Systemic sclerosis

A multisystem disease with widespread obliterative damage to small blood vessels associated with fibosis of the skin and internal organs

Clinical features

Raynaud's phenomenon

• 97% of cases
• Arterial spasm of hands and feet
• Three phases
• Pallor
• Cyanosis
• Erythema
• Numbness and pain

Skin

• Hands, face, feet, foearms
• Tight, waxy and tethered
• 'Beaking of nose'
• Microstomia
• Digital ulcers
• Telangiectasia
• Nasal fold capillary loops

GI tract

• Oesophagus
• Reflux
• Poor motility
• Dilatation
• Small bowel
• Bacterial overgrowth
• Malabsorption
• Renal
• Renal failure
• Malignant hypertension
• Cardiorespiratory system
• Pulmonary fibrosis (common cause of death)
• Primary or secondary pulmonary hypertension
• Arrhythmias
• Conduction defects
• Pericarditis

CREST syndrome

• Calcinosis (calcium deposis in skin and elsewhere)
• Raynaud's phenomenon
• oEsophageal dysmotility
• Sclerodactyly
• Telangiectasia

Investigations

• Normocytic, normochromic anaemia
• Urea and electrolytes and urinalysis including creatinine clearance
• Autoantibodies
• Speckled/nucleolar/anticentromere - 70-80%
• Rheumatoid factor - 30%
• Chest X-ray - reticulonodular shadowing
• Other tests according to organ involved

Management

• Education, counselling and family support
• Hand-warmers and vasodilators for Raynaud's
• Proton pump inhibitos and motility agents
• Antibiotics and nutritional supplements
• Antihypertensives
• I.v. prostacyclin