Long-term prognosis of idiopathic membranous glomerulonephritis. Study of 116 untreated patients.
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Biomedical subjects
Publications and source records attributed to D Droz.
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Acute anuric renal failure was observed in two patients with systemic lupus erythematosus (SLE) during the clinical and serologic active phase of the disease. Renal biopsies, performed during the acute episodes, showed only mild and focal mesangial cell proliferation without deposits. In contrast, tubulointerstitial lesions were predominant. Intense granular immune deposits along the tubular basement membrane, or immunofluorescence examination, were suggestive of immune complex deposition. One of these patients had severe high blood pressure and vascular lesions likely induced by immune complexes. In both, renal function was recovered. Immunologically-mediated tubular and vascular lesions in the course of SLE are discussed.
Serial specimens from transplanted kidneys were obtained in 11 patients with dense deposit disease (DDD). The recurrence of DDD was obvious in 9 patients and appeared very early after grafting. Three different types of evolution of the lesions were observed. In 4 patients no modification of the lesions occurred with time, and in 2 of them the dense alteration alone persisted without any other glomerular changes. In 3 patients a progression of the lesions was observed, whereas a regression occurred in 1 other patient. From these observations the following sequence of the morphologic changes can be proposed: the dense alteration appears first and constitutes the specific marker of that disease; the C3 deposition in the kidney occurs later following the appearance of the dense lesion.
Dense deposit disease of the kidney is a rare form of chronic glomerulonephritis frequently associated with serum complement abnormalities (low C3 levels) and a circulating C3 convertase activator of the alternative pathway, the C3 nephritic factor (NF). Eleven patients with end-stage dense deposit disease underwent kidney transplantation. Of the 11, 7 had pretransplant low C3 and NF. In the posttransplant period, persisting low C3 levels were associated with persisting NF, although not quantitatively so. The original glomerular lesion recurred in the graft within 6 months in 9 of 11. Of these 9, 2 had no complement abnormalities either prior to or after transplantation. Pretransplant complement abnormalities were rapidly corrected in 4 of 7 patients whether or not recurrence of the original lesion occurred. Thus, serum complement profiles before and after transplantation are neither predictive nor indicative of recurrence.
The serological and renal changes were studied simultaneously in 115 mice infected with Schistosoma mansoni. IgG and IgM, but not IgA anti-S. mansoni antibodies were detected in the sera, together with circulating immune complexes containing schistosomal antigen. Glomerular mesangial deposits of IgA, IgM and C3 were observed. Despite the strong correlation observed between the occurrence of the circulating immune complexes containing schistosomal antigen and the glomerular deposits, results concerning the behaviour of IgA suggest that portal hypertension and liver damage have a role in the pathogenesis of glomerular lesions.
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