Idiopathic Membranous Glomerulonephritis Research Articles

Long-term prognosis of idiopathic membranous glomerulonephritis: Study of 116 untreated patients

One hundred sixteen patients, mainly adults, with idiopathic membranous glomerulonephritis were studied to evaluate their clinical course and long-term prognosis. The onset was marked by a nephrotic syndrome in 88 patients (75.8 per cent) and by a proteinuria without the nephrotic syndrome in 28 patients (24.2 per cent). Clinical remission occurred in 23.4 per cent of cases, clinical improvement in 14.6 per cent, renal insufficiency in 19 per cent (with end-stage renal failure in 9.5 per cent) and the condition remained unchanged in 43 per cent. The actuarial survival curve shows that 76 per cent of the patients were alive at 10 years. Twenty-five per cent of the patients had hypertension during the course of the membranous glomerulonephritis. Seven women had nine pregnancies after the diagnosis of membranous glomerulonephritis was made, with successful deliveries in seven. Renal vein thrombosis was present in five of 16 patients examined for it. Glomerular lesions were classified into three groups, according to the classification of Bariety et al. At the first biopsy, 22 patients were classified as having type I lesions, 79 as having type II lesions and 15 as having type III lesions. The mean interval between discovery of the disease and renal biopsy according to the type of glomerular lesions, is shorter in patients with type I lesions than in those with type II and III lesions. Clinical improvement and clinical remission are more frequent in those with type I lesions than in those with type II and type III lesions. End-stage renal failure was never encountered in those with type I lesions and was present in 13 per cent of those with type II lesions and 7 per cent of those with type III lesions. The different histologic types can correspond to a progression of lesions with time but not to a progressive severity of the disease. In two cases there was complete resolution of the lesions and recovery of the normal appearance of the capillary wall.

Penicillamine Induced Membranous Glomerulonephritis

Renal biopsy material from thirty-five patients, who developed glomerulonephritis whilst receiving penicillamine, was studied. All patients had a membranous glomerulonephritis diagnosed either on 0.5 μ silver stained sections or by electron microscopy and occasionally confirmed by immunofluorescence. In 16 cases light microscopy alone of conventional 3 μ sections showed only a minimal change lesion. Clinical information and follow-up data show that as in idiopathic membranous glomerulonephritis the condition may present as persistent proteinuria, proteinuria going on to a nephrotic syndrome, or as an acute nephrotic syndrome. Proteinuria occurred 1-24 months after penicillamine was started with a median time of 7 months, and was unrelated to the amoung of pinicillamine given. Daily doses of 900-1800 mg/day of penicillamine were given. In 33 cases penicillamine was stopped within 3 months of proteinuria being first noted. The magnitude of the proteinuria may not reach a peak for 2-4 months after being note...

Renal Vein Thrombosis

THE ASSOCIATION between renal vein thrombosis and the nephrotic syndrome is well documented in the literature. Although some investigators have suggested that renal vein thrombosis precedes the onset of the nephrotic syndrome and hence serves as the initiating event,¹more recent observations imply that the nephrotic syndrome usually precedes the development of renal vein thrombosis. We report such a progression of events in a patient who had the nephrotic syndrome and in whom idiopathic membranous glomerulonephritis was found on histologic examination of the kidney. Later, renal vein thrombosis complicated the course of the patient's illness. <h3>Report of a Case</h3> A 54-year-old man was hospitalized with peripheral edema, mild hypertension, and proteinuria of three months' duration. The blood pressure on admission was 150/80 mm Hg, and there was pitting edema (2+) to the level of the knees bilaterally. Results of the remaining aspects of the physical examination were unremarkable. Evaluation

The pathogenesis of experimental membranous glomerulonephritis induced with homologous nephritogenic tubular antigen.

The renal tubular epithelial antigen (Tub-Ag) of rats was solublized by Pronase and purified by gel filtration and acrylamide gel electrophoresis. Purified Tub-Ag was a glycoprotein with S20,W value of 8.4. Utilizing radiolabeled Tug-Ag, a sensitive radioimmunoassay for Tub-Ag and homologous antibody (anti-Tub-Ag) was developed. Tub-Ag activity associated with a protein of the same molecular size was demonstrated in the serum, as well as in Pronase extracts of all the organs tested, including kidney, liver, lung, spleen, intestine, stomach, and heart. The physiochemical properties of the Tub-Ag of rats and its distribution were essentially the same as the Tub-Ag of humans, which had been found in immune deposits in the kidney of some patients with idiopathic membranous glomerulonephritis. Rats were immunized with the purified Tub-Ag emulsified in Freund's complete adjuvant and followed for Tub-Ag and anti-Tub-Ag in the serum, as well as for proteinuria and immunohistological changes in the kidney. Serum Tub-Ag dropped sharply after 20 days, when anti-Tub-Ag appeared in the circulation. Persistent, massive proteinuria appeared still later, more than 30 days after injection, when anti-Tub-Ag disappeared and Tub-Ag reappeared in the serum of some of those rats. In others, anti-Tub-Ag in the serum persisted throughout the observation period of 90 days. The pathology of the kidney of the rats with proteinuria was that of a typical membranous glomerulonephritis; thickening of glomerular capillary walls with granular deposits of gamma-globulin and Tub-Ag was observed. On the basis of these results, Tub-Ag in the serum, probably released from cellular membranes of various organs as a physiological metabolite, is considered to maintain the pathological process in the kidney by providing the antigen continuously to form immune complexes.

Therapeutic Renal Arterial Occlusion for Elimination of Proteinuria

A patient with severe nephrotic syndrome who was too debilitated to undergo surgical nephrectomies, underwent therapeutic bilateral renal artery occlusion by the selective injection of isobutyl 2-cyanoacrylate into the renal arteries. The therapy resulted in dramatic cessation of urine flow and elimination of proteinuria.

Extramembranous glomerulonephritis in childhood: relationship to systemic lupus erythematosus.

Extramembranous glomerulonephritis is an uncommon but distinct pathologic lesion in children. The diagnosis is established by the characteristic light, immunofluorescent, and ultrastructural abnormalities in renal biopsy specimens. This report describes seven of the ten children with this lesion studied in the past 11 years. Emphasis is given to the comparison of four children with idiopathic membranous glomerulonephritis with three others who presented with a nephrotic syndrome but subsequently developed evidence of systemic lupus erythematosus. Two of the latter three children, and three others with SLE and MGN not described in detail, demonstrated deposition of IgA by immunofluorescence along glomerular capillaries. Five of six children with SLE and MGN had microtubular structures in glomerular endothelial cells demonstrable by electron microscopy. These observations suggest that children with MGN require careful and continuing study for evidence of SLE.

Membranous glomerulonephritis mediated by renal tubular epithelial antigen-antibody complex

Autologous renal tubular epithelial antigen was demonstrated together with immunoglobulins and β 1c along the glomerular capillary walls in 4 out of 9 patients with idiopathic membranous glomerulonephritis, and the existence of the clinical entity of glomerulonephritis mediated by tubular epithelial antigen-antibody complexes was suggested. Although no differences in laboratory data were noted between tubular antigen-positive and -negative groups, clinical remission occurred only in the negative group. The fact that no remission was observed in the tubular antigen-positive group may be explained by the continuous supply of the antigen from endogenous sources that maintained the production of immune complexes and subsequent glomerular injury.

Human glomerular basement membrane: chemical composition in glomerulonephritis and pyelonepritis.

Abstract. The chemical composition of glomerular basement membrane (GBM), isolated from 18 kidneys in advanced stage of renal disease, has been studied. On the basis of morphological changes and clinical observations 7 kidneys from 7 patients were classified as showing chronic pyelonephritis, including interstitial nephritis, 5 as chronic membranoproliferative glomerulonephritis, 2 as idiopathic membranous glomerulonephritis, and 4 as chronic glomerulonephritis of unspecified type. Ten normal kidneys were included as controls. For several constituents obvious differences were found between GBM isolated from diseased and normal kidneys, most pronounced for the chronic membranoproliferative glomerulonephritis and idiopathic membranous glomerulonephritis groups. The differences could not be explained solely by contamination of the diseased kidney GBM with collagen. More than one simultaneous process may have contributed to the observed changes in the biochemical composition of diseased GBM.

Electron Microscopy in Surgical Pathology

Utilization of paraformaldehyde or even formalin fixed surgical specimens in combination with rapid (24 hour) processing techniques allow for electron microscopic evaluation of a number of difficult diagnostic problems in histopathology. The principle diagnostic uses of electron microscopy at the present time are in the following areas: 1) Renal biopsies with deviations from the normal observed primarily in glomeruli (tubular abnormalities have been subjected to less study) including, degree of cellularity, abnormalities (primarily thickening) of the glomerular basement membrane, deposits of various sorts in juxtaposition or within the basement membrane, accumulations of mesangial matrix or “glomerular scar fiber,” cellular changes including fusion of foot processes, and more recently demonstration of viral agents in the following lesions; acute glomerulonephritis, lipoid nephrosis, chronic glomerulonephritis, focal glomerulonephritis, idiopathic membranous glomerulonephritis, lupus nephritis, diabetic glomerulosclerosis, eclampsia, and amyloidosis;

Observations on the renal metabolism of protein in experimental and clinical nephrotic syndromes

Renal and peripheral arteriovenous differences (A-V/A) of albumin, peptide-N and α-amino-N were compared in normal and aminonucleoside treated rats. Significant accumulations of peptide-N were demonstrated in the renal venous plasma of nephrotic animals which was supported by similar results obtained when kidney and liver slices were incubated with RISA. Specific activity ratios (F/P) were determined for the TCA soluble (F) and precipitable (P) fractions of plasma from 5 normal persons, 8 patients with idiopathic nephrotic syndrome or membranous glomerulonephritis and 3 with cirrhosis. A significant difference was obtained between the normal and hypoalbuminemic subjects attributed to the greater accumulation of radioactivity in the protein-free filtrate in the latter group. These data suggest accelerated proteolytic activity in experimental and cliincal nephrotic syndromes but do not indicate that other abnormalities of albumin metabolism are not operative or that the defect is specific for renal disease.