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Case Number 26: Systemic idiopathic fibrosis associated with aortitis
  1. O Schultz,
  2. S Schuchmann,
  3. G-R Burmester,
  4. F Buttgereit
  1. Department of Rheumatology and Clinical Immunology, Charité University Hospital, D-10117 Berlin, Germany
  1. Correspondence to:
    Professor F Buttgereit;
    frank.buttgereit{at}charite.de

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A 41 year old woman presented with an eight month history of general weakness, relapsing pericardial effusion, vertigo symptoms, and a more recent history of dyspnoea. Routine laboratory tests were normal except for a raised C reactive protein concentration (69 mg/l), a leucocytosis (up to 13.3×109/l), and a dysproteinaemia (raised α2 and γ globulin fractions). Autoantibody tests showed positive rheumatoid factor and negative antinuclear antibody. An idiopathic fibrosclerotic process was suggested by an x ray and magnetic resonance imaging (MRI) examination that showed an increase in heart size, signs of congestion, a thickening of both renal fasciae (up to 15 mm), and a homogeneous and/or nodular mass around the aorta (fig 1A). The diagnosis of a combined mediastinal and retroperitoneal fibrosis was confirmed by biopsy. Moreover, MR angiography of the descending aorta demonstrated the following signs of infrarenal aortitis (fig 1B): (a) stenosis (30–40% lumen reduction); (b) thickening and structural abnormalities of the vessel wall with contrast enhancement.

Combined prednisolone/cyclophosphamide treatment was started. A recent follow up visit (four months later) showed no radiological signs of further progression.

Figure 1

Magnetic resonance imaging showing the thickening of both fasciae (A), a homogeneous and/or nodular mass around the aorta (A), and signs of infrarenal aortitis.

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Footnotes

  • Series editor: Gary D Wright

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