Clinical features

Skin - this is the predominant organ to be affected. A severe Raynaud's phenomenon is a common initial presentation.

• hands and feet - shiny non-splitting oedema with tight, atrophic skin, tightly adherent to the subcutaneous tissue. Mobility is lost.
• face - strikingly line-free forehead, small beaked nose, 'puckered mouth' (radial furrowing around the lips), multiple telangiectasia (in advanced cases). The patient may be unable to close their eyes due to skin tethering.
• mouth and soft palate - may shrink so that denture wearers complain of poorly fitting dentures.

Other possible manifestations include:

• musculoskeletal: arthralgia, myositis, non-erosive arthritis, "sausage fingers" from soft tissue swelling
• gastrointestinal: reflux oesophagitis, dysphagia, bowel dilatation resulting in loss of peristalsis, malabsorption, and steatorrhoea
• pulmonary: pulmonary fibrosis (may cause cor pulmonale), reflux pneumonitis, or rarely, pleural effusion or alveolar cell carcinoma. A late complication is a restriction of chest wall movement due to a tightening and thickening of the overlying skin
• renal: renal failure, malignant hypertension. Renal disease (with fibrinoid necrosis on histology) is the most common cause of death.
• cardiac: pericarditis, myocarditis, arrhythmias, left ventricular failure
• eyes - Sjogren's syndrome, keratoconjunctivitis