Hydronephrosis caused by a relapse of granulomatosis with polyangiitis (Wegener’s)

Abstract

We describe a case of relapsed granulomatosis with polyangiitis (Wegener's) (GPA) that presented with abdominal pain. (18)F-fluoro-2-deoxy-D: -glucose positron emission tomography (FDG-PET)/computed tomography (CT) clearly depicted an inflammation of the left peri-iliac arterial soft tissue, which was thought to be the cause of the ureteral obstruction and hydronephrosis. Our case shows that peri-iliac arterial inflammation occurs in GPA and causes hydronephrosis. In addition, FDG-PET/CT is a useful tool for management of this systemic inflammatory disease.

Wegener’s granulomatosis and retroperitoneal fibrosis: a case report and review of the literature

“…30 We also have to remark that in most of the reported cases a mass biopsy has not been performed. However, referring to our patient, the previous histological finding of a granulomatous lesion (uncommon in IgG4-RD) and the negativity of IgG4 serum levels strengthened the GPA diagnosis (Table 1 18,[25][26][27][28][31][32][33][34][35][36][37][38][39][40][41][42][43][44] ). Urogenital complications of Wegener's granulomatosis have been shown to be sensitive to corticosteroid and cyclophosphamide therapy.…”

“…Urogenital complications of Wegener's granulomatosis have been shown to be sensitive to corticosteroid and cyclophosphamide therapy. 31,32 Because of the toxicity associated with the long-term administration of cyclophosphamide, a number of alternative regimens have been investigated as initial therapy, though none has displaced the regimen of intravenous cyclophosphamide plus oral glucocorticoids. The treatment of choice for patients who cannot receive or who decline cyclophosphamide is rituximab.…”

Spontaneous splenic rupture complicating granulomatosis with polyangiitis (Wegener's granulomatosis)

“…For instance, hydronephrosis due to peri-iliac arterial inflammation or spontaneous perirenal hematoma has been reported as a complication of GPA. 6,7 If abdominal manifestations or vasculitis had been evaluated and identified before the initiation of treatment, or even after splenic infarction was suspected, more aggressive therapy, including cyclophosphamide along with a higher dose of corticosteroids should have been used in the present case.…”