Systemic sclerosis

Question 1

What is systemic sclerosis?

Answer 1

autoimmune inflammatory and fibrotic connective tissue disease

Question 2

Scleroderma?

Answer 2

Is the localised hardening of the skin and only affects the skin

Question 3

What are the two patterns of disease in systemic sclerosis?

Answer 3

Limited cutaneous SS

Diffuse cutaneous SS

Question 4

What are the features of limited cutaneous?

Answer 4

Skin changes limited up to just the elbow
Calcinosis
Raynauds
oEsophageal dysmotility 
Sclerodactyly
Telengiectasia

Question 5

What are the features of diffuse cutaneous SS?

Answer 5

"Malignant scleroderma"
CREST +
Microstomia 
Tethering of skin 
renal failure secondary to severe HTN
Pulmonary fibrosis - restrictive ventilatory defect + pulmonary HTN
Oesophageal atony --> reflux & aspiration 
Cardiovascular --> HTN + CAD

Question 6

3 key questions in the history?

Answer 6

1. Do you hands change colour in the cold - white, blue or red?
2. Do you get breathless?
3. Do you get indigestion or heartburn?

Question 7

Features of clinical presentation in systemic sclerosis?

Answer 7

Scleroderma - tight waxy skin without normal folds
Sclerodactyly - reduces ROM 
Microstomia
Beaking - tethering of skin around nose 
Telengiectasia ---> small dilated blood vessels in the skin 
Raynaud's 
Oesophageal dysmotility 
Systemic & pulmonary HTN
Pulmonary fibrosis 
Renal failure + HTN --> renal crisis 

Telengiectasia - on face,

Question 8

What to look for in examination?

Answer 8

Scleroderma & dactyly
Palpate for temp
Skin tethering 
Calcifications on extensor aspects of hands 
Check hand function - prayer sign
Three finger test for microstomia

Question 9

Investigations

Answer 9

ANA
Anti-centromere for Limited
Anti-Scl70 for diffuse
Nail fold capillaroscopy

Question 10

Diagnosis

Answer 10

Based on ACR and ELAR classification criteria –> clinical aspects, antibodies & nailfold capillaroscopy

Question 11

Management

Answer 11

No formal proven tx for SS

Steroids & immunosuppressants mainstay

Question 12

Conservative management

Answer 12

Physiotherapy to maintain ROM
Regular stretching to maintain ROM
Emollients for scleroderma
Avoiding cold triggers for raynauds

Question 13

Medical management

Answer 13

of symptoms 
Nifedipine for Raynauds
Omeprazole for indigestion 
Metoclopramide for GI --> pro-motility
Analgesia for joint pain 
Antihypertensives for HTN 
Pulmonary artery HTN - sildenafil or bosentan 
Supportive management for pulmonary fibrosis