1/27. Crossroads of the effects of cyclophosphamide pulse therapy for lupus nephritis--experience of 11 cases.In this study time for initial assessment of monthly intravenous cyclophosphamide (CP) pulse therapy is discussed for a better outcome with less complications. Eleven patients with lupus nephritis (LN) resistant to conventional therapy (serum creatinine level < or = 2.7 mg/dl) were given 500 mg/m2 of CP 7-9 times with an interval of one month. Urinary protein (Up) decreased in all patients after 3 courses of CP pulse therapy and kept similar levels thereafter. In one group of patients (n = 7), Up decreased to < 2 g/day after 3 courses, while in the other group (n = 4), it did not decrease to < 4 g/day. creatinine clearance increased by 0-100% in the former group, while it decreased by 5-20% in the latter group after 6-9 courses. Renal function of the patients with insufficient response after 3 courses tended to show no further improvement or worsened thereafter, although Up decreased during CP pulse therapy. A relatively small dose of CP (500 mg/m2) pulse therapy was useful in most LN patients regardless of the renal histology and it was thought important to assess its effects after 3 courses for a prediction of the clinical course. Modification of the protocol at that time might be necessary in regard to dose or interval of CP administration especially for patients with insufficient outcome.- - - - - - - - - - ranking = 1keywords = lupus (Clic here for more details about this article) |
2/27. Non-lupus nephropathy associated with antiphospholipid antibodies.Renal biopsy was performed in a 12-year-old girl with hematuria and proteinuria which was first detected at the age of 7, and the findings were the mesangial proliferative glomerulonephritis with IgG and C3 deposits. The routine blood examination for the biopsy disclosed the presence of the prolonged activated partial thromboplastin time and the biological false positive reaction in the syphilis test. These results led us to the further investigation, which revealed the presence of high titers of anticardiolipin antibodies. Since this girl presented no extra-renal symptoms of systemic lupus erythematosus (SLE) and had negative serologic tests for SLE, we hypothesize that her nephritis is closely related to antiphospholipid antibodies.- - - - - - - - - - ranking = 3.6367831871786keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
3/27. Hypercalcaemia in systemic lupus erythematosus.Hypercalcaemia is a common electrolyte abnormality. The vast majority of patients will be shown to have either hyperparathyroidism or malignancy. In less than 10% of patients other, less common causes of hypercalcaemia will be present. Systemic lupus erythematosus is a very rare cause of hypercalcaemia. It may be associated with lymphadenopathy and pleuritis to constitute a distinct clinical entity described as 'hypercalcaemia-lymphoedema syndrome'. In these cases the pathophysiology of the hypercalcaemia is not completely understood. In some cases it is associated with elevated levels of parathyroid-related peptide (PTHrP). In others the level of PTHrP is normal, and it has been suggested that autoantibodies may cause hypercalcaemia by activating the PTH receptor. We describe a case of a woman who presented with severe hypercalcaemia, developed the hypercalcaemia-lymphodema syndrome and fulfilled the diagnostic criteria of systemic lupus erythematosus.- - - - - - - - - - ranking = 16.441028598509keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
4/27. Delayed onset of systemic lupus erythematosus in a child with endothelial tubuloreticular inclusion.We report here on an 11-year-old Japanese girl who was found to have proteinuria by routine mass screening urinalysis for school children, and who developed systemic lupus erythematosus (SLE) 21 months later. The initial renal biopsy, performed 3 months after the first visit to tokyo Medical University Kasumigaura Hospital (TMUKH), revealed membranous glomerulonephritis. In an immunofluorescent study, IgG was the only positive immunoglobulin found. A "full-house" immunofluorescence glomerulopathy, well known as a predictive finding for lupus nephritis, was not detected. Endothelial tubuloreticular inclusions (ETI) were found by electron microscopy. Because the diagnosis of SLE was not established clinically and serologically, the patient was followed every 3 months without drugs. Her urinary findings returned to normal within 18 months. Three months after the last visit, she was sent to Tsukuba University Hospital (TUH) for fever, arthralgia, dyspnea and butterfly rash. She was diagnosed as having SLE, pleuritis, and pericarditis. Although she was treated with methylpredonisolone and oral prednisolone, she developed cardiac tamponade on the 12th day of admission during the course of pneumococcal septicemia. Finally, she was treated successfully with surgical procedures, antibiotics and oral prednisolone and was discharged. We conclude that ETI is a more significant early sign of SLE than "full-house" immmunofluorescence glomerulopathy, especially in pediatric cases.- - - - - - - - - - ranking = 14.383915935893keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
5/27. Lipoprotein glomerulopathy associated with psoriasis vulgaris: report of 2 cases with apolipoprotein e3/3.Lipoprotein glomerulopathy (LPG) is a rare disease, characterized by a special histology, including dilated glomerular capillaries filled with pale-stained and meshlike lipoprotein thrombi. It always presents with proteinuria or nephrotic syndrome. Although hyperlipidemia is not always seen, most patients have type III hyperlipoproteinemia with apolipoprotein (apo) E2/3 phenotyping. Although the clinical feature of LPG is rarely described, LPG associated with other glomerulopathy, including IgA nephropathy, membranous nephropathy, and lupus nephritis, has been documented. Until now, there have been no reports of psoriasis vulgaris associated with LPG. The authors present 2 cases of LPG with apo E3/3 genotyping associated with psoriasis vulgaris. The first patient was a 65-year-old woman who presented with nephrotic syndrome with daily urinary protein loss of 9.05 g and itchy erythematous scaly plaques on her trunk and lower limbs for 1 year. The renal biopsy results showed LPG, and the skin biopsy results showed psoriasis. The second patient was a 50-year-old man with history of psoriasis over his trunk and 4 limbs for 30 years. He also presented with nephrotic syndrome with daily urinary protein loss of 7.55 g. The renal biopsy results also showed LPG. The genotype of apo E showed E3/3, and lipoprotein electrophoresis showed a type III hyperlipoproteinemia-like pattern in both cases. The authors suggest that presence of apo E3/3 genotype cannot rule out the diagnosis of type III hyperlipoproteinemia and LPG. Besides, LPG should be included in the differential diagnosis of psoriatic patients with nephrotic syndrome, especially in Asian patients who show poor response to traditional therapy. Renal biopsy should be performed to make the definitive diagnosis.- - - - - - - - - - ranking = 0.2keywords = lupus (Clic here for more details about this article) |
6/27. Remission of refractory lupus nephritis with a protocol including rituximab.immunosuppression with corticosteroids and cyclophosphamide is the standard of care for lupus nephritis. We report a 19-year old woman with lupus nephritis and nephrotic syndrome who had not achieved complete remission after treatment with 15.7 g cyclophosphamide and 13.7 g prednisone. We planned a consolidation phase with: 1) cyclophosphamide 20 mg/kg i.v. every 28 days for three cycles; 2) anti-CD20 chimeric monoclonal antibody (rituximab) 375 mg/m2 i.v. weekly for four weeks; and 3) slow tapering of prednisone p.o., q.o.d., after a reinduction dose during rituximab administration. At the end of this phase the patient achieved complete remission. An indefinite maintenance treatment with methotrexate, cyclosporin and low-dose prednisone was then started. Twenty-four months later the patient remains in remission. In the immunosuppressive treatment of lupus nephritis the insertion of a consolidation phase with rituximab combined with cyclophosphamide achieves a therapeutically important and lasting deletion of the lymphocyte clone responsible for autoimmunity.- - - - - - - - - - ranking = 1.4keywords = lupus (Clic here for more details about this article) |
7/27. Prolonged membranous lupus nephritis with change of anti-ssDNA antibody titer and repeated renal relapse.We report a case of a 44-year-old woman with nephrotic syndrome who underwent renal biopsy three times. On each occasion, light microscopy showed membranous nephropathy with mild to moderate thickening of the glomerular capillary walls. Immunofluorescence microscopy showed predominant deposition of immunoglobulin (Ig) G, IgG1, IgG2, IgG3, and IgG4; C3; and C1q along the glomerular capillary walls and deposition of IgM and IgA in some parts of the walls. Electron microscopy revealed the accumulation of electron-dense deposits in the mesangium and the subepithelial area of the glomerular basement membrane. Virus-like particles were detected in the subendothelial cells in all three biopsy specimens. A definitive diagnosis of systemic lupus erythematosus (SLE) was made at the time of the second admission, when she was 31 years old. A diagnosis of membranous lupus nephritis was then made on the basis of the pathological and clinical findings. A change in anti-single-stranded (ss)dna antibody titers was of particular interest in this patient. Occasional small increases in anti-double-stranded (ds)dna antibody were found, but increased anti-ssDNA antibody titers occurred before there was any elevation of urinary protein during renal relapse, and a sustained increase in the titers was shown subsequently. Hypocomplementemia occurred in parallel with the increase of anti-ssDNA antibody. Immunosuppressive therapy with steroid promptly eliminated anti-dsDNA antibody, but anti-ssDNA antibody remained positive. The patient had normocomplementemia and proteinuria was absent. Later, anti-ssDNA antibody decreased. Renal function has remained in the normal range for 20 years.- - - - - - - - - - ranking = 3.8367831871786keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
8/27. Immunotactoid glomerulopathy with microtubular deposits, with reference to the characteristics of Japanese cases.We present the case of a 69-year-old man with nephrotic syndrome and renal insufficiency, who developed lobular glomerulonephritis. An electron microscopy examination of a renal biopsy showed microtubular structures of 24 nm in diameter in the subendothelial space and the paramesangial area. These deposits were PAS-positive and congo red-negative, and revealed predominantly positive staining for kappa light chain. There was no evidence of diseases with highly organized glomerular deposits, such as amyloidosis, cryoglobulinemia, systemic lupus erythematosus or paraproteinemia. Therefore, the patient was diagnosed to have immunotactoid glomerulopathy (ITG). During a seven-year course he has not developed any disease known to be associated with organized glomerular immune deposits. Hence, we believe ITG occurred as a primary glomerular disease in this case. We also highlight cases of ITG with microtubular deposits that have been reported in japan, compare these cases to previous reports, and show that the characteristics of the Japanese cases are male predominance; a high incidence of membranoproliferative glomerulonephritis (MPGN); a low incidence of monoclonal gammopathy and hematological malignancies and a higher incidence of hypocomplementemia.- - - - - - - - - - ranking = 2.8367831871786keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
9/27. naproxen-induced nephropathy in systemic lupus erythematosus.A 34-year-old female with an 8-month history of systemic lupus erythematosus and intermittent naproxen use presented with acute oliguric renal failure, hypoalbuminemia, 4 proteinuria, and an active urinary sediment. The clinical picture suggested a rapidly progressive lupus glomerulonephritis. Renal biopsy, however, demonstrated chronic, active interstitial nephritis without evidence of immune deposits by immunofluorescence or electron microscopy. Nonsclerotic glomeruli revealed diffuse foot process fusion without cellular proliferation. These findings were consistent with nonsteroidal anti-inflammatory drug induced nephropathy. Discontinuation of naproxen and institution of corticosteroid therapy was followed by improvement in renal function and remission of nephrotic syndrome. This case represents the first report of nonsteroidal antiinflammatory drug nephropathy associated with systemic lupus erythematosus.- - - - - - - - - - ranking = 17.220699123072keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
10/27. Antiproteinuric effect of captopril in a patient with lupus nephritis and intractable nephrotic syndrome.A 22 year old woman presented with lupus nephritis, hypertension, and intractable nephrotic syndrome. Albumin and furosemide given intravenously was ineffective. captopril administered in a daily dose of 62.5 mg was associated with a reduction in proteinuria from 28 g/24 hours to 11.5 g/24 hours over 10 weeks, resulting in a weight reduction of 16 kg. This was achieved with relative preservation of renal function. captopril should be considered in the treatment of intractable proteinuria in patients with lupus nephritis, or when cytotoxic drugs are refused, because of its efficacy and relative safety. captopril should, however, be used as an adjunct and not as a substitute for standard treatment.- - - - - - - - - - ranking = 1.2keywords = lupus (Clic here for more details about this article) |
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