Systemic Sclerosis (scleroderma) (Diagnosis (HRCT to look at lung…
Systemic Sclerosis (scleroderma)
Definition: this is a multisystem disease that involves the skin and Rynauds phenomenon
Exposure to vinyl chloride, silica dust and rapeseed oil
There is continued vascular damage and permeability. This causes T, B cells and monocytes and neutrophils to adhere and leak in.
These cells then stimulate the production of cytokines and growth factors that mediates vascular and connective tissue cells like fibroblasts.
Endothelial cell damage releases cytokines and releases endothelin-1 and vascoconstriction
Fibroblasts then activated by mediators cause collagen type 1 and 2
Widespread vascular damage that involves the small arteries, capillaries and aterioles
This causes there to be irreversible thickening of the connective tisse and vessel walls.
Damage to the small blood vessels causes lesions and subsequent ischaemia
HRCT to look at lung fibrosis
Urea and creatinine will rise in AKI
Blood: you will get normochromic and normocytic anaemia. Can also get haemolytic anaemia in patients with renal disease
Diffuse cutaneous scleroderma: Anti- RNA polymerase and Anti-topoisomerase-1-antibodies
Barium swallow to look at oesphageal motility
Limited cutaneous scleroderma: Anti-centromere antibodies (ACA)
Hand X-Ray to look at the calcium deposits around the fingers
Highest mortality rheumatic disease
Happens more common in females
Rare in children
Peak incidence is between 30-50 years old
Limited cutaneous scleroderma. Happens over many years
Raynauds phenomenon can start many years (up to 15) before any skin changes
Calcinosis deposits in the subcutaneous tissue
Sclerodactyly- local thickening/ tightness of the skin
Telangiectasia- spider veins
Diffuse cutaneous scleroderma- happens more rapidly and is more widespread
Lung disease: fibrosis and pulmonary vascular disease--> pulmonary fibrosisi
Renal involvement: AKD or CKD. Can lead to hypertension
GI involvement means that there is a loss of the strength of the osephagous: causing heart burn and dysphagia. There is a small intestine problem causing malabsorption and in the colon can cause pseudo obstruction
Raynauds: keep warm and also use a vasodilator like nifedipine.
Renal crisis: inhibition by using an ACE inhibitor
Use ECHO and pulmonary function tests to detect early hypertension.
PULMONARY FIBROSIS: cyclophosphamide or prednisolone
Host vs Graft disease