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Flashcards in Systemic Sclerosis/CREST Deck (7)
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1

Systemic Sclerosis

scleroderma (AI skin fibrosis) + vascular disease

= autoimmune disease where overproduction of collagen causes fibrosis of skin, organs as well as vasculopathy

2

Types of Systemic Sclerosis

1. Limited cutaneous systemic sclerosis
- fibrosis 'limited' to face, extremities
2. Diffuse cutaneous systemic sclerosis
- Can affect the whole body
- poor prognosis
- can affect whole organs (lungs, heart, GI, kidneys)

CREST
C - calcinosis
R - Raynauds
E - esophageal dysmotility
S - sclerodactyly
T - telangiectasia

3

Rx factors

FMHx and silica dust exposure

4

Presentation

Skin Involvement = bilateral thickening of hand extending proximal to MCP, thickening of fingers proximally with sclerodactyly distally. prayer sign, pigmentation change

Fingertip lesions = tip ulcers, pitting scars

Telangiectasia = blanchable, demarcated

Nailfold capillaries

Pulmonary artery HTN = intersitial lung disease

Raynauds
GORD
Flexion Contracture
Calcinosis of extensor surfaces

5

Ix:

FBC - anaemia, reactive thrombocytosis, haemolysis
Renal Function - Cr (can be decreased in diffuse)
ESR normal/raised
ANA/ENA
- ANA+
- RF + (30%)
- Anti-centromere Ab (75%) - LcSSc
- Anti-topoisomerase (scl70) + (40%) - DcSSC
- Anti-RNA poly+ (20%) -DcSSc
- Anti Ro +/-

Echo
- PA pressure and RH enlargement

PFTs
- low DLCO suggests PAH with a decreased FVC preceeding ILD

6

Management:

Education:
No prednisone

Lifestyle:
- smoking cessation
- regular exercise/PT
- splints for ROM and carpal tunnel

Analgesia: for calcinosis, raynauds, arthritis
- paracetamol + NSAIDS

Immunosuppression:
- Methotrexate/hydroxychloroquine (arthritis/myositis)
- Mycophenolate (lung involvement)
- Cyclophosphamide

Raynauds phenomena
- analgesia
- Nifedipine, fluoxitine, topical nitrate, sildenafil

Dysphagia
- supportive, dilitation
- PPI if GORD

AVOID PREDNISONE > 5mg daily as it can precipitate renal crisis

Monitoring:
FBC
Renal Function
Echo
PFTs

7

Acute renal crisis

New Onset HTN, raised Cr, intravascular hemolysis
- cilazapril
- hydration
- nifedipine