Systemic sclerosis

Question 1

Pathology?

Answer 1

Vasomotor disturbance, fibrosis and subsequent atrophy of skin and subcutaneous tissue
Excessive deposition of collagen causes skin and internal organ changes

Question 2

Two classifications?

Answer 2

Limited and diffuse

Question 3

Features of limited systemic sclerosis?

Answer 3

Skin involved tends to be hands, face, forearms and feet
Organs are involved later on
CREST syndrome

Question 4

CREST syndrome

Answer 4

Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Question 5

Features of diffuse systemic sclerosis?

Answer 5

Skin changes develop rapidly and involve the trunk too

Organs are involved earlier on in the disease

Question 6

Other features of systemic sclerosis

Answer 6

Beaked nose

Tightening of skin around the mouth

Question 7

Organ involvement of systemic sclerosis

Answer 7

Pulmonary hypertension
Pulmonary fibrosis
Dysphagia
Malabsorption
Bacterial overgrowth

Question 8

Which antibody is associated with limited systemic sclerosis?

Answer 8

Anti-centromere antibody

Question 9

Which antibody is associated with diffuse systemic sclerosis?

Answer 9

Anti-Scl-70 antibody

Question 10

Raynauds management

Answer 10

Ca2+ blockers
Iloprost
Bosentan

Question 11

Renal involvement management

Answer 11

ACEI

Question 12

GI involvement management

Answer 12

PPI for reflux

Question 13

Interstitial lung disease management

Answer 13

Immunosuppression

- cyclophosphamide