögren’s syndrome?
Filter paper is placed in the lower conjunctival sac and allowed to get wet for 5 minutes
What is a positive Schirmer’s test in Sjögren’s syndrome?
Less than 5mm of filter paper is wetted after 5 minutes
What % of patients with Sjögren’s syndrome have anti-60kD Ro and anti-La antibodies?
Up to 90%
Which Sjögren’s syndrome antibody is not specific?
Anti-60 kD Ro
What other conditions is anti-60 kD Ro antibody found in?
Other autoimmune disorders especially lupus
What is the level of anti-60 kD Ro and anti-La correlated with?
- Earlier onset of disease
- Longer disease duration
- Recurrent parotid gland enlargement
- Extra-glandular manifestations
What other investigations may be useful in Sjögren’s syndrome?
- Sialometry
- Minor salivary gland biopsy
- Parotid sialography
- Skin biopsy
- Angiography
- Urinalysis
What will sialometry show in Sjögren’s syndrome?
Decreased salivary flow
When is angiography useful in Sjögren’s syndrome?
When associated vasculitis with large vessel involvement
When is urinalysis important in Sjögren’s syndrome?
When there is suspected renal tubular acidosis
What are the differentials of Sjögren’s syndrome?
- Medication side-effects
- Anxiety
- Sarcoidosis
- Amyloidosis
- SLE
- Scleroderma
What medications can cause Sjögren’s syndrome-like side effects?
- Oral contraceptive
- Antihistamines
- Beta-blockers
- Phenothiazine
- Atropine
What is the mainstay of Sjögren’s syndrome treatment focused on?
Symptom management and supportive therapy
What symptoms can be managed in Sjögren’s syndrome?
- Dry eyes
- Dry mouth
- MSK symptoms
- Vasculitis
- Renal tubular acidosis
- Neuropathy
How can dry eyes be treated in Sjögren’s syndrome?
Artificial tears with ciclosporin drops or cholinergic drugs if insufficient
How can dry mouth be treated in Sjögren’s syndrome?
Salivary substitutes with addition of cholinergic drugs if insufficient
How can MSK symptoms be managed in Sjögren’s syndrome?
- Paracetamol
- NSAIDs
- With or without corticosteroids, hydroxychloroquine or methotrexate if insufficient
How can vasculitis be managed in Sjögren’s syndrome?
- Corticosteroids
- IV immunoglobulin if insufficient
How can renal tubular acidosis be managed in Sjögren’s syndrome?
Potassium repletion and alkali
How can neuropathy be treated in Sjögren’s syndrome?
IV immunoglobulin
What are the potential complications of Sjögren’s syndrome?
- Associated with other autoimmune disease
- Associated with non-Hodgkin lymphoma
- Increased risk of neonatal lupus erythematosus and congenital heart block in babies of women with Sjögren’s syndrome
