Systemic Sclerosis (Scleroderma) - Week 17

Question 1

What is systemic sclerosis and scleroderma?

Answer 1

Systemic sclerosis - autoimmune inflammatory and fibroid connective tissue disease. Affects skins and organs

Scleroderma - hardening of the skin

Question 2

What are the two types of systemic sclerosis?

Answer 2

Limited cutaneous systemic sclerosis (lcSScl)
Diffuse cutaneous systemic sclerosis (dcSScl)

Question 3

Epidemiology of systemic sclerosis?

Answer 3

Female - 40 to 50 years

Question 4

Pathophysiology of systemic sclerosis?

Answer 4

Autoimmune - unclear

Question 5

Presentation of limited cutaneous systemic sclerosis?

Answer 5

C - Calcinosis
R - Raynaud’s
E - oEsophageal dysmotility
S - Sclerodacyly
T - Telangiectasia

Extremities

Question 6

Presentation of diffuse cutaneous systemic sclerosis?

Answer 6

CREST from limited AND:

Cardiovascular, lung and kidney involvement

Chest, trunk, upper arms shoulder involvement

Question 7

Investigation of systemic sclerosis?

Answer 7

Positive ANA (non specific antibody)

Positive Anti-centromere (Limited)
Positive Anti-Scl-70 (diffused)

Nailfold Capillaroscopy - abnormal in SS

EULAR criteria for diagnosis

Question 8

Management of systemic sclerosis?

Answer 8

Steroids (not high dose) and immunosuppressants

Question 9

Additional presentation of scleroderma?

Answer 9

Skin tightening on fingers hands and face (sclerodactyly)
Itching
Digital ulcers
Renal disease