Systemic Sclerosis Flashcards

1
Q

What are the two types of systemic sclerosis?

A
Limited/Cutaneous= Scleroderma (formerly called CREST)
Diffuse= Systemic sclerosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the features of the limited type/cutaneous sclerosis ?

A

This was previously called crest syndrome

It just affects the hands, feet and face

Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly 
Telangiectasia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What antibody is associated with limited sclerosis?

A

Anti-centromere

Think mere- it’s merely limited disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is an important complication or development of limited scleroderma?

A

Pulmonary hypertension which can become life threatening

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the treatment for pulmonary hypertension in limited systemic sclerosis?

A

Sildenafil

Bosetin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What does sclerodactyly look like?

A

Inability to fully extend fingers

The ischaemia created due to inflammation can cause digital ulcers to form.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Where does diffuse systemic sclerosis affect?

A

The whole body- it causes scleroderma and fibrosis of the internal organs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What antibody is systemic sclerosis?

A

Anti-SCL 70

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the management for systemic sclerosis?

A

Immunosuppression
Steroids and steroid sparing regimes
E.g. Methotrexate, Azathioprine, cyclophosphamide, Rituximab (Anti CD20/Anti B Cell)
Anti-fibrotic agents- under investigation for pulmonary fibrosis

Manage symptoms
Raynaud’s- Nifedipine
GORD- Omeprazole
PHTN- Sildenafil, Bosetin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly