© 2003 by BMJ Publishing Group & European League Against Rheumatism
UNUSUAL AND MEMORABLE
Case Number 26: Systemic idiopathic fibrosis associated with aortitis
Series editor: Gary D Wright
Department of Rheumatology and Clinical Immunology, Charité University Hospital, D-10117 Berlin, Germany
Correspondence to:
Correspondence to:
Professor F Buttgereit;
frank.buttgereit@charite.de
Keywords: fibrosis; aortitis
| The first 150 words of the full text of this article appear below. |
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 13.3x109/l), and a dysproteinaemia (raised 
2 and 
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
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