Systemic Sclerosis

Question 1

What is systemic sclerosis associated with.

Answer 1

Malignancy.

Question 2

What is systemic sclerosis.

Answer 2

Multisystemic connective tissue damage causing inflammation, fibrosis and vascular damage to the skin and internal organs.

Question 3

What can systemic sclerosis damage. (2)

Answer 3

Skin.

Internal organs.

Question 4

How many syndromes of systemic sclerosis are there.

Answer 4

Two.

Question 5

What are the two syndromes of systemic sclerosis.

Answer 5

Limited cutaneous systemic sclerosis.

Diffuse cutaneous systemic sclerosis.

Question 6

What are the characteristics of limited cutaneous systemic sclerosis. (3)

Answer 6

Skin involvement distally.
Systemic involvement occurs late and is rare.
Anticentromere antibody (ACA) positive in 70%.

Question 7

What percentage of patients with limited cutaneous systemic sclerosis are ACA positive.

Answer 7

70%.

Question 8

What are the characteristics of diffuse cutaneous systemic sclerosis. (2)

Answer 8

Early systemic involvement.

Scl-70 (topoisomerase) antibody positive in 30%.

Question 9

What percentage of patients with diffuse cutaneous systemic sclerosis are positive for Scl-70 antibody.

Answer 9

30%.

Question 10

What are the symptoms of systemic sclerosis. (14)

Answer 10

Pruritus. 
Raynaud's. 
Dysphagia. 
Nausea. 
Vomiting. 
Abdominal pain. 
Diarrhoea. 
Faecal incontinence. 
Shortness of breath. 
non-productive cough. 
Palpitations. 
Weakness. 
Arthralgia. 
Dry eyes.

Question 11

What are the major clinical features of systemic sclerosis. (3)

Answer 11

Centrally located skin sclerosis - arms, face and neck.

Question 12

What are the minor clinical features of systemic sclerosis. (4)

Answer 12

Sclerodactyly (tight skin over fingers).
Erosions.
Atrophy of the fingertips.
Bilateral lung fibrosis.

Question 13

What are the blood results in a patient with systemic sclerosis. (7)

Answer 13

Raised MCV. 
No change in ESR. 
Raised urea and creatinine (if there is renal involvement). 
Raised CK (mild). 
Raised Ig.
Raised ANA (90%).

Question 14

What can limited cutaneous systemic sclerosis do.

Answer 14

CREST syndrome (formerly known as).

Question 15

What are the features of CREST syndrome. (5)

Answer 15

Calcinosis (subcutaneous tissue). 
Raynaud's. 
oEsophageal and gut dismotility. 
Sclerodactyly (swollen tight digits). 
Telangiectasia.

Question 16

What skin involvement is there in limited cutaneous systemic sclerosis. (3)

Answer 16

Limited to the face hands and feet.

Question 17

What skin involvement is there in diffuse cutaneous systemic sclerosis.

Answer 17

Whole body in severe cases.