Ch 382: Systemic Sclerosis (Scleroderma) and Related Disorders

Question 1

Systemic sclerosis (SSc) is an uncommon __________ tissue disorder, characterized by __________ involvement, heterogeneous clinical manifestations, a _______ and often __________ course, and significant disability and mortality.

Answer 1

connective
multisystem
chronic
progressive

(p. 2154)

Question 2

What causes systemic sclerosis?

Answer 2

Cause is unclear. Multiple genes contribute to disease susceptibility; however, environmental exposures are likely to play a major role is SSc.

Question 3

What are the 3 cardinal pathophysiologic processes which account for the protean clinical manifestations of SSc:

Answer 3

(1) diffuse microangiopathy
(2) inflammation and autoimmunity
(3) visceral and vascular fibrosis in multiple organs

(p. 2156)

Question 4

Patients with SSc can be broadly grouped into…

Answer 4

…diffuse cutaneous and limited cutaneous.

Question 5

In some patients, Raynaud’s phenomenon and other characteristic features of SSc occur in the absence of skin thickening. This syndrome has been termed ___ ____ __________.

Answer 5

SSc sine scleroderma

p. 2155