Systemic Sclerosis Flashcards
What is systemic sclerosis?
- Multisystem autoimmune disease
- Previously known as scleroderma
- Increased fibroblast activity = abnormal growth of connective tissue which leads to vascualr damage and fibrosis
2 subtypes of systemic sclerosis
- Limited SSc
- Diffuse SSc
Limited scleroderma aka CREST syndrome signs/symptoms
- Calcinosis - deposits in tips of fingers –> then cause ulcers/infection
- Raynaud’s
- oEsophageal dysfunction
- Sclerodactyl - tight shiny skin
- Telangiectasia - dilated capillaries (spider naevi)
Also cannot forget pulmonary hypertension
CREST syndrome presentation
- Many years of Raynaud’s symptoms before any scleroderma
- Development of pulmonary hypertension after 10 yrs of symptoms
Diffuse scleroderma - more/less common?
Less common
Higher risk mortality
Diffuse scleroderma presentation
Sudden onset of skin involvement
Proximal to elbows and knees
Investigations for systemic sclerosis
- Inflammatory markers normal
- X-ray hands - calcinosis
- CXR
- HRCT
- PFT - pulmonary disease
- ECG and ECHO - pulmonary hypertension? heart failure, myocarditis, arrhythmias
Antibodies in systemic sclerosis
- Positive ANA 90%
- Anti centromere antibody - limited SSc
- Scl-70 (topisomerase) and anti RNA polymerase III antibodies associated with diffuse
Main thing to look at in PFTs
- Restrictive lung pattern - fibrosis
- Transfer factor - DLCO decreases
Treatment for systemic sclerosis
- No cure
- Psychological support
- Calcium antagonist/sildenafil/iloprost infusion for Raynauds
- Methotrexate and mycophenolate mofetil may reduce thickening of skin
- Prednisolone for flares
- ACEi prevent HTN crisis and reduce mortality from renal failure
- PPI for GI symptoms
Scleroderma M:F
5 times more common in women
What is localised scleroderma called?
Morphoea
Consequences of vascular involvement of systemic sclerosis
- Raynauds
- Ischaemic digital ulcers
- Hypertensive crisis
- Pulmonary arterial hypertension
What makes systemic sclerosis diagnosis unlikely?
Absence of Raynauds
What is scleroderma renal crisis?
- Accelerated HTN
- Lead to renal failure if not treated quick
- Seen early in diffuse scleroderma
What are the changes seen in Raynaud’s and why?
- White - ischaemia
- Blue - venous stasis
- Red - reactive hyperaemia
Pain occurs when blood flow returns, may also get parasthesia
Effects on heart of systemic sclerosis
- Restrictive pericarditis - fibrous pericardium stiff
- Heart block - from fibrotic tissue
Effects on GI from systemic sclerosis
- Malabsorption
- Reflux
- Oesophageal dysfunction
- Gastroparesis
- Pseudobstruction
Sexual effects of SS
- Erectile dysfunction
Why do we do echo in SS?
- Asses for cor pulmonale
- Pulmonary HTN –> cor pulmonale
Treatment for renal crisis
ACEi - Captopril - onset 60-90mins
Transplant
What is renal crisis?
AKI due to impaired blood flow to kidney from fibrosis
RF for renal crisis
- Diffuse disease
- Steroids
- Anti RNA polymerase
- Anticentromere
Presentation of renal crisis
- Malignant HTN
- Encephalopathy
- AKI
- Urinalysis normal
- Low platelets - MAHA (becomes hard to distinguish between HUS and TTP)
Treatment for calcinosis of fingers if ulcerated/infected
Minocycline
What can be used for telangiectasia?
Laser therapy
Treatment for itchy skin
- Emolients
- Capsaicin
- Menthol
Treatment for pulmonary HTN
- Bosentan
- Tadalafil
