Chap 185 Scleroderma and Raynaud Phenomenon

Question 1

encompasses a range of conditions unified by the presence

of fibrosis of the skin

Answer 1

Juvenile scleroderma

Question 2

2 major categories of Juvenile scleroderma

Answer 2

juvenile localized scleroderma (JLS , also known as morphea ), which is
largely limited to the skin, and juvenile systemic sclerosis (JSSc ), with
multisystem organ involvement.

Question 3

predominant type seen

in pediatric populations (>95%),

Answer 3

jls`

Question 4

___ believed to be a key process in the pathogenesis of both
localized and systemic scleroderma, given the high percentage of affected
children with autoantibodies

Answer 4

Autoimmunity

Question 5

prevalence of JS

Answer 5

rare, with an estimated prevalence of 1 in 100,000

children.

Question 6

what is more common in children? LS or SSc

Answer 6

LS

Question 7

what is the most common subtype

Answer 7

linear scleroderma

Question 8

initial skin manifestations of localized disease

Answer 8

erythema or a bluish hue seen around an area of waxy

induration; subtle erythema may be the only presenting sign

Question 9

subtype of LS may have symptoms unique to central nervous system (CNS)
involvement, such as seizures, hemifacial atrophy, ipsilateral uveitis, and
learning/behavioral changes

Answer 9

en coup de sabre

Question 10

___ causes ulceration of the fingertips

with subsequent loss of tissue pulp and tapered fingers (sclerodactyly )

Answer 10

Severe Raynaud phenomenon

Question 11

Resorption of the distal tufts of the distal phalanges may occur

Answer 11

(acroosteolysis )

Question 12

most common visceral manifestation of SSc and

includes both arterial and interstitial involvement

Answer 12

Pulmonary disease

Question 13

___ is a poor prognostic sign,

developing because of lung disease or independently as part of the vasculopathy

Answer 13

Pulmonary arterial hypertension

Question 14

Mortality from JSSc is usually a result of ___

Answer 14

cardiopulmonary disease

Question 15

___is the most frequent initial symptom in pediatric
systemic sclerosis, present in 70% of affected children months to years before
other manifestations

Answer 15

Raynaud phenomenon (RP)

Question 16

RP refers to the classic triphasic sequence of ___

Answer 16

blanching,
cyanosis, and erythema of the digits induced by cold exposure and/or emotional
stress

Question 17

true or false: RP is typically independent of an underlying rheumatic disease (Raynaud
disease) but can result from rheumatic diseases such as scleroderma, systemic
lupus erythematosus (SLE), and mixed connective tissue disease

Answer 17

true

Question 18

color changes are brought about by:

Answer 18

(1) initial arterial vasoconstriction,
resulting in hypoperfusion and pallor (blanching), (2) venous stasis (cyanosis),
and (3) reflex vasodilation caused by the factors released from the ischemic
phase (erythema)

Question 19

Digital ulcers associated with RP are

indicative of ___

Answer 19

underlying rheumatic disease

Question 20

____ often begins in adolescence and is characterized by
symmetric occurrence, the absence of tissue necrosis and gangrene, and the lack
of manifestations of an underlying rheumatic disease

Answer 20

Raynaud disease

Question 21

____is a vasospastic
disorder resulting in cool, painless, bluish discoloration in the hands and
sometimes feet despite normal tissue perfusion. It may be exacerbated by
stimulant medications used to treat attention-deficit disorder

Answer 21

Acrocyanosis

Question 22

____is a
condition with episodic color changes and the development of nodules related to
severe cold exposure and spasm-induced vessel and tissue damage; it has been
associated with SLE

Answer 22

Chilblains

Question 23

____ comprises a group of
unrelated diseases characterized by patchy or diffuse cutaneous fibrosis without
the other manifestations of scleroderma. These include phenylketonuria,
syndromes of premature aging, and localized idiopathic fibrosis

Answer 23

Pseudoscleroderma

Question 24

____ is
a transient, self-limited disease of both children and adults that has sudden onset
after a febrile illness (especially streptococcal infections) and is characterized by
patchy sclerodermatous lesions on the neck and shoulders and extending to the
face, trunk, and arms

Answer 24

Scleredema

Question 25

average disease duration of JLS

Answer 25

3-5yrs

Question 26

treatment: For lesions involving deeper structures, ___ is

recommended.

Answer 26

systemic therapy (A combination of methotrexate and corticosteroids )