Exam Cram

• Systemic sclerosis = CREST + systemic features: lung fibrosis, haematoproteinuria

• Limited cutaneous sclerosis = CREST = calcinosis, Raynaud's, esophagitis, sclerodactyly, telangiectasia

• Systemic sclerosis:  anti-topoisomerases, 

• Ant-SCL 70: limited cutaneous sclerosis

Systemic sclerosis is also caused scleroderma. 

Systemic Sclerosis

Systemic sclerosis can affect only the skin and oesphagus (CREST syndrome) or the renal and lungs. 

Complications

• Lung: pulmonary fibrosis, Pulmonary Hypertension, Pulmonary Haemorrhage

• GI: GORD, peptic strictures, Small intestinal bacterial overgrowth

• Renal: renal crisis, angiopathic Haemolytic Anaemia (HA)  

Long Term

Annual: 

• Pulmonary Function Tests, 

• ECHO & ECG

• Skin tightness

CREST Syndrome

Background

CREST stands for

1. Calcinosis

2. Raynaud's

3. Esophageal dysfunction

4. Sclerodactyly

5. Telangiectasia

Crest syndrome describes Scleroderma affecting largely the skin

Investigations

• Anti-centromere antibodies

Scleroderma Renal Crisis

Background

• Steroids are thought to trigger a renal crisis. 

• Renal disease a key source of mortality in scleroderma.

• 40% may require dialysis, mortality 30% 5 years

Assessment

History

• Anorexia, nausea, headaches

• 7 days

• Recent steroid use

Examination

• Bilateral crackles at base

• Scleroderma signs: sclerodactyl, peripheral calcinosis

Investigations

• Raised creatinine

• Haematoproteinuria

• Anti RNA polymerase III

Management

1. ACE inhibitors

2. Dialysis as required

Systemic Sclerosis and ILD

Systemic sclerosis is a cause of interstitial lung disease. 

Management: 

1. MMF

Written in 2024