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Postural changes, dysphagia, and systemic sclerosis
  1. Pilar Barreraa,
  2. Alphons A den Broedera,
  3. Frank H J van den Hoogena,
  4. Baziel G M van Engelenb,
  5. Leo B A van de Puttea
  1. aDepartments of Rheumatology, band Neurology, cUniversity Hospital, Nijmegen, the Netherlands
  1. Dr P Barrera, Department of Rheumatology, University Hospital Nijmegen, PO Box 9101, 6500 HB Nijmegen, the Netherlands.

https://doi.org/10.1136/ard.57.6.331

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    Case history

    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.

    Key messages

    • The cardinal manifestation of most myopathies is proximal weakness. This symptom may be mild and confused with fatigue, but also so severe as to result in head drop and dysphagia.

    • Abnormal “liver function tests” may be manifestations of myopathy.

    • With a few exceptions, such as D-Pen, drugs do not cause PM but a toxic non-inflammatory myopathy.

    • Approximately 40%–60% of patients develop adverse events while receiving D-Pen treatment. Although infrequently, the drug induces autoimmune phenomena that fully mimic idiopathic autoimmune diseases.

    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 38 u/l; normal <25) and a slight proteinuria (0.44 g/day).

    The diagnosis ankylosing spondylitis was considered mainly because of his posture. HLA B27 typing was positive, however radiological examination showed only cervical arthrosis and periarticular calcifications in hands and feet. Radiographs and computed tomography of the pelvic joints showed no evidence of sacroiliitis. The anteflexion of the head was considered to …

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