Systemic Weber-Christian disease with reversible bilateral ureteric obstruction.
It has been proposed that idiopathic retroperitoneal fibrosis may be a consequence of 'healed' retroperitoneal lesions of systemic Weber-Christian disease. However ureteric obstruction which is the hallmark of idiopathic retroperitoneal fibrosis, has not been described in systemic Weber-Christian disease. We report a patient with systemic Weber-Christian disease who, during a relapse, developed bilateral ureteric obstruction which resolved when the Weber-Christian disease remitted. The radiological appearances were consistent with a diagnosis of idiopathic retroperitoneal fibrosis, but the clinical course was slightly atypical in that the ureteric obstruction resolved completely and rapidly. Ureteric obstruction can complicate systemic Weber-Christian disease and this observation gives support to the hypothesis that idiopathic retroperitoneal fibrosis is related to systemic Weber-Christian disease.
Brawn
L A
LA
University Department of Therapeutics, Royal Hallamshire Hospital, Sheffield, UK.
Ramsay
L E
LE
Shortland
J R
JR
Williams
J L
JL
D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D008297:Male; D008875:Middle Aged; D010201:Panniculitis, Nodular Nonsuppurative / Q000150:complications* / Q000276:immunology; D012185:Retroperitoneal Fibrosis / Q000473:pathology; D014517:Ureteral Obstruction / Q000209:etiology*
D007070:Immunoglobulin A