systemic sclerosis

Question 1

what is systemic sclerosis?

Answer 1

Multisystem autoimmune disease.

Previously known as scleroderma.

Increased fibroblast activity resulting in abnormal growth of connective tissue which leads to vascular damage and fibrosis.

2 main sub types:
limited Systemic sclerosis
diffuse Systemic sclerosis

Question 2

what are the common signs and symptoms of limited scleroderma?

Answer 2

also known as CREST syndrome

• Calcinosis cutis (accumulation of calcium crystal salt in skin)
• Raynauds phenomenon
• Eosophageal dysmotility
• sclerodactyly (hardening of skin that causes fingers to curl inward and look like a claw)
• Telangiectasia (spider navei)

involves face hands and feet

Question 3

what is diffuse scleroderma?

Answer 3

Less common.

Higher risk of mortality.

Characterised by sudden onset of skin involvement, and is proximal to the elbows and knees.

can be whole body

Question 4

what will investigations into systemic sclerosis show?

Answer 4

• inflammatory markers normal
• X ray hand will show calcinosis
• CXR, HRCT, PFT can show pulmonary disease
• ECG and ECHO can show PA hypertension, HF, myocarditis and arrythmias

Question 5

what do antibody tests in systemic sclerosis show?

Answer 5

• ANA + in most patients
• anti centromere antibody in limited SSc
• Scl-70 (topisomerase) and anti RNA polymerase III antibodies strongly associated with diffuse SSC

Question 6

how systemic sclerosis treated?

Answer 6

• no cure
• psychological support may be needed
• Ca antagonist/sildenafil/iloprost infusion for Raynauds Symptoms
• methotrexate and mycophenolae mofetl may reduce skin thickening
• ACE inhibitors for hypertension and renal issues
• short course prednisolone for flares
• PPI for GI symptoms

Question 7

what does vascular involvement in systemic sclerosis involve?

Answer 7

Raynauds phenomenon

ischaemic digital ulcers

hypertensive crisis

pulmonary arterial hypertension