Sjogren's Syndrome

Question 1

What is Sjögren’s syndrome?

Answer 1

An autoimmune condition that affects the exocrine glands, leading to dryness of the mucous membranes e.g. mouth, eyes, vagina.

Question 2

What is the difference between Primary and Secondary Sjögren’s syndrome?

Answer 2

Primary = Isolation.
Secondary = Related to SLE or RA (usually 10 years later).

Question 3

What antibodies are associated with Sjögren’s syndrome? (2).

Answer 3

1. Anti-Ro.
2. Anti-La.
   * Other - RF, ANA.

Question 4

Epidemiology of Sjögren’s syndrome.

Answer 4

9x commoner in females.

Question 5

Clinical Features of Sjögren’s syndrome (8).

Answer 5

1. Keratoconjunctivitis Sicca (Dry Eyes).
2. Xerostomia (Dry Mouth).
3. Vaginal Dryness & Dyspareunia.
4. Arthralgia.
5. Raynaud’s, Myalgia.
6. Sensory Polyneuropathy.
7. Recurrent Parotitis.
8. Subclinical Renal Tubular Acidosis.

Question 6

What is the investigation of Sjögren’s syndrome?

Answer 6

Schirmer Test : insert a folded piece of filter paper under the lower eyelid with a string hanging out over the eyelid for 5 minutes and measure distance of moisture.

Question 7

Results of Schirmer Test in Sjogren’s Syndrome.

Answer 7

15mm in a healthy young adult; less than 10mm is significant.

Question 8

Other Investigations in Sjögren’s syndrome (3).

Answer 8

1. Histology : Focal Lymphocytic Infiltration.
2. Bloods : Hypergammaglobulinaemia and Low C4.
3. Other Antibodies : RF, ANA.

Question 9

Management of Sjögren’s syndrome (4).

Answer 9

1. Artificial Tears e.g. Hypromellose.
2. Artificial Saliva or Pilocarpine to stimulate salivation.
3. Vaginal Lubricants.
4. Hydroxychloroquine - halt progression of disease.

Question 10

Complications of Dry Mucous Membranes (3).

Answer 10

1. EYE - Infections e.g. Conjunctivitis, Corneal Ulcers.
2. ORAL - Dental Cavities, Candida Infection.
3. VAGINAL - Candidiasis and Sexual Dysfunction.

Question 11

Systemic Complications of Sjögren’s syndrome (5).

Answer 11

1. Pneumonia and Bronchiectasis.
2. Non-Hodgkin’s Lymphoma.
3. Peripheral Neuropathy.
4. Vasculitis.
5. Renal Impairment.

Question 12

Prognosis of Sjögren’s syndrome.

Answer 12

Marked increased risk of lymphoid malignancy (40-60 fold).

Question 13

Pathophysiology of Sjögren’s syndrome.

Answer 13

Type IV Hypersensitivity Reaction - Lymphocyte-Mediated Autoimmune Destruction of minor salivary and lacrimal glands, leading to atrophy and fibrosis.