Scleroderma

Question 1

What are some signs of scleroderma?

Answer 1

Sclerodactyly (tightening of skin on fingers)

Raynaud’s phenomenon

Loss of tissue pulp at the end of the fingers

Calcinosis

Telangiectasia

Fine inspiratory crackles

Loss of forehead creases and ability to smile

Question 2

What is limited scleroderma defined as?

Answer 2

Involving the skin up to the elbow without chest, abdominal or internal organ involvement

Also oesophageal involvement

Question 3

What are some non-dermatological manifestations of scleroderma?

Answer 3

Arthritis in a rheumatoid distribution, carpal tunnel symptoms

GIT - Dysphagia, heartburn, diarrhoea

Renal - HTN, chronic kidney disease

Resp - Interstitial lung disease, pleurisy, pulmonary HTN

CV - pericarditis, palpitations, dilated cardiomyopathy

Other - ED, hypothyroidism

Question 4

What are some differential diagnoses of scleroderma?

Answer 4

Graft vs host disease

Eosinophilic fasciitis

Nephrogenic systemic fibrosis

Diabetic cheiroarthropathy

Question 5

What does CREST stand for?

Answer 5

Calcinosis

Raynaud’s phenomenon

Esophagus involvement

Sclerodactyly

Telangiectasia

Question 6

What drug can be used to prevent collagen cross-linking in scleroderma?

Answer 6

d-penicillamine

Question 7

What is the pathophysiology of scleroderma?

Answer 7

1. Increased deposition of collagen and other macromolecules in the skin and viscera
2. Severe fibroproliferative lesions in the small arteries and arterioles
3. Alteration of humeral and cellular immunity

Question 8

When is cyclophosphamide given in scleroderma?

Answer 8

If there is lung involvement

Question 9

What are the principle of treatment for scleroderma?

Answer 9

Avoid vasospasm (smoking, beta-blockers, cold)

Treat malabsorption

Reduced collagen cross-linking with d-penicillamine

Cyclophosphamide if lung involvement

NSAIDs for arthritis

Aggressive anti-hypertensives to prevent renal failure