Connective Tissue Diseases

Question 1

What CTD is this: autoimmune condition characterised by lymphocytic infiltrates in exocrine organs

Answer 1

Sjogrens

Question 2

What is the classic presentation of sjogrens?

Answer 2

Dry eyes and mouth, vaginal dryness.

Other symptoms are more general: arthralgia, fatigue

Question 3

Which cancer risk is increased with Sjogrens?

Answer 3

Lymphoma

Question 4

What test do you do for Sjogrens and what is a positive result?

Answer 4

Schirmers test

Filter paper used to assess tear flow, less than 5mm on the paper in 5 mins indicates Sjogrens

Question 5

Which antibodies are associated with Sjogrens syndrome?

Answer 5

Anti-Ro and Anti-La

Question 6

What is the main concern with anti-ro and anti-la antibodies?

Answer 6

Neonatal complete heart block

Question 7

What are the typical features of Sjogrens found on a lip biopsy?

Answer 7

Tight clumps of lymphocytes adjacent to normal gland tissue

Question 8

What are the occular symptoms of Sjogrens?

Answer 8

Dry eyes

Foreign body sensation

Question 9

What are the oral symptoms of sjogrens?

Answer 9

Dry mouth

Recurrently swollen salivary glands

Question 10

How is Sjogrens managed?

Answer 10

Symptomatically
Lubricating eye drops
Saliva replacements
Pilocaprine
Immunosuppression

Question 11

When would immunosuppression be considered in Sjogrens

Answer 11

As with most rhuem conditions, when there is organ involvement

Question 12

Which CTD is this describing: an autoimmune condition characterised by diffuse fibrosis and vascular abnormalities

Answer 12

Systemic sclerosis

Question 13

What is the most common cause of death with systemic sclerosis?

Answer 13

Pulmonary hypertension

Question 14

What are the three phases of systemic sclerosis?

Answer 14

Odematous
Indurative
Atrophic

Question 15

What clinical sign is almost always present in systemic sclerosis?

Answer 15

Raynauds

Question 16

Which CTD is most strongly associated with calcinosis?

Answer 16

Systemic sclerosis

Question 17

What is major criteria for systemic sclerosis?

Answer 17

Central skin involvement: arms, face, neck

Question 18

What is minor criteria for systemic sclerosis?

Answer 18

Sclerodactyly and atrophy of fingertips

Bilateral lung fibrosis

Question 19

What may gut involvement in systemic sclerosis lead to?

Answer 19

Dysphagia, malabsorption and bacterial overgrowth

Question 20

What would qualify as diffuse systemic sclerosis?

Answer 20

Skins changes over the trunk
Early significant organ involvement
Anti-Scl-70

Question 21

What would qualify as limited systemic sclerosis?

Answer 21

Skin involvement tends to be confined to face, hands, forearms and feet
Anti-centromere antibody

Question 22

How is systemic sclerosis treated?

Answer 22

Pulmonary function testing
Echo
Monitoring of renal function
Autoantibody screen

Question 23

How is Raynauds managed?

Answer 23

CCBs
Iloprost
Bosentan

Question 24

How does bosentan work?

Answer 24

Dual endothelin receptor antagonism resulting in vasodilation

Question 25

How is renal involvement in systemic sclerosis treated?

Answer 25

ACEi

Question 26

How is lung involvement in systemic sclerosis treated

Answer 26

Immunosuppression - usually with cyclocphosphamide

As with most rhuem, immunosuppress once you get organ involvement

Question 27

What is mixed connective tissue disease?

Answer 27

A defined condition which has features of a variety of other CTDs

Question 28

Which antibody is most assocaited with mixed connective tissue disease?

Answer 28

Anti-RNP