Association of vasculitic glomerulonephritis with membranous nephropathy: a report of 10 cases.
نویسندگان
چکیده
BACKGROUND The concomitant occurrence of a vasculitic glomerulonephritis and membranous nephropathy in the same patient is unusual. We report data on 10 patients with this unusual combination. METHODS Ten patients (nine males/one female; median age 63.5 years, range 30-70 years) presented between 1981 and 1995 with: acute renal failure (n = 3), nephrotic syndrome (n = 4), non-nephrotic range proteinuria and renal insufficiency (n = 3). The median serum creatinine at presentation was 296 mumol/l (range 65-1749 mumol/l). One patient had a vasculitic transformation from membranous nephropathy 5 years after the original presentation, coincident with an acute deterioration of renal function requiring dialysis; in all other patients the two glomerular disorders were seen together at presentation. Treatment was with oral prednisolone and cyclophosphamide (eight patients), of whom one also had plasma exchange; and oral prednisolone and azathioprine (one patient). Specific immunosuppressive treatment was withheld in one patient with histological evidence of chronic renal damage. Sera from four patients out of nine tested were positive for ANCA. RESULTS After a median follow-up of 3.5 years (range 2 months-10 years), renal function had improved in three patients and remained stable in two. Two patients required renal replacement therapy. Three patients had died: one was ANCA-negative and died of a systemic vasculitis, and the other two died of sepsis. CONCLUSION Membranous nephropathy complicated by a vasculitic glomerulonephritis: (1) has a more aggressive clinical course than membranous nephropathy alone, (2) appears to have an association with ANCA, (3) should be considered in those patients with an accelerated decline in renal function, and (4) may respond to treatment with immunosuppressive drugs.
منابع مشابه
Development of IgA nephropathy 14 years after diagnosis of membranous nephropathy.
Membranous nephropathy is characterized clinically by nephrotic proteinuria and pathologically by the presence of electron dense IgG deposits below the epithelium in glomerular basement membrane. Although about 20–30% of cases of membranous nephropathy are secondary, the majority of cases are idiopathic. The clinical features of IgA nephropathy include haematuria and proteinuria. Although IgA n...
متن کاملCo-existence of Type 1 Diabetes Mellitus and Nephrotic Syndrome with Membranous Glomerulonephritis in a 6 Year Old Boy: Report of a Case
Background: Nephrotic syndrome and type 1 diabetes mellitus are two common chronic conditions in children. Co-existence of these conditions is an extremely rare finding. Case presentation: This report presents a 6-year-old boy who was diagnosed with nephrotic syndrome five years ago and type 1 diabetes mellitus in infancy. Renal biopsy showed membranous glomerulonephritis, which is a rare h...
متن کاملReport of a Case of Immunoglobulin A Nephropathy in a Patient Without a Family History of Kidney Disease
Introduction: IgA Nephropathy is the most common form of glomerulonephritis and the major cause of end-stage renal disease. Gross or microscopic hematuria is a common symptom of hematuria and occurs in less than 5% to 10% of patients with IGM with rapidly progressive glomerulonephritis. The aim of this study was to investigate the diagnostic biomarkers of this disease after biopsy. Methods: IgA...
متن کاملClinicopathologic features and outcome of membranous nephropathy in Markaz Tebi children hospital
Background and Objectives: This research study was conducted to determine the correlation between the clinicopathologic features and the outcome of membranous nephropathy. Materials and Methods: Data were retrospectively reviewed from all patients with a diagnosis of membranous nephropathy. Demographic, ...
متن کاملCoexistant of Fabry Disease and IgA Glomerulonephritis in a 39 year old male
Anderson-Fabry disease is a rare inherited X-linked lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase A. Hereby we report a 39 year old male that presented with proteinuria and edema. Histopathologic, immunofluorescence and ultrastractural examination of renal tissue were in favor of Fabry disease in associate with IgA nephropathy. Fabry's disease associated ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
دوره 12 5 شماره
صفحات -
تاریخ انتشار 1997