Prostatic involvement in Wegener's granulomatosis

Wegener's granulomatosis involving the prostate gland is unusual. We report 3 cases of this condition in which typical necrotizing granulomas with vasculitis were seen histologically. These lesions may cause gross hematuria or obstructive voiding, including urinary retention. Management includes prostatectomy when the symptoms are severe but an initial trial of aggressive medical therapy may be successful. Treatment requires a knowledge of the natural history of Wegener's granulomatosis and its usual response to medical therapy.     

Prostatic involvement in Wegener's granulomatosis

Two cases of Wegener's granulomatosis presenting with prostatic involvement are described and compiled with the five previously detailed cases. Each of these patients presented with obstructive symptoms, proteinuria, leukocyturia, and hematuria. The urinary sediment normalized with treatment of the underlying granulomatous vasculitis. Wegener's granulomatosis is a rare cause of prostatic obstructive symptoms, but should be considered whenever the relatively unusual entity of granulomatous prostatitis is diagnosed. One patient was initially treated exclusively with trimethoprim-sulfamethoxazole (TMP-SMX). He responded, but noted recurrence during the 15th month of treatment. We also report on this patient's antineutrophil cytoplasmic antibody (ANCA) titers, which correlated with clinical assessment and predicted recurrence 2 months before elevation of the Westergren sedimentation rate (WSR) and clinical diagnosis.     

Wegener's granulomatosis: clinical course in 108 patients with renal involvement.

BACKGROUND: The aim of this study was to evaluate the clinical course of patients with Wegener's granulomatosis and renal involvement, with special reference to relapse rate, renal and patient survival and morbidity from serious infections. METHODS: A retrospective analysis was carried out of 108 patients presenting with Wegener's granulomatosis and active renal disease in eight hospitals in Norway between 1988 and 1998. Multivariate analysis was used to investigate whether selected variables predicted relapse, renal and patient survival and serious infections. RESULTS: Median follow-up was 41.5 months. Twenty-two patients (20.4%) were admitted with a need for dialysis. Complete remission was obtained in 81.5% after a median of 4 months, and 54.7% relapsed after a median of 22. 5 months. Two- and five-year renal survival was 86 and 75%, respectively, and 22.8% developed end-stage renal disease (ESRD). Two- and five-year patient survival was 88 and 74%, respectively, and the cumulative mortality was 3.8 times higher than expected. The relative risk of relapse increased with the use of intravenous pulse cyclophosphamide compared with daily oral cyclophosphamide. Initial renal function predicted renal survival, and low serum albumin and high age at treatment start increased the mortality risk. Thirty one per cent of the patients were hospitalized for serious infections during follow-up. Old age increased the risk of having an infection. CONCLUSIONS: The current treatment of Wegener's granulomatosis does not prevent relapse, development of ESRD and serious treatment-induced infections in a considerable fraction of the patients. Alternative strategies for the management of this disease will be an important objective for further studies.     

Single-center experience with renal transplantation in patients with Wegener's granulomatosis

Between 1980 and 1995, 13 patients with end-stage renal disease due to Wegener's granulomatosis received 14 renal transplants (10 cadaveric, 4 living related). The mean follow-up in the 13 successfully transplanted patients was 50 months (4–107 months). One patient had primary nonfunction and received another graft 4 months later. Three episodes of acute rejection occurred in two patients, and one of these patients lost her graft due to severe vascular rejection 4 months after transplantation. Two patients died with well-functioning grafts (one of metastatic cancer and one of sepsis). One patient presented with perisinusitis and had a mild recurrence of Wegener's disease. None of the patients developed recurrent disease in the transplanted organ. At the last follow-up, the mean creatinine ( ± SD) in the 12 patients with functioning grafts was 1.6 ± 0.6 mg/dl. We conclude that renal transplantation is an excellent treatment for renal failure due to Wegener's granulomatosis. Recurrence of the disease is uncommon in patients under immunosuppression, but careful monitoring is extremely important. Received: 1 July 1996 Received after revision: 6 September 1996 Accepted: 23 September 1996     

Wegener's granulomatosis presenting as a renal mass

Wegener's granulomatosis is a systemic necrotizing vasculitis that usually involves the kidneys, typically causing segmental necrotizing glomerulonephritis. An association between Wegener's granulomatosis and renal cell carcinoma was recently reported. We describe a case of Wegener's granulomatosis presenting as a renal mass in a 72-year-old woman. Histologic examination of the mass revealed granulomatous inflammation, an extremely rare manifestation of this disease. We also review the incidence of renal mass in Wegener's granulomatosis and highlight the importance of excluding the coexistence of renal cell carcinoma.     

Wegener's granulomatosis presenting as renal mass: a case for nephron-sparing surgery

Wegener's granulomatosis (WG) is a systemic disease well recognized by physicians. The upper and lower respiratory symptoms associated with the disease are easily recognized; however, its presentation as a solid renal mass is underappreciated. We present a case of a 61-year-old man who presented with a 5.2-cm renal mass diagnosed as WG after nephron-sparing surgery. The patient presented with mild symptoms suggestive of WG, but the antineutrophil cytoplasmic antibody test was negative. The renal mass and concerns about possible malignancy obscured the diagnosis of WG. This case illustrates that WG should be considered in the differential diagnosis of solid renal masses.     

Wegener's granulomatosis presenting as otomastoiditis. A case report

A 55-year-old male presented with left-sided otorrhoea, hearing loss and tinnitus of 3 months duration. On clinical examination polypoid tissue was seen prolapsing in the external ear canal. A CT scan of the mastoid cells and middle ear showed otomastoiditis with osteolysis. Oral antibiotic therapy and eardrops were started. When a facial nerve paresis appeared one month later, a mastoidectomy was performed. The mastoid cells and middle ear were filled with a connective tissue-like substance. Postoperative corticosteroids were administered. Despite the therapy the facial nerve problem aggravated and the patient developed severe parietotemporal headache, meningeal irritation and somnolence. The diagnosis of neurosarcoidosis was hypothesised. Blood analysis, including c-ANCA's, culture of the otorrhoea and biopsies of the connective tissue were inconclusive. A CT scan of the brain showed thickening of the left tentorium. A biopsy of the dura indicated a diagnosis of Wegener's granulomatosis. The patient was treated with immunosuppressive medication with satisfactory results.     

Unusual evolution in Wegener's granulomatosis: recovery of pulmonary involvement while renal disease progressed to end-stage

A 54-year-old male patient was admitted for acute respiratory distress with fever. He was suffering from chronic sinusitis/rhinitis and had persistent otitis for the past 2 months before admission despite several antibiotics courses. He developed a complex pulmonary involvement (embolism and diffuse alveolar hemorrhage) with acute glomerular disease (proteinuria and hematuria but initially no renal failure). Clinical suspicion of Wegener's granulomatosis was confirmed by the positive high titer of antineutrophil cytoplasmic antibodies (c-ANCA with antiproteinase 3 specificity) and despite a negative nasal biopsy. Treatment including cyclophosphamide and methylprednisolone intravenous pulses permitted pulmonary recovery over 4 weeks contrasting with the development of rapidly progressive glomerulonephritis and polyneuropathy of lower limbs. Renal biopsy showed pauci-immune crescentic and necrotizing glomerulonephritis. However, despite additional plasma exchanges, acute kidney injury worsened and the patient ended up in dialysis. Such a dissociated evolution was unexpected in this case since pulmonary and renal involvements reflected the same pathological process (small vessels vasculitis/capillaritis) and the same pathogenic mechanism (antiproteinase 3 autoantibodies).     

Wegener's granulomatosis with renal involvement: patient survival and correlations between initial renal function, renal histology, therapy and renal outcome.

Patient survival and renal outcome were followed in 25 patients with biopsy confirmed Wegener's granulomatosis and renal involvement. Fourteen out of 25 patients required dialysis on admission, 11/25 patients did not. All patients were treated with a novel protocol comprising methylprednisolone and cyclophosphamide. The median follow-up observation was 36 months (12-113 months). With the exception of 1 patient (who died from causes not related to Wegener's granulomatosis) all patients are alive. Among the patients initially requiring dialysis (n = 14) 4 are in terminal renal failure after 0, 7, 21 and 38 months respectively. In the nondialysis group (n = 11) only 1 patient subsequently required chronic dialysis 30 months after clinical admission. Renal failure was due to non-compliance with immunosuppressive therapy in at least 2 patients. Percentage of obsolescent glomeruli and the degree of tubulointerstitial lesions, but not active glomerular lesions (crescents, necroses) predicted renal outcome. The major cause of renal functional impairment was relapse of Wegener's granulomatosis usually within 2 years after clinical remission. Therefore prolonged treatment with cyclophosphamide for at least 2 years after clinical remission is recommended. Two patients with initially negative immunohistology had a second renal biopsy which revealed de novo appearance of mesangial IgA deposits.     

Correlation of percentage of normal glomeruli with renal outcome in Wegener's granulomatosis

BACKGROUND/AIMS: We examined the correlations between renal biopsy findings in Wegener's granulomatosis and renal function at baseline and 1 year in 22 patients who presented between 1988 and 1999. METHODS: Renal histology was independently reviewed by 2 pathologists who were masked to the clinical data. The primary outcome was the relationship of the percentage of normal glomeruli to reciprocal serum creatinine at baseline and 1 year. Other histologic data were collected using a series of ordinal rating scales. Acute and chronic sum scores were calculated. RESULTS: The median serum creatinine (SCr) at baseline was 3.9 mg/dl (0.8-14 mg/dl). Seven patients initially required hemodialysis, of whom 4 subsequently regained independent renal function. By 1 year of follow-up, the median (SCr) for patients with independent renal function was 1.9 mg/dl (0.9-6.8 mg/dl). Reciprocal SCr at baseline correlated with the percentage of normal glomeruli (r = 0.584, p = 0.005), crescent score (r = -0.595, p = 0.003), interstitial fibrosis as a percentage of cortical surface area (r = -0.669, p = 0.002), interstitial inflammation (r = -0.439, p = 0.041), eosinophil (r = -0.495, p = 0.019), neutrophil score (r = -0.557, p = 0.005), and acute sum score (r = -0.499, p = 0.018). However, only the percentage of normal glomeruli (r = 0.493, p = 0.023), the crescent score (r = -0.452, p = 0.035), and interstitial fibrosis as a percentage of cortical surface area (r = -0.466, p = 0.052) correlated with reciprocal SCr at 1 year of follow-up. CONCLUSION: In this relatively small data set, the percentage of normal glomeruli, the crescent score, and interstitial fibrosis as a percentage of cortical surface area correlated with renal function at both baseline and 1 year of follow-up.     

Wegener's granulomatosis with urethral-penile location. Apropos of a case

The authors report a case of Wegener granulomatosis presenting as a necrotizing urethral tumor in a 44 year-old man. Involvement of the urethra by Wegener granulomatosis in unusual which explains the delay in diagnosis particularly when the urethral localization is isolated. The prognosis is severe despite urinary diversion and appropriate therapy. Five cases from the literature are reviewed. Prednisone and cyclophosphamide therapy for Wegener granulomatosis now gives dramatic results, but an urethral localization could occur in the subsequent course of the disease with the same severe prognosis.     

Wegener's granulomatosis in an 11-year-old child. A case report

Early diagnosis and treatment of Wegener's granulomatosis with cyclophosphamide has considerably improved the prognosis in this previously fatal disease. Experience with this disease in an 11-year-old child is reported.     

Anemia in patients with Wegener's granulomatosis

AIMS: Anemia is commonly observed among patients with chronic kidney disease (CKD). No such information is available for patients with a history of systemic vasculitis. METHODS: We examined the prevalence of anemia, the response to therapy with erythropoiesis-stimulating agents (ESA), and the association of anemia with the kidney function in clinically stable patients with Wegener's granulomatosis in a retrospective, single-center study. RESULTS: The mean hemoglobin concentration of 36 patients (mean age: 58 years; 15 female, 21 male; mean duration of disease: 4.6 years) was 13.0+/-2.1 g/dl, and the mean estimated glomerular filtration rate (eGFR) was 41+/-21 ml/min/1.73 m(2). 14 of 36 patients (38.8%) presented with anemia (hemoglobin concentration < 12 g/dl in women, < 13 g/dl in men, or ESA therapy). In patients with a CKD Stage 3 or 4, anemia was present about twice as much as compared to the Third National Health and Nutrition Examination Survey (NHANES III) population. The hemoglobin concentration, however, was not associated with a change of kidney function (p = 0.1578). CONCLUSIONS: We found a higher prevalence of anemia in patients with Wegener's granulomatosis, as compared to the NHANES III population. The hemoglobin concentrations showed no association with changes of kidney function.     

Pregnancy in a patient with Wegener's granulomatosis - a case report

Background: Pregnancy in patients with Wegener's granulamotosis (WG) is rare, and differential diagnosis of WG flare and preeclampsia is difficult. Case: A pregnant 35 year old with WG was referred with diagnosis of severe preeclampsia; caesarean section was performed. Intubation of the patient was difficult due to subglottic stenosis. Because of the clinical symptom, the case was considered preeclampsia, but p-ANCA of the patient was positive. In pregnancies with WG, differential diagnosis of WG flare-ups from preeclampsia should be made from clinical symptoms and laboratory findings. Serum ANCA titers are not useful in the differential diagnosis of WG flare-ups and preeclampsia because it may be positive in preeclampsia. Conclusion: Differential diagnosis of WG flare-up and preeclampsia should be made by clinical features. In the patients with subglottic stenosis, general anesthesia should not be preferred due to the probability of difficult intubation.