Systemic sclerosis

Question 1

Define Systemic sclerosis

Answer 1

also called scleroderma

2 types-
Limited cutaneous Systemic sclerosis–only affect hands and forearm/feet lets
They mainly have anticentromere

Diffuse cutaneous Systemic sclerosis

Question 2

Signs and Sx of Systemic sclerosis

Answer 2

CREST acronym (the e is oesgophagus

Calcisosis-often quite striking white
Raynauds is common-severe
Oesophageal dysmotility-fibrosis
sclerodactyly-tight skin
Telegectasia

Limited cutaneous-CREST, and pulm HTN main

Diffuse-CREST, interstitial lung disease, kidney disease (as vessels thicken tons)–ischemia
Pulm HTN is rarer
and more-

Question 3

Aetiology and risk factors of Systemic sclerosis

Answer 3

Loss of B cell tolerance and ANA vs centromeres/SCL70
Inflammation all over-activate fibroblast, fibrosis causes skin tight
also microvascular disease from endothelial disease

Risk factors
FHX
Other ANA related AID

Question 4

Investigations of Systemic sclerosis

Answer 4

ANA-anti centromere (more in limited) and Anti SCL70 (more in Diffuse)

CXR for lungs
U&E for Kidneys, etc

Question 5

Management of Systemic sclerosis

Answer 5

can immunosupress-if skin (prednisolone, steroid sparing)

If more vascular symptoms-Nifedipine helps with the reynaduds (vasodilator)