Systemic Sclerosis

Question 1

Are scleroderma and systemic sclerosis the same?

Answer 1

No, technically not but the terms are used interchangeably

-Most patients with scleroderma have systemic sclerosis

Question 2

What is scleroderma?

Answer 2

Hardening of skin

Question 3

What is syetmic sclerosis?

Answer 3

Autoimmune inflammatory connective tissue disease

-Affects skin and internal organs

Question 4

How many types of systemic sclerosis are there?

Answer 4

Limited cutaneous systemic sclerosis

Diffuse cutaneous systemic sclerosis

Question 5

What did limited cutaneous systemic sclerosis used to be known by?

Answer 5

CREST syndrome

Question 6

What does CREST syndrome stand for?

Answer 6

C- Calcinosis
R- Reynaud's phenomenon 
E- oEsophageal dysmotility 
S- Sclerodactyly 
T- Telangiectasia

Question 7

What is diffuse cutaneous systemic sclerosis?

Answer 7

Affects internal organs + features of CREST

Question 8

Features of DCSS?

Answer 8

• Scleroderma
• Sclerodactyly
• Telangiectasia
• Calcinosis
• Reynauds
• Systemic/pulmonary HT
• Pulmonary fibrosis
• Oesophageal dysmotility
• Scleroderma renal crisis

Question 9

Auto-antibodies in diffuse cutaneous systemic slcerosis?

Answer 9

ANA = +ve
Anti-centromere ABs (LCSS)
Anti-Scl- 70 ABs (DCSS)

Question 10

Diagnosis of diffuse cutaneous systemic sclerosis?

Answer 10

Nailfold capillaroscopy

Based on classification criterias

Question 11

What is nailfold capillaroscopy?

Answer 11

• Examine skin where it meets fingernail
• Examines health if peripheral capillaries
• Shows abnormal capillaries, avascular areas and micro-haemorrhages

Question 12

Management of diffuse cutaneous systemic sclerosis?

Answer 12

Steroids

Immunosuppressants

Question 13

Non-medical treatmnet of SS?

Answer 13

Regular emollients

Avoiding cold

Question 14

Medical treatment of systemic sclerosis?

Answer 14

• Nifedipine
• Anti-acid (PPIs)
• Analgesia
• ABs
• Anti-hypertensives
• Supportive