Systemic Sclerosis

Question 1

What is systemic sclerosis

-what are the consequences

Answer 1

Excessive rapid production and accumulation of collagen that involves internal organs

Proximal cutaneous involvement
Early renal, GI, ILD
Pulmonary HTN

Question 2

What are the skin symptoms

Answer 2

Hardening and scarring => tight red, scaly skin

Nailfold capillaries

Digital ulcers

Question 3

What are the lung symptoms

Answer 3

Pulmonary fibrosis (honeycombing)
Pulmonary HTN => dilated pulmonary vessels

Question 4

What are the Gi symptoms

Answer 4

Esophageal stricturing => relfux esophagitis

Decreased motility

Question 5

What are the renal symptoms

Answer 5

Poor prognostic factor, cause of death

• increased collagen in renal vessels => narrowing, leads to decreased blood flow
• hyperreninnemia (to increase blood flow)
• malignant HTN => renal damage
• kidney failure with accumulation of waste products in blood
• microangiopathic hemolytic anemia due to RBC trauma

Question 6

Describe limited cutaneous systemic sclerosis

Answer 6

CREST syndrome

• calcinosis
• Raynaud’s
• esophageal involvement
• sclerodactyly, distal involvement
• telangiectasia

Question 7

How would you treat systemic sclerosis

Answer 7

Early treatment => low does steroids (high dose may trigger renal crisis), immunosuppresion

Late treatment => symptomatic tretament, BP control