Systemic Sclerosis/CREST

Question 1

Systemic Sclerosis

Answer 1

scleroderma (AI skin fibrosis) + vascular disease

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

Question 2

Types of Systemic Sclerosis

Answer 2

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

Question 3

Rx factors

Answer 3

FMHx and silica dust exposure

Question 4

Presentation

Answer 4

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

Question 5

Ix:

Answer 5

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

Question 6

Management:

Answer 6

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

Question 7

Acute renal crisis

Answer 7

New Onset HTN, raised Cr, intravascular hemolysis

• cilazapril
• hydration
• nifedipine