Systemic Sclerosis

Question 1

What is the epidemiology of Systemic Sclerosis?

Answer 1

Middle aged women

Question 2

What are the two types if Systemic Sclerosis?

Answer 2

Limited cutaneous - 70%

Diffuse - 30%

Question 3

What are the features of limited cutaneous Systemic Sclerosis?

Answer 3

CREST syndrome
C - Clacinosis
R - Raynaud's
E - Oesophageal/gut dysmotility = GORD
S - Sclerodactyly
T - Telangiectasia
*Skin involvement limited to hands, face and feet - Microstomia, Beak nose*
15% have pulmonary HTN

Question 4

What are the features of diffuse Systemic Sclerosis?

Answer 4

Diffuse skin involvement + organ fibrosis
GI - GORD, Incontinence, Dysphagia
Lung - Fibrosis, Pulmonary HTN
Renal - Acute hypertensive crisis - common cause of death
Cardiac - Arrhythmias, conduction deficits

Question 5

What investigations can be done in a patient with suspected Systemic Sclerosis?

Answer 5

Bloods - FBC (Anaemia) U+Es (Renal impairment), Antibodies
Urine - Dipstix (Proteinuria, Haematuria)
CXR - Cardiomegaly, Bibasal fibrosis
Hand x ray - Calcinosis
Barium swallow - impaired oesophageal motility
High resolution CT - lung fibrosis (Honeycombing)
Echo

Question 6

What is the conservative management of Systemic Sclerosis?

Answer 6

Exercise and skin lubricants - reduce contractures

Hand warmers for Raynauds

Question 7

What is the medical management of Systemic Sclerosis?

Answer 7

Immunosuppressants
Raynauds - Ca channel blockers (Nifedipine - Dihydropiridine), ACEi, IV prostacyclin
Renal - ACEi
PPIs, Prokinetics eg metoclopramide
Bosentan if pulmonary HTN