Connective Tissue Disease 2 - Scleroderma

Question 1

What is scleroderma?

What are the two presentations?

Answer 1

It means ‘hard skin’ so it is the formation of fibrous tissue and there are two distinct presentations:

LOCALISED (eg morphea) or SYSTEMIC

1) Diffuse systemic Scleroderma
2) Limited systemic Scleroderma

Question 2

What antibody is diffuse Scleroderma associated with?

Answer 2

Anti - Scl70

Question 3

What antibody is limited cutaneous Scleroderma associated with?

Answer 3

Anti centromere antibodies

Question 4

What are the characteristics of diffuse Scleroderma ?

Answer 4

• Any of the CTD symptoms
• Thickening of skin proximal to elbow
• constitutional symptoms
• Increased chance of organ involvement i.e renal failure, pulmonary hypertension, GI symptoms

Question 5

What are the characteristics of limited Scleroderma?

Answer 5

Any of the CTD symptoms

thickening of skin distal to elbow

CREST

Calcinosis

Raynauds

Esophogeal dysmobility

Sclerodactyly

Telengectasia

Organ involvement is rare and there No consitutional symptoms

Question 6

What is this and what do you get it in?

Answer 6

Calcinosis

Limited cutenous systemic sclerosis

Question 7

What is this and when do you get it?

Answer 7

Calcinosis

Limited cutenous systemic sclerosis

Question 8

What is this and when do you get it?

Answer 8

Sclerodactyly

Limited cutenous systemic sclerosis

Question 9

What is this and when do you get it?

Answer 9

Limited Scleroderma on the face

Question 10

What antibody is positive in both systemic and localised cuteanous systemic sclerosis?

Answer 10

ANA

Question 11

What is the treatment for the disease?

Answer 11

There is no disease modifying treatment so the aim is to tackle symptoms.

1. ACE inhibitors to target renal failures
2. PPI to target oesophageal incompetence
3. ciprofloxacin to stop bacterial overgrowth in midgut

Question 12

What is morphea?

Answer 12

Localised single area of scleroderma