Systemic Sclerosis

Question 1

What is systemic sclerosis (SSc)

Answer 1

A systemic connective tissue disease characterised by vasomotor disturbances (Raynauds), fibrosis and subsequent atrophy of the skin and subcutaneous tissue
Excessive collagen deposition causes skin and internal organ changes

Question 2

How common is systemic sclerosis?

Answer 2

2.3-10 per 1 million people

Question 3

Cutaneous presentation of SSc

Answer 3

1.Oedematous
2.Indurative
3.Atrophic
Skin becomes thickened and tight
Raynauds phenomenon is a common early finding
Beaking
Tightening of skin around mouth
Telangiectasia
Calcinosis

Question 4

Major cutaneous features of SSc

Answer 4

Centrally located skin sclerosis that affects the arms, face and or neck

Question 5

Minor cutaneous features of SSc

Answer 5

Sclerodactyly and atrophy of the fingertips and bilateral lung fibrosis

Question 6

Organ involvement in SSc

Answer 6

Pulmonary hypertension
Pulmonary fibrosis
Accelerated hypertension
Renal crisis
Dysphagia
Malabsorption
Bacterial overgrowth of the small bowel
Inflammatory arthritis
Myositis

Question 7

Define limited systemic sclerosis

Answer 7

Skin involved tens to be confined to the face, hands, forearms and feet. Organ involvement tends to occur later. Anti-centromere antibody association.

Question 8

Define diffuse systemic sclerosis

Answer 8

Skin changes develop more rapidly and can involve the trunk (crosses the elbows). Early significant organ involvement. Anti-Scl-70 antibody association.

Question 9

Systemic sclerosis investigations

Answer 9

Anti-centromere and anti-Scl-70 autoantibody association

Organ screening: pulmonary function testing, echo and monitoring of renal function.

Question 10

Management of systemic sclerosis

Answer 10

Raynauds/digital ulcers = calcium channel blockers, iloprost, bosentan
Renal involvement = ACE inhibitors
GI involvement = proton pump inhibitors for reflux
ILD = immunosuppression (cyclophosphamide)