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Idiopathic Retroperitoneal Fibrosis. A Retrospective Analysis of 60 Cases
Author(s) -
BAKER L. R. I.,
MALLINSON W. J. W.,
GREGORY M. C.,
MENZIES E. A. D.,
CATTELL W. R.,
WHITFIELD H. N.,
HENDRY W. F.,
WICKHAM J. E. A.,
JOEKES A. M.
Publication year - 1987
Publication title -
british journal of urology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.773
H-Index - 148
eISSN - 1464-410X
pISSN - 0007-1331
DOI - 10.1111/j.1464-410x.1987.tb05028.x
Subject(s) - medicine , ureterolysis , surgery , polyuria , retroperitoneal fibrosis , renal function , prednisolone , nausea , erythrocyte sedimentation rate , fibrosis , diabetes mellitus , endocrinology
Summary— Sixty patients with idiopathic retroperitoneal fibrosis presenting between 1965 and 1984 are reviewed. Their mean age at presentation was 56 years and the male: female ratio was 3:1. The commonest presenting symptoms were flank and abdominal pain, weight loss, nausea and polyuria. Physical examination was usually normal, expect for the presence of hypertension. Anaemia and elevation of erythrocyte sedimentation rate were usually present. Proteinuria was found in less than a third of patients at presentation and significant bacteriuria was uncommon. The correct diagnosis was made or suspected in very few patients before referral. The cumulative actuarial survival rate was 86% at 1 year and 78% at 2 years. Seventeen patients died; they were significantly older and more uraemic at the time of referral than those who survived. A few patients did well with either corticosteroid therapy or ureterolysis alone. In the majority, both operation and steroid treatment were necessary. In bilateral obstruction with residual function in both kidneys, bilateral ureterolysis proved superior to unilateral operation (each followed by steroid therapy) in conserving renal function. Operation alone or steroid therapy alone should be considered in cases where steroids or surgery repectively present particular hazards. The less traumatic unilateral operation should be considered in poor risk patients and in those whose renal function is absent on one side. In many survivors, disease activity has persisted for many years. Life‐long follow‐up is recommended.