Scleroderma

Question 1

P

Answer 1

Autoimmune CT disorder
Localised - children, confined to skin and SC tissue
OR systemic sclerosis - CREST syndrome

OVerproduction of collagen - hardening of skin tissue
Small cessels involved in SSc (CREST)

Question 2

Antibodies?

Answer 2

ANA positive

Anti-centromere in CREST syndrome

Question 3

A

Answer 3

ANA positive
FHx
Female
Cytomegalovirus triggger

Question 4

CF CREST

Answer 4

Calcinosis underneath fingertips
Raynaud’s phenomenon - first sign
Esophageal dysmotility - dysphagia or GORD
Sclerodactyly - stiff fingers
Telagiectasia - dilated small blood vessels

Question 5

CF

Answer 5

Skin thickness
Swellig puffiness of skin

Lung - pulmonary HTN
Heart - pericardial effusions, right heart failure
Kidney - renal impairment

Question 6

What is Raynaud’s phenomenon?

Answer 6

Transient vasospasm of peripheral blood vessels leading to hypoxia
Ischaemic gangrene and digital ulcers in extremes
Stress and cold are triggers

Primary
Secondary due to SSc

Digits change colour from white to blue to red

Question 7

Ix

Answer 7

ANA
Anti-Scl 70 (topoisomerase-1) - lung fibrosis
Anti-centromere antibody - CREST
Anti-RNA polymerase - diffuse sclerosis with kidney involvement

Pulmonary function test
CXR
HRCT
Echo
Barium swallow

Question 8

Mx

Answer 8

Skin:
Low dose prednisolone or methotrexate

Vascular:
Vasodilator - CCB,

GI
Avoid eating before bed
Avoid caffeine
PPI
Antibiotics

Renal
ACE inihibitor

Cardiac
Oral pregniolone
Monitor BP

REsp
Cyclophosphamide if ILD