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Retroperitoneal fibrosis due to Wegener’s granulomatosis: a misdiagnosis as tuberculosis

2002; Elsevier BV; Volume: 113; Issue: 2 Linguagem: Inglês

10.1016/s0002-9343(02)01170-1

1555-7162

Hassane Izzedine, Aude Servais, Vincent Launay‐Vacher, Gilbert Deray,

Abdominal vascular conditions and treatments

Retroperitoneal fibrosis may occur as an isolated finding or in association with drugs, medical conditions, such as vasculitis (e.g., Wegener’s granulomatosis), or infection (e.g., tuberculosis). We report a case of retroperitoneal fibrosis due to Wegener’s granulomatosis that was mistaken for tuberculosis. A 51-year-old man presented with abdominal pain, urinary symptoms, weight loss, and fever in October 2000. Abdominal sonography and intravenous urography showed a left hydronephrosis with a nonsecreting kidney, and a compensating hypertrophied right kidney. Retroperitoneal fibrosis sheathing the ureter was discovered during a left nephrectomy. The pathological examination showed epithelioid granulomas with necrosis, giant cells, and an infiltrate of lymphoepithelioid cells consistent with a diagnosis of retroperitoneal fibrosis–related tuberculosis. Serum creatinine level was 106 μmol/L before surgery and 95 μmol/L after surgery. Antituberculous treatment comprising rifampicin, isoniazid, ethambutol, and pyrazinamid was started. Three months later, he developed a rapid, progressive oliguric renal insufficiency in his right kidney (serum creatinine, 500 μmol/L). Right percutaneous nephrostomy and ureteral catheterization using double J stents were performed, without renal function improvement. He was then referred to the nephrology department. Laboratory findings showed a C-reactive protein level of 133 mg/L, a serum creatinine level of 550 μmol/L, proteinuria of 2.5 g/d, and microscopic hematuria. A transjugular kidney biopsy specimen disclosed a pauci-immune crescentic glomerulonephritis with necrotizing vasculitis. He had also developed anemia and dyspnea. Thoracic computed tomographic scan and bronchic endoscopy evaluation showed an alveolar hemorrhage. A diagnosis of Wegener’s granulomatosis was made. Cytoplasmic antineutrophil cytoplasm antibodies to proteinase 3 were detected in a titer of 1:320. The patient received methylprednisone (1 g/d) for 3 consecutive days and one cyclophosphamide pulse of 1000 mg, relayed by prednisone (1 mg/kg/d) associated with a monthly cyclophosphamide pulse. One year later, renal function improved partially (serum creatinine, 230 μmol/L; proteinuria, 1 g/24 h, without hematuria). Retroperitoneal fibrosis is uncommon, with a prevalence of 1 in 100,000 persons. It leads to extensive fibrosis throughout the retroperitoneum, causing ureteric obstruction and hydronephrosis (1Amis E.S. Retroperitoneal fibrosis.J Roentgenol. 1991; 157: 321-329Crossref PubMed Scopus (173) Google Scholar). Prostatitis is the most common extrarenal urogenital manifestation of Wegener’s granulomatosis (2Middleton G. Karp D. Lee E. Cush J. Wegener’s granulomatosis presenting as lower back pain with prostatitis and ureteral obstruction.J Rheumatol. 1994; 21: 566-569PubMed Google Scholar). Retroperitoneal fibrosis may appear as the sole preliminary symptom of Wegener’s granulomatosis (3Metselaar H.J. ten Kate F.J. Weimar W. Ureter obstruction as a complication of Wegener’s granulomatosis.Eur Urol. 1985; 11: 63-64PubMed Google Scholar, 4Adelizzi R.A. Shockley F.K. Pietras J.R. Wegener’s granulomatosis with ureteric obstruction.J Rheumatol. 1986; 13: 448-451PubMed Google Scholar, 5Leche J. Feuilhade C. Ferroir J.P. et al.Wegener’s disease of urologic and neurologic onset.Ann Med Interne (Paris). 1985; 136 (in French): 353PubMed Google Scholar, 6ter Maaten J.C. Franssen C.F.M. Daenekindt A.A. Hoorntje S.J. Triple Wegener’s granulomatosis in the urogenital tract.Nephron. 1993; 63: 358-359Crossref PubMed Google Scholar). In a report of tuberculosis associated with Wegener’s granulomatosis (7Gordon C. Luqmani R. Fields P. et al.Two cases of “Wegener’s tuberculosis”.Br J Rheumatol. 1993; 32: 143-149Crossref PubMed Scopus (19) Google Scholar), tuberculosis was localized in the lungs in one patient and in the synovial fluid in another, but not in the retroperitoneum. Urogenital complications of Wegener’s granulomatosis have been shown to be sensitive to corticosteroid and cyclophosphamide therapy (2Middleton G. Karp D. Lee E. Cush J. Wegener’s granulomatosis presenting as lower back pain with prostatitis and ureteral obstruction.J Rheumatol. 1994; 21: 566-569PubMed Google Scholar, 3Metselaar H.J. ten Kate F.J. Weimar W. Ureter obstruction as a complication of Wegener’s granulomatosis.Eur Urol. 1985; 11: 63-64PubMed Google Scholar, 4Adelizzi R.A. Shockley F.K. Pietras J.R. Wegener’s granulomatosis with ureteric obstruction.J Rheumatol. 1986; 13: 448-451PubMed Google Scholar, 5Leche J. Feuilhade C. Ferroir J.P. et al.Wegener’s disease of urologic and neurologic onset.Ann Med Interne (Paris). 1985; 136 (in French): 353PubMed Google Scholar, 6ter Maaten J.C. Franssen C.F.M. Daenekindt A.A. Hoorntje S.J. Triple Wegener’s granulomatosis in the urogenital tract.Nephron. 1993; 63: 358-359Crossref PubMed Google Scholar, 8Baker S.B. Robinson D.R. Unusual renal manifestations of Wegener’s granulomatosis. Report of two cases.Am J Med. 1978; 64: 883-889Abstract Full Text PDF PubMed Scopus (55) Google Scholar, 9Le Thi Huong D. Papo T. Piette J.C. et al.Monthly intravenous pulse cyclophosphamide therapy in Wegener’s granulomatosis.Clin Exp Rheumatol. 1996; 14: 9-16PubMed Google Scholar) (Table). However, a case of Wegener’s granulomatosis that responded to antituberculous therapy has been reported (10Toyoshima M. Chida K. Suda T. et al.Wegener’s granulomatosis responding to antituberculous drugs.Chest. 2001; 119: 643-645Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar), suggesting that antituberculous drugs such as trimethoprim-sulfamethoxazole may eliminate causal or precipitating agents in Wegener’s granulomatosis.TableStudies of Retroperitoneal Fibrosis in Wegener’s GranulomatosisStudy Author (Reference)Age (years)SexSymptomsRetroperitoneal Fibrosis as First SymptomMedical TherapyUrological TherapyCourseMiddleton 2Middleton G. Karp D. Lee E. Cush J. Wegener’s granulomatosis presenting as lower back pain with prostatitis and ureteral obstruction.J Rheumatol. 1994; 21: 566-569PubMed Google Scholar65MLower back pain, intermittent fever, urinary hesitancy, dribbling, nocturia, weight lossNoCyclophosphamideBalloon dilatation, ureteral stentResolution of obstructive syndrome within 4 weeks; 9 months later, serum creatinine level at 1.8 to 2 mg/dL; symptom free after 11 months without therapyMetselaar 3Metselaar H.J. ten Kate F.J. Weimar W. Ureter obstruction as a complication of Wegener’s granulomatosis.Eur Urol. 1985; 11: 63-64PubMed Google Scholar50MAbdominal painYesCorticotherapy, cyclophosphamide—Improvement of renal function; hemodialysis discontinued after 4 monthsAdelizzi 4Adelizzi R.A. Shockley F.K. Pietras J.R. Wegener’s granulomatosis with ureteric obstruction.J Rheumatol. 1986; 13: 448-451PubMed Google Scholar30MPain in the right buttock and leg, nausea and vomiting, intermittent fevers, fatigue, weight lossYesCyclophosphamide, corticotherapy—Leche 5Leche J. Feuilhade C. Ferroir J.P. et al.Wegener’s disease of urologic and neurologic onset.Ann Med Interne (Paris). 1985; 136 (in French): 353PubMed Google Scholar37FAnuria, intermittent fever, abdominal pain, fatigueYesCorticotherapy, cyclophosphamide—Remissionter Maaten 6ter Maaten J.C. Franssen C.F.M. Daenekindt A.A. Hoorntje S.J. Triple Wegener’s granulomatosis in the urogenital tract.Nephron. 1993; 63: 358-359Crossref PubMed Google Scholar49MPersistent pain in the groinYesCyclophosphamide, corticotherapyUreterolysisRenal function deteriorated gradually during the following 7 years; undergoing chronic intermittent hemodialysisBaker 8Baker S.B. Robinson D.R. Unusual renal manifestations of Wegener’s granulomatosis. Report of two cases.Am J Med. 1978; 64: 883-889Abstract Full Text PDF PubMed Scopus (55) Google Scholar60FFatigue, weakness, intermittent fever, sweats and chillsNoCyclophosphamide, corticotherapyTransureteroureterostomyRemissionLe Thi Huong 9Le Thi Huong D. Papo T. Piette J.C. et al.Monthly intravenous pulse cyclophosphamide therapy in Wegener’s granulomatosis.Clin Exp Rheumatol. 1996; 14: 9-16PubMed Google Scholar56MRenal colic, hematuriaNoCorticotherapy, cyclophosphamide—Alive, in remission 5 years later; persistent asymptomatic ureteral stenosis without hydronephrosisLe Thi Huong 9Le Thi Huong D. Papo T. Piette J.C. et al.Monthly intravenous pulse cyclophosphamide therapy in Wegener’s granulomatosis.Clin Exp Rheumatol. 1996; 14: 9-16PubMed Google Scholar72FAnuriaNoCorticotherapy, cyclophosphamideSurgical therapy, double J stentsStents removed after 2 months; died 3 years later from acute respiratory failureF = female; M = male. Open table in a new tab F = female; M = male. Awareness that retroperitoneal fibrosis can be the sole preliminary symptom of Wegener’s granulomatosis can prevent misdiagnosis, and hence improve therapeutic approaches.