Systemic sclerosis

Question 1

What is systemic sclerosis?

Answer 1

Connective tissue disorder causing thickening of skin/joints and internal organs

Question 2

What are the types of systemic sclerosis? Which is more common?

Answer 2

Limited cutaneous and diffuse cutaneous

Limited cutaneous is more common

Question 3

What is the skin involvement in the two different types of systemic sclerosis?

Answer 3

Limited cutaneous: Distal aspects limbs, neck, face

Diffuse cutaneous: Trunk and proximal limbs

Question 4

What are the typical symptoms of limited cutaneous systemic sclerosis?

Answer 4

Calcinosis, raynaud’s, esophageal dysfunction, sclerodactyly, telangiectasia
Extra-cutaenous organ involvement can occur

Question 5

What are the ANAs involved systemic sclerosis?

Answer 5

ANA positive in 90%
Limited cutaneous: Anti-centromere
Diffuse cutaneous: Anti-Scl70 and Anti-RNA polymerase III

Question 6

What are the common extra-cutaneous manifestations of diffuse cutaneous systemic sclerosis?

Answer 6

Heart: Myocarditis, pericarditis, fibrosis
Lungs: Pulmonary HTN, fibrosis, interstitial lung disease
GI: Dysphagia, reflux, constipation, bloating, gas, cramping
Joints and muscles: Arthralgia, myalgia
Kidneys: Reduce renal function
Scleroderma renal crisis: Oliguric renal failure with malignant hypertension

Question 7

What is the management of systemic sclerosis?

Answer 7

Depends on the symptoms
General: Prevent dry skin with moisturiser and soap
Pharma: Immunosuppressants e.g. methotrexate
Long term screening for organ involvement and organ specific therapies