Stenosing Ureteritis in Henoch-Schonlein Purpura

Abstract

We report on a boy with Henoch-Schönlein purpura in whom flank pain and gross hematuria developed during the early phase of the disease. Urologic investigations revealed hydronephrosis and ureteral stenosis. Pyeloplasty was done to relieve ureteropelvic junction obstruction. Severe hemorrhagic ureteritis and vasculitis were noted on the ureteral biopsy. Recovery was slow and residual hydronephrosis persisted. Although abdominal pain usually accompanies Henoch-Schönlein purpura, colicky flank pain associated with hematuria should alert the physician to the presence of ureteritis. Recognition and early surgical treatment of this urologic complication of Henoch-Schönlein purpura may prevent a potentially serious outcome.

Total Bilateral Ureteral Replacement for Stenosing Ureteritis in Henoch–Schönlein Purpura

“…The role of corticosteroid treatment for preventing this evolution is uncertain: a favorable response was observed in only 4 cases. More often, there is a complete obstruction at the ureteropelvic junction requiring pyeloplasty without any delay [6, 7, 8, 9]. In most cases, there is no identifiable extrinsic source of proximal obstruction and the ureteral lumen is significantly narrowed but does not contain blood clots or show lithiasis.…”

Masked severe stenosing ureteritis: a rare complication of Henoch-Schönlein purpura

“…The CT urography excluded other causes of ureteral obstruction (such as calcified blood clots or retroperitoneal fibrosis) that are associated with HSP [4,5,16]. The ureteral obstruction did not appear to be associated with the use of cyclophosphamide.…”

“…In an extensive review of the literature, both in English and other languages, 20 previously reported cases in children were found. Of these, 11 have been reported in the English literature [2][3][4][5][6][7][8][9][10]. The paper presented here is the first extensive review of HSP-associated ureteritis.…”

“…On the other hand, remission has been reported in patients without any treatment [2,12,13,20]. Progressive stenosis requiring surgical intervention despite early corticosteroid treatment due to gastrointestinal or renal involvement has also been reported [5,9,11,19]. It seems that the occurrence of mild stenosis, distal to UPJ, is associated with a favorable response to corticosteroids [4,6,21,23].…”

“…Involvement of the lower urinary tract seems unusual, and only few cases of HSP-associated stenosing ureteritis, typically presenting with severe manifestations, have been reported in the English literature [2][3][4][5][6][7][8][9][10]. However, the true incidence and spectrum of this complication remains unknown, as some cases were diagnosed incidentally [7,9,[11][12][13][14].…”