Presentation: Persistent Hematuria after Induction of Remission in Patients with Glomerulonephritis Due to Wegener’s Granulomatosis (2007)

2004 Persistent Hematuria after Induction of Remission in Patients with Glomerulonephritis Due to Wegener’s Granulomatosis

Background: Renal disease activity in Wegener’s Granulomatosis (WG) is monitored by serum creatinine, creatinine clearance, routine urinalysis and microscopic urine examination. Remission has been defined as improvement and stability of renal function as measured by serum creatinine, resolution of hematuria and absence of extra- renal features of disease activity. Persistent proteinuria is not considered an indicator of active renal disease but reflects chronic damage. However, persistent microscopic hematuria with or without red blood cell (RBC) casts has been considered by some authorities as a sign of persistent disease activity. We have questioned this assumption and have sought evidence to suggest that persistent microhematuria may also be a sign of damage.
Purpose: To study patients with WG who have been treated for active disease resulting in resolution of all features of disease activity but continued to have persistent hematuria in face of apparently quiescent disease.
Methods: WG database of 82 patients was accessed to identify patients with persistent microscopic hematuria who appear in all other regards to be in remission. Remission was defined as BVAS/WG=0, stabilization and improvement in serum creatinine with immunosuppressive medications being tapered or discontinued. Inclusion criteria-1) Hematuria with or without cellular casts from active glomerulonephritis; 2) presence of persistent hematuria in remission; and 3) Birmingham Vasculitis Activity Score (BVAS/WG) of 0 (remission). Data on demographics, baseline creatinine, urinalysis and BVAS/WG at the time diagnosis and at the follow up visits were recorded.
Results: A total of 8/82 patients met inclusion criteria (4 females, 4 males). Median age at diagnosis = 38 (range 17-61) years. At the time of active disease median serum creatinine = 1.3mg/dl (range 0.6-5.5 mg/dl) and median BVAS/WG = 6.5 (range 4-21). Baseline urinalysis showed ≥1+ proteinuria and hematuria with red blood cell casts. 7/8 patients were treated with cyclophosphamide. After effective therapy median creatinine = 1.0mg/dl (range 0.4- 1.6mg/dl). Median duration of remission = 11.5 (6-39) months. 5/8 patients relapsed but only 3 patients had a renal relapse. Median duration of second remissions = 21(15-72) months. All patients had persistent microhematuria during the periods of remission. 3/8 patients were in extended remissions and completely off of all immunosuppressive therapies for median duration of 3 (2-4) years but continued to have microhematuria.
Limitations: Retrospective review with absence of biopsies to confirm absence of active renal disease.
Conclusion: Hematuria from WG -glomerulonephritis may persist in the presence of remission and absence of progressive decline in renal function. In this setting aggressive immunosuppressive therapy for presumed active GN is not indicated.

 M.N. Magrey, None; C. Koening, None; G.S. Hoffman, None.