Systemic Sclerosis Flashcards
What is sytemic sclerosis ?
multisystemic AI disease characterised by functional and structural abnormalities of non-inflammatory blood vessels, fibrosis of skin and internal organs
What is normal epid for systemic sclerosis?
females
What are face feet symptoms of limited systemic sclerosis?
- Calcinosis cutis
- Raynaud’s
- oesophageal dysmotility
- Sclerodactyly
- Telangiectasia.
What is often present in limited?
- Pulmonary hypertension is often present so monitor carefully
- More benign that diffuse
What would diffuse widespread organ involvement for systemic sclerosis mean?
- rapid progression with early visceral involvement
- prognosis is often poor
- monitor BP meticulously
What is the presentation of systemic sclerosis?
usually manifests as puffy taut skin with no wrinkles and finger tip pitting
What are the renal manifestations of systemic sclerosis?
scleroderma hypertensive crisis
What are the pulmonary manifestations of systemic sclerosis?
- interstitial fibrosis
2. PH
What are GI manifestations of systemic sclerosis?
- dysmotility
- reflux
- watermelon stomach
What are the cardio manifestations of systemic sclerosis?
pericarditis or effusion
What investigations are used for systemic sclerosis?
1 Autoantibodies
2. Most other investigations are non-specific and look at complications of the disease
What autoantibodies are found in systemic sclerosis?
- Anti-centromere: Usually present in limited type
2. Anti-Scl 70 and Anti-RNA polymerase III: Usually common in diffuse type
Is there a cure for systemic sclerosis?
no
What is the maintenance therapy for systmeic sclerosis?
immunosuppression and treatment for individual symptoms
How do you treat renal crisis for systemic sclerosis?
ACE-I or ARB with aggressive hydration (IMPORTANT)
