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A - MSRA ENT > Sjögren’s syndrome > Flashcards

Sjögren’s syndrome Flashcards

1
Q

What is Sjögren’s syndrome?

A

A long-term autoimmune disease affecting the body’s moisture producing glands

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2
Q

What is the current thought of pathophysiology of Sjögren’s syndrome?

A

Specific, self-perpetuating, immune-system mediated loss of exocrine glands

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3
Q

What cells are particularly affected in Sjögren’s syndrome?

A
  • Acinar cells

- Ductal cells

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4
Q

Why is the current understanding of Sjögren’s syndrome limited?

A

It can explain some symptoms but not the systemic effects of the disease

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5
Q

What is thought to contribute to Sjögren’s syndrome?

A
  • Genetic factors
  • Environmental factors
  • Other factors
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6
Q

What are the risk factors for Sjögren’s syndrome?

A
  • Female
  • Other autoimmune diseases
  • 20-30 years
  • Post-menopausal
  • Family history
  • HLA class II markers
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7
Q

What other autoimmune disorders increase risk of Sjögren’s syndrome?

A
  • SLE
  • RA
  • Scleroderma
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8
Q

What are the common presenting symptoms of Sjögren’s syndrome?

A
  • Dry mouth
  • Keratoconjunctivtis sicca
  • Vaginal dryness
  • Dry skin
  • Dry nose
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9
Q

What are other symptoms suggestive of a dry mouth seen in Sjögren’s syndrome?

A
  • Recurrent mouth infections
  • Swollen parotid glands
  • Dysphonia
  • Difficulty swallowing and eating
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10
Q

What symptoms can suggest keratoconjunctivitis sicca in Sjögren’s syndrome?

A
  • Blurred vision

- Constant eye discomfort

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11
Q

What other organs of the body can be affected by Sjögren’s syndrome?

A
  • Kidneys
  • Blood vessels
  • Lungs
  • Liver
  • Pancreas
  • Brain
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12
Q

What systemic symptoms can present in Sjögren’s syndrome?

A
  • Debilitating fatigue
  • Joint pain
  • QoL impairment
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13
Q

What are the first line investigations for Sjögren’s syndrome?

A
  • Schirmer’s test

- Anti-60 kD (SS-A) Ro and anti-La (SS-B)

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14
Q

What is Schirmer’s test for Sjögren’s syndrome?

A

Filter paper is placed in the lower conjunctival sac and allowed to get wet for 5 minutes

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15
Q

What is a positive Schirmer’s test in Sjögren’s syndrome?

A

Less than 5mm of filter paper is wetted after 5 minutes

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16
Q

What % of patients with Sjögren’s syndrome have anti-60kD Ro and anti-La antibodies?

A

Up to 90%

17
Q

Which Sjögren’s syndrome antibody is not specific?

A

Anti-60 kD Ro

18
Q

What other conditions is anti-60 kD Ro antibody found in?

A

Other autoimmune disorders especially lupus

19
Q

What is the level of anti-60 kD Ro and anti-La correlated with?

A
  • Earlier onset of disease
  • Longer disease duration
  • Recurrent parotid gland enlargement
  • Extra-glandular manifestations
20
Q

What other investigations may be useful in Sjögren’s syndrome?

A
  • Sialometry
  • Minor salivary gland biopsy
  • Parotid sialography
  • Skin biopsy
  • Angiography
  • Urinalysis
21
Q

What will sialometry show in Sjögren’s syndrome?

A

Decreased salivary flow

22
Q

When is angiography useful in Sjögren’s syndrome?

A

When associated vasculitis with large vessel involvement

23
Q

When is urinalysis important in Sjögren’s syndrome?

A

When there is suspected renal tubular acidosis

24
Q

What are the differentials of Sjögren’s syndrome?

A
  • Medication side-effects
  • Anxiety
  • Sarcoidosis
  • Amyloidosis
  • SLE
  • Scleroderma
25
Q

What medications can cause Sjögren’s syndrome-like side effects?

A
  • Oral contraceptive
  • Antihistamines
  • Beta-blockers
  • Phenothiazine
  • Atropine
26
Q

What is the mainstay of Sjögren’s syndrome treatment focused on?

A

Symptom management and supportive therapy

27
Q

What symptoms can be managed in Sjögren’s syndrome?

A
  • Dry eyes
  • Dry mouth
  • MSK symptoms
  • Vasculitis
  • Renal tubular acidosis
  • Neuropathy
28
Q

How can dry eyes be treated in Sjögren’s syndrome?

A

Artificial tears with ciclosporin drops or cholinergic drugs if insufficient

29
Q

How can dry mouth be treated in Sjögren’s syndrome?

A

Salivary substitutes with addition of cholinergic drugs if insufficient

30
Q

How can MSK symptoms be managed in Sjögren’s syndrome?

A
  • Paracetamol
  • NSAIDs
  • With or without corticosteroids, hydroxychloroquine or methotrexate if insufficient
31
Q

How can vasculitis be managed in Sjögren’s syndrome?

A
  • Corticosteroids

- IV immunoglobulin if insufficient

32
Q

How can renal tubular acidosis be managed in Sjögren’s syndrome?

A

Potassium repletion and alkali

33
Q

How can neuropathy be treated in Sjögren’s syndrome?

A

IV immunoglobulin

34
Q

What are the potential complications of Sjögren’s syndrome?

A
  • 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

A - MSRA ENT (22 decks)

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