Recurrence and spontaneous remission of nephrotic syndrome in secondary renal amyloidosis

Secondary systemic amyloidosis (AA) occurs in association with chronic inflammatory disorders and chronic infections. Regression can occur after therapeutically induced remission of the underlying disease; spontaneous remissions has been reported infrequently. We report a 61 year-old woman, with antecedent pulmonary tuberculosis, who developed a nephrotic syndrome at the time of a respiratory infection. Renal biopsy showed secondary amyloidosis. Remission in the nephrotic syndrome appeared spontaneous, but it recurred in the course of pneumonia, and had a second spontaneous remission a maintained at present.     

Renal cell carcinoma co-existent with other renal disease: clinico-pathological features in pre-dialysis patients and those receiving dialysis or renal transplantation.

BACKGROUND: Patients on chronic dialysis are prone to developing acquired cystic kidney disease (ACKD), which may lead to the development of renal cell carcinoma (RCC). The risk factors for the development of RCC so far have not been determined in pre-dialysis patients with co-existent renal disease. The aim of this study was to evaluate the clinico-pathological features of RCC in pre-dialysis patients with associated renal diseases or in those undergoing chronic dialysis and renal transplantation. METHODS: We studied 32 kidneys from 31 patients with RCC and associated renal diseases. Of those, 18 kidneys were from 17 patients not on renal replacement therapy (RRT) when diagnosed with RCC; 14 patients received dialysis or dialysis followed by renal transplantation. Several clinico-pathological features were analysed and compared between the two groups. RESULTS: Overall, there was a preponderance of males (75%); nephrosclerosis was the predominant co-existent disease (31%). The median intervals from renal disease to RCC in the dialysis and transplanted groups were significantly longer than in the pre-dialysis group (15.8+/-1.1 vs 2.4+/-0.7 years, P<0.0001). In contrast to pre-dialysis RCC, the dialysis and transplant RCC groups had greater frequency of ACKD (100 vs 28%, P<0.0001), papillary type RCC (43 vs 11%, P<0.05) and multifocal tumours (43 vs 5%, P<0.05). At the end of the study, 71% of dialysis and transplanted patients and 72% of pre-dialysis patients were alive. CONCLUSIONS: ACKD develops in dialysis patients, as it does in those with renal disease prior to RRT. The duration of renal disease, rather than the dialysis procedure itself, appears to be the main determinant of ACKD and RCC. The RCC occurring in patients with ACKD and prolonged RRT is more frequently of the papillary type and multifocal than the RCC occurring in patients with no or few acquired cysts and a short history of renal disease. Long-term outcomes did not differ between the two groups.     

Familial incidence of C3 nephritic factor

C3 nephritic factor (NEF) has been found in 3 siblings presenting different (or none) degrees of renal disease. Other relatives, including their dead father, suffered from a renal illness. In 2 of the siblings, NEF activity was restricted to IgG1 and IgG3 subclasses. Familial NEF incidence and a shared C3 allotype and a common HLA haplotype including BfS alleles for the 3 NEF-positive siblings suggest that at least in our cases genetical factors may be involved in NEF generation.     

Idiopathic membranous nephropathy in an elderly patient with type 2 diabetes mellitus

We report an 85 years-old patient with type 2 diabetes mellitus and both clinical and biochemical nephrotic syndrome. The renal biopsy showed membranous nephropathy at stage I-II. There was no evidence of malignancy. The patient was treated with steroids, and two months later the proteinuria had not improved. The objects under discussion are the factors that should lead to suspect the existence of glomerulonephritis, other than diabetic glomerulosclerosis, suggesting the need for kidney biopsy. We also focus on the prognostic and therapeutic relevance, as well as on the common pathogenic aspects.     

Simultaneous presence of antibodies against the glomerular basement membrane and anti-myeloperoxidase antibodies in 2 patients with rapidly progressive glomerulonephritis

We report two patients with rapidly progressive glomerulonephritis without alveolar hemorrhage. Renal biopsy showed extracapillary glomerulonephritis with linear deposits of immunoglobulin G. Serologically anti-glomerular basement membrane antibodies (Ac AMBG) and ANCA anti-myeloperoxidase were present. All patients were treated with steroids, cyclophosphamide and plasma exchange. One patient needed dialysis, and other one died from a renal biopsy complication. We discuss the epidemiologic, pathogenic and prognostic aspects of this association.     

Complement factor H variants I890 and L1007 while commonly associated with atypical hemolytic uremic syndrome are polymorphisms with no functional significance

Mutations and polymorphisms in the gene-encoding factor H (CFH) are associated with atypical hemolytic uremic syndrome, dense deposit disease, and age-related macular degeneration. Many of these CFH genetic variations disrupt the regulatory role of factor H, supporting the concept that dysregulation of complement is a unifying pathogenic feature of these disorders. Evidence of a causal relationship with the disease is, however, not available for all CFH genetic variations found in patients, which is a potential cause of misinterpretations with important consequences for the patients and their relatives. CFH I890 and L1007 are two genetic variations repeatedly associated with atypical hemolytic uremic syndrome and also found in patients with dense deposit disease and age-related macular degeneration. Here we report an extensive genetic and functional analysis of these CFH variants. Our results indicate that I890 and L1007 segregate together as part of a distinct and relatively infrequent CFH haplotype in Caucasians. Extensive analysis of the S890/V1007 (control) and I890/L1007 (disease-associated) factor H protein variants failed to provide evidence that these amino acid changes have functional implications. Thus, the presence of the I890 and L1007 variants in healthy individuals and their high frequency in sub-Saharan African and African-American populations strongly suggest that I890 and L1007 are rare factor H polymorphisms unrelated to disease.