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Systemic sclerosis (scleroderma) is a multisystem connective tissue disease of unknown aetiology, characterised by progressive fibrosis of the skin and internal organs including the lungs and gastrointestinal tract.1 Pathological calcification of soft tissues (known as calcinosis) is a common feature in the CREST syndrome of scleroderma (calcinosis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia). By contrast, internal organ calcification is rare, and isolated cases of spinal calcinosis and calcific constrictive pericarditis have been reported.2 3 We report here the cases of two patients with systemic sclerosis whose CT examination disclosed extensive brain calcifications.
Case 1, a 48 year old man was referred to hospital because of polyarthralgia involving the wrists and ankles, Raynaud’s phenomenon, and tightness of hand skin. The patient’s wife had noticed that during the previous months, he had a slowed mentation and a depressive mood. Physical examination disclosed a sclerodactyly but no telangiectasias. Routine haematological tests were normal. Antinuclear antibodies were positive at a 1/2000 dilution with nucleolar fluorescence. Rheumatoid factors, antidouble stranded DNA and antiphospholipid antibodies were negative. There was no cryoglobulinaemia. Complement was normal. Lung function tests showed a restrictive syndrome (forced vital capacity=75% predicted). Chest radiography was normal, as were oesophageal manometry and cardiac ultrasonographic examination. A diagnosis of systemic sclerosis …