ABSTRACT
We describe a 20-year-old female with systemic lupus erythematosus (SLE) who developed renal failure during continuous ambulatory peritoneal dialysis (CAPD). The patient was treated with mizoribine (MZR). MZR peak concentration was 1.8 mug/ml and was sufficient for clinical efficacy as measured by serological data. Treatment with MZR was safe and useful even while undergoing CAPD. Achieving optimal MZR blood concentration was important for treatment of SLE, even though the patient was in end-stage renal failure.
Subject(s)
Kidney Failure, Chronic/therapy , Lupus Nephritis/therapy , Peritoneal Dialysis , Ribonucleosides/therapeutic use , Female , Humans , IMP Dehydrogenase/antagonists & inhibitors , Immunosuppressive Agents/blood , Immunosuppressive Agents/therapeutic use , Kidney Failure, Chronic/complications , Lupus Nephritis/complications , Ribonucleosides/blood , Treatment Outcome , Young AdultABSTRACT
In this report we describe the case of identical twin sisters that developed systemic lupus erythematosus (SLE). These patients have in common major histocompatibility complex class I and class II alleles and identical red blood cell antigens, which is a clear indication of monozygotic twins. Both twins showed high titers of anti-dsDNA antibody. However, only one of them manifested signs of lupus psychosis and was positive for the LE test, rheumatoid factor, anti-Scl 70, anti-SSA, and antiribosomal P antibodies. Both sisters lived together; therefore, the environmental factors were considered to be the same. Interestingly, these patients expressed different types of autoantibodies and the manifestation of disease was also quite different. When one of the twins was diagnosed with SLE, we began to closely follow up signs of the disease in the other twin periodically. This enabled us to promptly diagnose the second twin with SLE and she was successfully treated without progression of the disease. It is important to mention that following up the subsequent history of an identical twin diagnosed with SLE allowed early detection of the disease in the other twin.
ABSTRACT
The origin of crescent-forming cells in crescentic glomerulonephritis has not been clarified in spite of the application of monoclonal antibodies (mAbs) against glomerular epithelial cells or monocytes/macrophages. This study was undertaken to characterize the cellular composition of crescents using a new marker, mAb OS-3, produced against macrophagic cells derived from podocytes in normal rat glomerular culture. Monoclonal antibody OS-3 was confirmed to be reactive with some normal epithelial cells of Bowman's capsule. Female Wistar Kyoto rats were injected with rabbit antiglomerular basement membrane (GBM) serum and killed at 2 h, 1, 3, 7, 14 days and 2 months, respectively. The mAb OS-3-positive cells were segmentally observed in glomeruli at 3 days, increased at 14 days, but decreased at 2 months. These cells lacked reactivity with antipodocalyxin in double immunofluorescence (IF) staining. In immunoelectron microscopy of a glomerulus on day 3 and 7, however, reaction products were observed within cells located on the outer surface of the GBM, which were considered to be podocyte in terms of its localization. In conclusion, we have shown a possibility that damaged podocytes partly constitute crescent-forming cells with phenotypic changes, visualized by positive staining with mAb OS-3. We propose a novel concept of crescent formation, suggesting that crescents may be partly composed of phenotypically changed cells, which could not be detected by typical markers for glomerular epithelial cells or monocytes/macrophages.
Subject(s)
Antibodies, Monoclonal , Glomerulonephritis/immunology , Kidney Glomerulus/immunology , Macrophages/immunology , Podocytes/immunology , Animals , Autoantibodies , Bowman Capsule/immunology , Disease Models, Animal , Epithelial Cells/immunology , Female , Fluorescent Antibody Technique , Glomerulonephritis/physiopathology , Kidney Glomerulus/ultrastructure , Macrophages/ultrastructure , Mice , Mice, Inbred BALB C , Microscopy, Immunoelectron , Phenotype , Podocytes/ultrastructure , Rabbits , Rats , Rats, Inbred WKY , Time Factors , Tissue Culture TechniquesABSTRACT
BACKGROUND: We established the reversible and the prolonged models of mesangial proliferative glomerulonephritis (GN) with anti-Thy 1 antibody 1-22-3. However, the essential factors leading to the prolonged glomerular alterations have not been identified. METHODS: The expressions of several chemokines and cytokines were compared in the reversible and the prolonged models. Expression of fractalkine and the number of the fractalkine receptor CX3CR1-positive cells in the glomeruli in the prolonged model were significantly higher than those in the reversible model. Then, the localization of fractalkine and the characteristics of CX3CR1+ cells were analyzed in glomeruli. To elucidate the significance of the fractalkine expression, we analyzed the expression in the model treated with angiotensin II receptor antagonist, candesartan. RESULTS: Immunostaining of fractalkine was detected on endothelial cells on the fifth day, and fractalkine staining also was detected in the mesangial area on day 14. Major parts of the CX3CR1+ cells in the glomeruli were macrophages, especially ED3+ cells. Candesartan treatment ameliorated the glomerular morphological findings at six weeks after disease induction. Although the treatment did not ameliorate the morphological finding at two weeks, decreased expression of fractalkine and CX3CR1+ were already detected at two weeks in rats treated with candesartan. CONCLUSIONS: Fractalkine expression and the recruitment of CX3CR1+ cells in glomeruli might play an important role in the development of the prolonged disease. These expressions could be predictors of the prolonged disease of the mesangial proliferative glomerulonephritis.
