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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

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