1/50. 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 = glomerulonephritis (Clic here for more details about this article) |
2/50. 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 = 2.5keywords = glomerulonephritis (Clic here for more details about this article) |
3/50. 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 = 2.5keywords = glomerulonephritis (Clic here for more details about this article) |
4/50. 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 = 5keywords = glomerulonephritis (Clic here for more details about this article) |
5/50. 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 = 3keywords = glomerulonephritis (Clic here for more details about this article) |
6/50. 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 = 3.5keywords = glomerulonephritis (Clic here for more details about this article) |
7/50. role of lipids in the progression of renal disease in systemic lupus erythematosus patients.Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease marked by immune-complex mediated lesions in small blood vessels of various organs, especially the kidneys, although other factors may also be implicated in the pathogenesis of the disease. This article focuses on the role of lipids in the progression of glomerular, vascular and tubulo-interstitial lesions in two patients with lupus nephritis associated with pronounced hyper- and dyslipidemia. The pathogenesis of progressive glomerulosclerosis in both patients appears to be multifactorial. In addition to immune complex mediated lupus glomerulonephritis, progressively active in the first patient, severe nephrotic-range persistent proteinuria, arterial hypertension associated with hyperfiltration and hyperperfusion injuries and, to a minor extent, hyper- and dyslipidemia were observed. Immunological and non-immunological factors were shown to contribute to the development of tubulo-interstitial lesions. In both patients, in addition to local immune deposits, prominent tubulo-interstitial lipid deposits were probably causally related to both hyperlipidemia and the increased permeability of the glomerular filtration barrier. Tubular lesions were highlighted by intracytoplasmic lipid droplets as well as small cleft-like spaces found to be impacted in the tubular lumina. They were seen to penetrate tubular epithelial cells and eventually lodge in the interstitium, surrounded by mononuclear cell infiltrates and foam cells. In both patients, hypertensive angiopathy and extraglomerular vascular immune deposits were demonstrated. In addition, in the second patient, arteriolar and small arterial hyaline was found at the age of 28 years to be full of lipids and calcium precipitates, suggesting a peripheral atherosclerosis-like process which never occurs as a natural age-related condition. In conclusion, all parts of the nephron may be involved in the pathogenetic process causally related or influenced by hyper- or dyslipidemia. Associated either with endothelial cell injury and consequent insudation of lipids in the vascular walls, glomerular filtration barrier injury with hyperfiltration, or tubulo-interstitial lipid deposition, the mechanism of tissue damage by lipids in all parts of the nephron shares similarities with the pathogenesis of systemic atherosclerosis.- - - - - - - - - - ranking = 0.5keywords = glomerulonephritis (Clic here for more details about this article) |
8/50. Rapidly progressive glomerulonephritis with D-penicillamine.We present 3 cases of anti-myeloperoxidase, anti-neutrophil cytoplasmic antibody (MPO-ANCA)-positive rapidly progressive glomerulonephritis developed during the treatment with D-penicillamine (D-PC) for rheumatoid arthritis. Rheumatoid arthritis was diagnosed in these patients, and D-PC was administered to them at doses of 100, 200, and 300 mg per day for 32, 42, and 39 months, respectively. They developed proteinuria, hematuria, renal insufficiency, and anemia, and D-PC was stopped. On admission, MPO-ANCA was strongly positive in their sera. Renal biopsy showed glomerulonephritis with cellular crescents. Immunofluorescence examination revealed deposits of granular IgG, IgM, IgA, C1q, and C3 in the mesangium. The 3 patients were treated with steroid pulse therapy along with administration of anticoagulants, and cyclophosphamide was also used in 2 patients. Their renal function improved gradually and MPO-ANCA disappeared after immunosuppressive treatment.- - - - - - - - - - ranking = 3keywords = glomerulonephritis (Clic here for more details about this article) |
9/50. retroperitoneal fibrosis and immune-complex glomerulonephritis.A 63-year-old man with rapidly progressive glomerulonephritis of the immune-complex type showed typical findings of idiopathic retroperitoneal fibrosis involving the left ureter resulting in hydronephrosis. Treatment with steroid improved both conditions. Our case and previously reported cases showing the same association of conditions support the hypothesis that the association is not fortuitous but reflects a common immunological mechanism.- - - - - - - - - - ranking = 2.5keywords = glomerulonephritis (Clic here for more details about this article) |
10/50. Progressive renal failure and blindness due to retinal hemorrhage after interferon therapy for hepatitis c virus-associated membranoproliferative glomerulonephritis.We treated a 67-year-old Japanese woman with membranoproliferative glomerulonephritis (MPGN) and chronic active hepatitis associated with hepatitis C virus (HCV) infection. Treatment commenced with a daily dose of 6 MU IFN alpha-2b for 2 weeks, which was changed to three times weekly thereafter. After 2 weeks, HCV rna in the serum was undetectable and there was a concomitant reduction in proteinuria. Treatment with IFN alpha-2b was discontinued because of severe headache and fever. Five weeks after the discontinuation of IFN alpha-2b, the patient experienced the sudden onset of visual loss due to retinal hemorrhage. Subsequently, proteinuria and renal function progressively deteriorated though HCV rna was undetectable. This case exemplifies the need for careful monitoring of renal function and retinal lesions not only in patients receiving IFN but also in those following the discontinuation of IFN treatment.- - - - - - - - - - ranking = 3.1701597215501keywords = glomerulonephritis, membranoproliferative, membranoproliferative glomerulonephritis (Clic here for more details about this article) |
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