CTD-Systemic sclerosis

Question 1

systemic sclerosis

Answer 1

Excessive collagen deposition causes skin and internal organ changes.

Question 2

Characteristics of systemic sclerosis (2)

Answer 2

vasomotor disturbances (Raynauds)

fibrosis and subsequent atrophy of the skin subcutaneous tissue.

Question 3

presentation (5)

Answer 3

CREST

Calcinosis

Raynuads

oEsophageal distility

Sclerodactyly

Tengalesia

Question 4

what are the features divided into? (2)

Answer 4

major and minor:

Major: includes centrally located skin sclerosis that affects the arms, face, and/or neck

Minor: includes sclerodactyly and atrophy of the fingertips and bilateral lung fibrosis.

Question 5

organ involvement

Answer 5

Pulmonary hypertension, pulmonary fibrosis and accelerated hypertension leading to renal crisis are important organ manifestations.

Gut involvement may lead to dysphagia, malabsorption and bacterial overgrowth of the small bowel.

Inflammatory arthritis and myositis may be seen.

Question 6

classification

Answer 6

divided into limited and diffuse forms.

Limited: skin involved tends to be confined to face, hands and forearms and feet. Organ involvement tends to occur later. Anti-centromere antibody association.

Diffuse: skin changes develop more rapidly and can involve the trunk. Early significant organ involvement. Anti-Scl-70 antibody association.

Question 7

investigations (5)

Answer 7

limited- anti-centromere antibody

diffuse- anti-Scl-70.

Organ screening

echo

monitoring of renal function.

Question 8

management (5)

Answer 8

no one overall treatment for SSc.

Raynauds/digital ulcers: calcium channel blockers, Iloprost, bosentan

Renal involvement: ACE inhibitors

GI involvement: proton pump inhibitors for reflux

Interstitial lung disease: immunosuppression, usually with cyclophosphamide.