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Systemic Sclerosis Title Ñ Systemic Sclerosis Definition Ñ Multisystem disorder of unknown aetiology Characterised by: Ñ Excessive fibrosis of tissues Pathogenesis Causes Ñ Not known Scleroderma like disorders may occur on exposure to: Environmental agents Ñ Vinyl chloride, silica, adulterated rapeseed oil, trichloroethylene Drugs Ñ Bleomycin Pathological Process Ñ Begins with vascular damage involving the small arteries, arterioles, capillaries Ñ Endothelial damage Ñ Release of cytokines Ñ Increased vascular permeability Ñ Migration of cells into the extracellular matrix Ñ Activation of fibroblasts Ñ Fibroblasts synthesise collagen with resultant fibrosis of the skin and affected organs Ñ Damage to the blood vessels leads to obliteration of these vessels and chronic ischaemia Clinical Features History Sex Ñ Females > Males Age Ñ Age 30-50 years Types of Scleroderma Ñ Localised: Morphea Linear scleroderma Ñ Limited Cutaneous Scleroderma (LcSSc) Ñ Diffuse Cutaneous Scleroderma (DcSSc) Ñ Scleroderma sine scleroderma Ñ Environmentally induced scleroderma Ñ Scleroderma Ñ Eosinophil fasciitis Skin changes Ñ Oedematous phase: Painless oedema Ñ Thickening and tightening of the skin Ñ Atrophy and contractures Limited Cutaneous Scleroderma (formerly CREST) IS Ñ Acral distribution: Face, hands, forearms, feet Ñ Tight skin Ñ Flexion deformities Ñ Ulcers Ñ Telangiectasiae Ñ Dilated nail fold capillaries CVS Ñ Raynaud’s phenomenon: Many years before skin features RS Ñ Pulmonary hypertension Ñ Interstitial pulmonary disease GIT Ñ Small mouth Ñ Oesophageal involvement Diffuse Cutaneous Scleroderma E&M Ñ Lethargy Ñ Anorexia Ñ Weight loss IS Ñ Oedema of the skin followed by skin sclerosis CVS Ñ Myocardial fibrosis with conduction defects and arrhythmias Ñ Pericarditis RS Ñ Pulmonary fibrosis Ñ Pulmonary hypertension GIT Ñ Heartburn, reflux, dysphagia Ñ Faecal incontinence Ñ Dilatation and atony of the small bowel with secondary small bowel bacterial overgrowth KUS Ñ Renal involvement: May be acute or chronic HS Ñ Normocytic normochromic anaemia LMS Ñ Diffuse swelling and stiffness of the fingers followed by sclerosis Scleroderma sine scleroderma Ñ Raynaud’s Ñ Involvement of internal organs Ñ Without skin changes Localised scleroderma Ñ Morphea: Discrete cutaneous plaques or induration Ñ Linear scleroderma: Band of fibrosis involving the skin, soft tissue, muscle, bone Environmentally induced scleroderma Ñ Changes induced by: Bleomycin Polyvinyl chloride (PVC) Scleroderma Ñ Painless, self-limiting oedema of the face, neck and upper trunk: Children Eosinophilic fasciitis Ñ Swelling and thickening of the forearms and legs following trauma or exercise Ñ Eosinophilia Ñ ESR: Raised Investigations Fluids Blood Haematology Ñ Normocytic normochromic anaemia Ñ ESR: Raised Immunology Ñ Anticentromere antibodies (ACA): LcSSc Ñ Scl 70: DcSSc Ñ ANA: May be positive Ñ Rheumatoid factor: May be positive Ñ Antimitochondrial antibody: May be positive Ñ Antibodies to RNA polymerases I and III Biochemistry Ñ Urea and creatinine: Increased in renal disease Urine Ñ Proteinuria Imaging Hand X-ray Ñ Deposits of calcium around fingers Ñ Erosion and absorption of the tufts of the distal phalanges Barium swallow Ñ Impaired oesophageal motility CT Scan Ñ High resolution CT: Demonstrates pulmonary fibrosis Management Support Drugs Ñ Raynaud’s: · Calcium antagonists · ACE inhibitors · Prostacyclin analogues (intravenous) Iloprost · Calcitonin gene related peptide (intravenous) Ñ Oesophageal symptoms: Proton pump inhibitors Prokinetics Ñ Malabsorption: Cyclical antibiotics for bacterial overgrowth Ñ Renal involvement: · Antihypertensives: ACE inhibitors, angiotensin II receptor blockers · Avoid high dose steroids: > 20mg Ñ Pulmonary hypertension: · Oxygen · Warfarin · Endothelin receptor antagonists: Bosentan · Prostacyclin analogues: Epoprostenol, treprostinil, beraprost, iloprost · Phosphodiesterase type 5 (PDE-5) inhibitors: Sildenafil Ñ Pulmonary fibrosis: Immunosuppression Surgery Ñ Digital sympathetectomy: Critical ischaemia Lifestyle Adjustments Diet Ñ Nutritional supplementation Exercise Ñ Prevent contractures Abuse Ñ Smoking: Cessation Environment Ñ Avoid exposure to cold Equipment Ñ Dialysis for renal failure Prognosis Ñ LcSSc: 70% 10-year survival Pulmonary hypertension significant later cause of death Ñ DcSSc: 55% 10-year survival Pulmonary, cardiac, renal involvement
Systemic Sclerosis
Title
Ñ Systemic Sclerosis
Definition
Ñ Multisystem disorder of unknown aetiology
Characterised by:
Ñ Excessive fibrosis of tissues
Pathogenesis
Causes
Ñ Not known
Scleroderma like disorders may occur on exposure to:
Environmental agents
Ñ Vinyl chloride, silica, adulterated rapeseed oil, trichloroethylene
Drugs
Ñ Bleomycin
Pathological Process
Ñ Begins with vascular damage involving the small arteries, arterioles, capillaries
Ñ Endothelial damage
Ñ Release of cytokines
Ñ Increased vascular permeability
Ñ Migration of cells into the extracellular matrix
Ñ Activation of fibroblasts
Ñ Fibroblasts synthesise collagen with resultant fibrosis of the skin and affected organs
Ñ Damage to the blood vessels leads to obliteration of these vessels and chronic ischaemia
Clinical Features
Sex
Ñ Females > Males
Age
Ñ Age 30-50 years
Types of Scleroderma
Ñ Localised:
Morphea
Linear scleroderma
Ñ Limited Cutaneous Scleroderma (LcSSc)
Ñ Diffuse Cutaneous Scleroderma (DcSSc)
Ñ Scleroderma sine scleroderma
Ñ Environmentally induced scleroderma
Ñ Scleroderma
Ñ Eosinophil fasciitis
Skin changes
Ñ Oedematous phase:
Painless oedema
Ñ Thickening and tightening of the skin
Ñ Atrophy and contractures
Limited Cutaneous Scleroderma (formerly CREST)
IS
Ñ Acral distribution:
Face, hands, forearms, feet
Ñ Tight skin
Ñ Flexion deformities
Ñ Ulcers
Ñ Telangiectasiae
Ñ Dilated nail fold capillaries
CVS
Ñ Raynaud’s phenomenon:
Many years before skin features
RS
Ñ Pulmonary hypertension
Ñ Interstitial pulmonary disease
GIT
Ñ Small mouth
Ñ Oesophageal involvement
Diffuse Cutaneous Scleroderma
E&M
Ñ Lethargy
Ñ Anorexia
Ñ Weight loss
Ñ Oedema of the skin followed by skin sclerosis
Ñ Myocardial fibrosis with conduction defects and arrhythmias
Ñ Pericarditis
Ñ Pulmonary fibrosis
Ñ Heartburn, reflux, dysphagia
Ñ Faecal incontinence
Ñ Dilatation and atony of the small bowel with secondary small bowel bacterial overgrowth
KUS
Ñ Renal involvement:
May be acute or chronic
HS
Ñ Normocytic normochromic anaemia
LMS
Ñ Diffuse swelling and stiffness of the fingers followed by sclerosis
Scleroderma sine scleroderma
Ñ Raynaud’s
Ñ Involvement of internal organs
Ñ Without skin changes
Localised scleroderma
Ñ Morphea:
Discrete cutaneous plaques or induration
Ñ Linear scleroderma:
Band of fibrosis involving the skin, soft tissue, muscle, bone
Environmentally induced scleroderma
Ñ Changes induced by:
Bleomycin
Polyvinyl chloride (PVC)
Scleroderma
Ñ Painless, self-limiting oedema of the face, neck and upper trunk:
Children
Eosinophilic fasciitis
Ñ Swelling and thickening of the forearms and legs following trauma or exercise
Ñ Eosinophilia
Ñ ESR:
Raised
Investigations
Blood
Immunology
Ñ Anticentromere antibodies (ACA):
LcSSc
Ñ Scl 70:
DcSSc
Ñ ANA:
May be positive
Ñ Rheumatoid factor:
Ñ Antimitochondrial antibody:
Ñ Antibodies to RNA polymerases I and III
Biochemistry
Ñ Urea and creatinine:
Increased in renal disease
Urine
Ñ Proteinuria
Imaging
Hand X-ray
Ñ Deposits of calcium around fingers
Ñ Erosion and absorption of the tufts of the distal phalanges
Barium swallow
Ñ Impaired oesophageal motility
CT Scan
Ñ High resolution CT:
Demonstrates pulmonary fibrosis
Management
Ñ Raynaud’s:
· Calcium antagonists
· ACE inhibitors
· Prostacyclin analogues (intravenous)
Iloprost
· Calcitonin gene related peptide (intravenous)
Ñ Oesophageal symptoms:
Proton pump inhibitors
Prokinetics
Ñ Malabsorption:
Cyclical antibiotics for bacterial overgrowth
· Antihypertensives:
ACE inhibitors, angiotensin II receptor blockers
· Avoid high dose steroids:
> 20mg
Ñ Pulmonary hypertension:
· Oxygen
· Warfarin
· Endothelin receptor antagonists:
Bosentan
· Prostacyclin analogues:
Epoprostenol, treprostinil, beraprost, iloprost
· Phosphodiesterase type 5 (PDE-5) inhibitors:
Sildenafil
Ñ Pulmonary fibrosis:
Immunosuppression
Surgery
Ñ Digital sympathetectomy:
Critical ischaemia
Lifestyle Adjustments
Diet
Ñ Nutritional supplementation
Exercise
Ñ Prevent contractures
Abuse
Ñ Smoking:
Cessation
Environment
Ñ Avoid exposure to cold
Equipment
Ñ Dialysis for renal failure
Prognosis
Ñ LcSSc:
70% 10-year survival
Pulmonary hypertension significant later cause of death
Ñ DcSSc:
55% 10-year survival
Pulmonary, cardiac, renal involvement
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