Systemic Sclerosis

Question 1

A multisystem disease characterized by ? sclerosis and ? disease.

Pathophysiology;
o ? fibrosis leads to ? damage in a range of tissues.
o The ? is most commonly affected (dermal ? leading to
hardened ?), but also major organs such as the ?, heart, ? and kidneys damaged.

Answer 1

skin
reynauds
perivascular
ischaemic
skin
thickening
skin
lungs
oesophagus

Question 2

Limited cutaneous scleroderma (70%);

Patients present with a long history of ? phenomenon, then skin ? at the ?.
o The face can also be involved, leading to ?.

Answer 2

reynaud
tightening
extremities
microstomia

Question 3

Limited cutaneous scleroderma (70%);

Early symptoms;
0 constitutional symptoms such as ? very common.
o ????.
0 ? on digital tips.

Later symptoms;
0 Oesophageal ?.
0 ? bowel ?.
0 Pulmonary ?/ pulmonary ?.

Answer 3

fatigue
GORD
ulcers
strictures
small, malabsorption
fibrosis
htn

Question 4

Limited cutaneous scleroderma (70%);

Previously known as '?????' syndrome;
o ?, ?, ? dysmotility, ? &
?.
o This term was abandoned as it under-emphasises life threatening
complications such as pulmonary ?

Answer 4

CREST
calcinosis
raynaud
oesophageal
sclerodactyly
telangiectasia
htn

Question 5

Limited cutaneous scleroderma (70%);

Management
Digital ? or ? (Reynaud’s).
Removal of ?.
- Treatment of oesophageal problems.

Answer 5

sympathectomy
vasodilators
calcinoses

Question 6

Diffuse cutaneous scleroderma (30%);

Patients have a ? history of ? disease, and skin sclerosis is ? progressive, peaking at ? years.
Systemic symptoms are greater, including ?, ? loss and ?.

Answer 6

short
raynauds
rapidly
two
lethargy
wt
anorexia

Question 7

Diffuse cutaneous scleroderma (30%);

Complications will occur within the first ? years;
o ? fibrosis.
o Pulmonary ?.
o ? fibrosis.

Answer 7

three
myocardial
fibrosis
renal

Question 8

Diffuse cutaneous scleroderma (30%);

Management;
?;
o Aiming to prevent complications, so can be gradually withdrawn over
years as the disease stabilizes.
Again ? and ? are helpful for symptoms.

Answer 8

immunosuppression
vasodilators
sympathectomy

Question 9

Investigation;

Limited cutaneous scleroderma;
o Anti-? antibodies positive (60%).

Diffuse cutaneous scleroderma;
o Anti-?-? antibodies (40%), and ??? ? antibodies.
o These can both also be present in limited cutaneous scleroderma,
however less commonly.

Answer 9

centromere
scl-70
RNA polymerase

Question 10

Prognosis;

Systemic sclerosis has the ? mortality of any of the autoimmune rheumatic diseases.

Limited cutaneous scleroderma has a ? survival (?% at 10 years) due to
? severe ? ? damage.

Answer 10

highest
higher
70
less
internal organ