Masterclass
Postural changes, dysphagia, and systemic sclerosis
a Departments of Rheumatology, b and Neurology, c University
Hospital, Nijmegen, the Netherlands
Correspondence to: Dr P Barrera, Department of Rheumatology, University Hospital Nijmegen, PO Box 9101, 6500 HB Nijmegen, the Netherlands.
Accepted for publication 2 April 1998
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Case history |
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In 1993, a 61 year old, previously healthy man, developed
stiffness and skin thickening of the hands and upper limbs, Raynaud's phenomenon, and exertional dyspnea. Examination showed a diffuse systemic sclerosis (SSc), moderate hypertension (160/95 mm Hg), a
reduced pulmonary diffusion capacity (TLC 107% predicted, DLCO 68%
predicted), and asymptomatic oesophageal dysmotility. Treatment with D-penicillamine (D-Pen) 750 mg/day and enalapril was started and, except for extension of the skin involvement to the trunk and
lower limbs, the disease's course was uneventful for three years.
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In August 1996 he was examined in another centre because of complaints
of progressive fatigue, cervical anteflexion, proximal dysphagia,
and weight loss. The patient denied having low back pain, and there
were no symptoms suggesting arthritis, psoriasis, Reiter's syndrome or
inflammatory bowel diseases. Routine laboratory investigations showed
an increased erythrocyte sedimentation rate (ESR) (63 mm/1st h),
creatinine (108 µM/l; normal 60-100 µM/l), aspartate
aminotransferase (ASAT 53 u/l; normal <25), and alanine aminotransferase (ALAT
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