Seminar G Sjorgens Syndrome

Question 1

Definition

Answer 1

Chronic autoimmune disease characterised by lymphocytic infiltrates and destruction of epithelial exocrine glands.

Question 2

Principal features

Answer 2

Keratoconjunctivitis (dry eyes)

Xerostomia (Dry mouth)

Question 3

Primary syndrome

Answer 3

Occurs alone

Question 4

Secondary Sjogrens

Answer 4

In association with other autoimmune rheumatic diseases such as RA, SLE and scleroderma

Question 5

Epi

Answer 5

More common in 9:1

Question 6

Autoimmune post viral infections

Answer 6

Cytomegalovirus (CMV) and EBV have been found in patients with Sjorgens

Question 7

Initial Presentation

Answer 7

Dry gritty eyes
Difficulty in swallowing dry foods
Problems with their teeth or dentures

Question 8

Progression of the disease organ symptoms

Answer 8

Constitutional symptoms
MSK aches and pains
Raynauds

Question 9

Constitutional

Answer 9

Fever, malaise

Question 10

Glandular

Answer 10

Xerostomia, dental caries
Parotid gland enlargement
Keratoconjunctivitis sicca, corneal ulceration
Dry vagina, dyspareunia

Question 11

MSK

Answer 11

Arthralgia, arthritis

Myalgia

Question 12

Skin

Answer 12

Raynaud’s phenomenon
Palpable purpura (Cutaneous vasculitis)

Question 13

Kidney

Answer 13

Interstial nephritis

Renal tubular acidosis and renal stones

Question 14

Lung

Answer 14

Interstial lung disease

Question 15

Lymphoreticular

Answer 15

Splenomegaly
Lymphadenopathy
Lymphoma

Question 16

Schirmer’s tests →

Answer 16

Strip of paper is placed beneath the lower eyelid and left for five minutes – wetness of paper <5mm is strongly suggestive of decreased tear secretion.

Question 17

Rose Bengal Stain

Answer 17

Placed on the conjunctival sac, damaged by corneal epithelium takes up the strain which is then easily visible under slit lamp examination.

Question 18

Sialography

Answer 18

Measuring the anatomy of the major salivary gland ducts using radiographic contrast material –can be used to assist diagnosis

Question 19

Gold standard is

Answer 19

Salivary gland biopsy, will show lymphocytic infiltrates

Question 20

Laboratory features include

Answer 20

Anaemia of chronic disease
Raised ESR (not usually raised CRP)
Raised Gammaglobulins
Anti-RO (or SSA)
Anti-La (SSB)

Question 21

Dry Eyes treatment

Answer 21

Artificial tears (prevents corneal scarring)

Question 22

Xerostomia treatment

Answer 22

• Drugs with antocholinergic side-effects avoided (tricyclic antidepressants)
• Sugar free lozangers
• Adequate oral hygience and dental followup
• Piocarpine is sometimes used, but this is hampered by systemic adverse effects

Question 23

Glucocorticoids

Answer 23

For Major Organ involvement Rare only for severe extraglandular disease such as renal involvement and vasculitis

Question 24

Risk of Malignancy →

Answer 24

Patient with Sjogrens Syndrome 40x relative risk of developing lymphoid malignancy vs. age sex and race matched controls.
• B cell origin
Treat