1/68. Successful treatment of rapidly progressive lupus nephritis associated with anti-MPO antibodies by intravenous immunoglobulins.We report a case of systemic lupus erythematosus (SLE) associated with crescentic glomerulonephritis and myeloperoxidase-specific anti-neutrophil cytoplasmic antibodies (MPO-ANCA). A 34-year-old Japanese female patient diagnosed with SLE developed rapidly progressive renal failure and nephrotic syndrome. Haemodialysis was required to restore renal function. methylprednisolone pulse therapy followed by plasmapheresis did not suppress the progression of renal failure, so she was treated with high-dose intravenous immunoglobulin (IV-IG) therapy, which was well tolerated and effectively prevented renal failure. A renal biopsy showed diffuse proliferative lupus nephritis (WHO classification IVc) with predominant crescent formation and scant subendothelial immune deposits. These findings indicate that, in addition to lupus nephritis, which usually results from the deposition of circulating or locally formed immune complexes, MPO-ANCA may be involved in the pathogenesis of crescentic glomerulonephritis. Furthermore, we propose that IV-IG is an effective therapy for MPO-ANCA-related renal crisis in lupus nephritis.- - - - - - - - - - ranking = 1keywords = nephritis (Clic here for more details about this article) |
2/68. Familial occurrence of systemic vasculitis and rapidly progressive glomerulonephritis.Two familial clusters of systemic vasculitis are described. In one family, microscopic polyangiitis and rapidly progressive glomerulonephritis occurred in HLA-identical siblings; in the second family, 3 second- and fourth-degree related members were affected by Wegener's granulomatosis. Published clusters of systemic vasculitides and Goodpasture's syndrome are reviewed, and, together with the observed families, the evidence for genetic susceptibility and a causative role of environmental factors for these diseases with special emphasis on the HLA system is discussed.- - - - - - - - - - ranking = 0.55555555555556keywords = nephritis (Clic here for more details about this article) |
3/68. Effect of open label pulse cyclophosphamide therapy on MRI measures of disease activity in five patients with refractory relapsing-remitting multiple sclerosis.OBJECTIVE: To evaluate the response to cyclophosphamide (CTX) of five patients who failed an average three treatments with multiple other therapeutic agents, using serial monthly MRI measures. methods: Five patients with relapsing-remitting multiple sclerosis (MS) and documented MRI disease activity were started on monthly pulse intravenous CTX at a dose of 1 g/m2. CTX was administered without an induction phase according to the protocol similar to the treatment of lupus nephritis. The five patients were followed with monthly MRI and clinical evaluation for a mean of 28 months. RESULTS: All the patients showed a rapid reduction in the contrast-enhancing lesion frequency and in three patients there was a decrease in the T2 lesion load within the first 5 months after starting CTX treatment. The administration of CTX during overnight hospitalization was safe and well tolerated. CONCLUSIONS: These findings suggest that aggressive immunosuppressive therapy may be useful in some rapidly deteriorating refractory patients and further controlled study should be considered in order to full evaluate this type of treatment as a potential therapy in MS.- - - - - - - - - - ranking = 0.11111111111111keywords = nephritis (Clic here for more details about this article) |
4/68. Low serum C3, leukopenia, and thrombocytopenia: unusual features of henoch-schonlein purpura.Henoch-Schonlein purpura (HSP) affects predominantly the skin, joints, gastrointestinal tract and kidney. Although the pathogenesis is probably of immune origin and complement activation is thought to play a role, laboratory findings including the serum level of the complement components are usually normal. We present a patient with a severe form of HSP nephritis who had unusual laboratory findings of a low level of C3, mild leukopenia and thrombocytopenia. These findings may further support the importance of complement activation in the pathogenesis of HSP.- - - - - - - - - - ranking = 0.11111111111111keywords = nephritis (Clic here for more details about this article) |
5/68. Rapidly progressive fibrosing interstitial nephritis associated with Chinese herbal drugs.Rapidly progressive fibrosing interstitial nephritis after a slimming regimen containing aristolochic acid has been identified as Chinese herbs nephropathy (CHNP). From 1995 to 1998, we observed 12 Chinese people from different areas of taiwan who underwent renal biopsy for unexplained renal failure. Medical history gave no clue to the causes of impaired renal function except for the ingestion of traditional Chinese herbs. Although these patients ingested herbal drugs from various sources for different purposes, their renal biopsy samples showed amazingly similar histological findings, with extensive hypocellular interstitial fibrosis and atrophy and loss of tubules in all cases. Glomeruli were apparently intact. They also had similar clinical features, such as normal or mildly elevated blood pressure, early and severe anemia, low-grade proteinuria, glycosuria, and insignificant urinary sediments. Renal function deteriorated rapidly in most patients despite discontinuation of the herbal medicines. Seven patients underwent dialysis, and the remainder experienced slowly progressive renal failure. Bladder carcinoma was found in one patient. Morphologically and clinically, the nephropathy in our patients was similar to CHNP, reported in belgium. Because of the complexity and unknown types of herbs used in different clinical situations, unidentified phytotoxins other than aristolochic acid might be responsible for this unique disease entity. We conclude that the relation of this nephropathy to the consumption of Chinese herbs is striking. Using uncontrolled herbal remedies carries a high risk for developing interstitial renal fibrosis and urothelial malignancy.- - - - - - - - - - ranking = 0.55555555555556keywords = nephritis (Clic here for more details about this article) |
6/68. diabetes mellitus associated with rapidly progressive glomerulonephritis with perinuclear antineutrophil cytoplasm antibodies.A 55-year-old woman who had been treated for diabetes mellitus for twenty-five years developed interstitial pneumonia and rapidly progressive glomerulonephritis (RPGN). The findings of light microscopy revealed fibrocellular crescent formation in all glomeruli and infiltration of lymphoid cells in interstitium. There were no deposits in the intracapillary area and mesangial area on both immunofluorescence and electron microscopy. Her interstitial pneumonia improved with pulse therapy of methylprednisolone and her hematuria disappeared with mix treatment of cyclophosphamide and double filtration plasmapheresis (DFPP). Her serum creatinine level improved from 2.2 mg/dl to 1.5 mg/dl. Interstitial pneumonia and hematuria did not recur at twelve months after the first hospitalization. This report presents a rare case with RPGN associated with diabetes mellitus who recovered with combination therapy of cyclophosphamide, steroid and DFPP.- - - - - - - - - - ranking = 0.55555555555556keywords = nephritis (Clic here for more details about this article) |
7/68. Emphysematous pyelonephritis- conversion of type i to type II appearance on serial CT studies.Recently, emphysematous pyelonephritis (EPN) has been classified into two subtypes based on CT findings. We recently experienced a patient whose CT image changed from type I (extensive destruction of the renal parenchyma with a large amount of air density without any fluid collection) to type II (containing a large amount of fluid) during the course of conservative treatment. We believe that some patients with type I EPN can change to type II EPN.- - - - - - - - - - ranking = 0.55555555555556keywords = nephritis (Clic here for more details about this article) |
8/68. C1q nephropathy presenting as rapidly progressive crescentic glomerulonephritis.C1q nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial immunoglobulins and complement deposits, most notably C1q, and the absence of clinical and laboratory evidence of systemic lupus erythematosus. histology in C1q nephropathy is characterized by a slight to severe increase in mesangial cellularity and matrix, with or without segmental sclerosis. C1q nephropathy usually presents with nephrotic-range proteinuria in older children and young adults, and has a poor response to steroids. patients may have decreased creatinine clearance at presentation, but progression to end-stage renal disease (ESRD) is slow. Severe crescentic glomerulonephritis has not been reported in C1q nephropathy. We describe a 3-year-old Hispanic girl who presented with renal insufficiency. kidney biopsy showed C1q nephropathy with severe crescentic glomerulonephritis. The clinical and serological evaluation ruled out systemic lupus erythematosus or other immunological or infectious etiologies. In spite of immunosuppressive therapy, she progressed to ESRD within 14 weeks and is currently on chronic peritoneal dialysis. The atypical features of C1q nephropathy observed in our patient, which have not been described in earlier reports, are an early age of onset, severe crescentic glomerulonephritis, and rapid progression to ESRD. C1q nephropathy should be added to the differential diagnosis of glomerulonephritis in young children and in the patient with crescentic glomerulonephritis.- - - - - - - - - - ranking = 1.1111111111111keywords = nephritis (Clic here for more details about this article) |
9/68. Apparent progression of acute glomerulonephritis to dense deposit disease.One week after the diagnosis of meningococcal meningitis, an 8-year-old boy presented with acute renal failure and hypocomplementemia. A renal biopsy showed "postinfectious glomerulonephritis" and acute tubular necrosis. hematuria, proteinuria, and low complement levels persisted, and 2 years later a follow-up renal biopsy revealed dense deposit disease. The apparent progression of postinfectious glomerulonephritis to dense deposit disease as observed in this patient has not been previously described.- - - - - - - - - - ranking = 0.66666666666667keywords = nephritis (Clic here for more details about this article) |
10/68. recurrence of rapidly progressive glomerulonephritis probably associated with two different kinds of drugs.We describe a rare case that developed a rapidly progressive glomerulonephritis twice in a 69-year-old man during a course of treatment, first with allopurinol and then with piperacillin. The cessation of each treatment was followed by spontaneous recovery in renal function. A renal biopsy showed crescentic glomerulonephritis with mild tubulointerstitial change and a skin biopsy showed leukocytoclastic vasculitis. This is, to our knowledge, a very rare case of crescentic glomerulonephritis, probably associated with vasculitis during a course of treatment with two different kinds of drugs.- - - - - - - - - - ranking = 0.77777777777778keywords = nephritis (Clic here for more details about this article) |
| | Next -> |