Long-term follow-up of aggressively treated idiopathic rapidly progressive glomerulonephritis

Bruns, Frank; Adler, Sheldon; Fraley, Donald S.; Segel, David P.

doi:10.1016/0002-9343(89)90336-7

Cited by 34 publications

(21 citation statements)

References 18 publications

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“…Between 3 and 12 mo, death was exclusively caused by therapy. Considering earlier reports that the addition of plasma exchange and intravenous methylprednisolone facilitates the improvement of renal function within the first 3 mo after disease onset, [22][23][24] it is most striking that death as a result of therapy almost exclusively occurs in the period after 3 mo. This phenomenon may be ascribed to the cumulative dosage of immunosuppressive treatment, when combining standard treatment with adjunctive treatment, according to the regimen used in this study.…”

Section: Discussionmentioning

confidence: 97%

Chances of Renal Recovery for Dialysis-Dependent ANCA-Associated Glomerulonephritis

Wijngaarden¹,

Hauer²,

Wolterbeek³

et al. 2007

Journal of the American Society of Nephrology

112

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In patients who have anti-neutrophil cytoplasm autoantibody (ANCA)-associated glomerulonephritis and are on dialysis at time of diagnosis, renal function is sometimes insufficiently restored by immunosuppressive treatment, which often coincides with potentially lethal adverse effects. This study investigated the clinical and histologic variables that determine the chances of dialysis independence, dialysis dependence, or death after 12 mo in these patients. Sixty-nine patients who had ANCA-associated glomerulonephritis and were dialysis dependent at diagnosis received uniform, standard immunosuppressive therapy plus either intravenous methylprednisolone or plasma exchange. Eleven clinical and histologic variables were assessed. Univariate and binary logistic regression analyses were performed. Predictive parameters were entered into a two-step binary logistic regression analysis to differentiate among the outcomes of dialysis independence, dialysis dependence, or death. The point at which the chance of therapy-related death exceeded the chance of dialysis independence was determined. The chance of recovery exceeded the chance of dying in most cases. Intravenous methylprednisolone as adjunctive therapy plus Ͻ18% normal glomeruli and severe tubular atrophy increased the chance of therapy-related death over the chance of dialysis independence. Plasma exchange treatment plus severe tubular atrophy and Ͻ2% normal glomeruli increased the chance of therapy-related death over that of dialysis independence. Even with ominous histologic findings, the chance of renal recovery exceeds the chance of therapy-related death when these patients are treated with plasma exchange as adjunctive therapy.

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Section: Discussionmentioning

confidence: 97%

Chances of Renal Recovery for Dialysis-Dependent ANCA-Associated Glomerulonephritis

Wijngaarden¹,

Hauer²,

Wolterbeek³

et al. 2007

Journal of the American Society of Nephrology

112

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“…Crescentic GN usually manifests with an acute ne phritic syndrome [1][2][3][4], In a small number of adult patients, however, an insidious onset with nephrotic syn drome [ 10,17], and rarely with nonspecific symptoms [9], with a gradual deterioration of renal function has been observed. A similar evolution of the disease was found in 28% of our cases.…”

Section: Discussionmentioning

confidence: 99%

“…In earlier reports, oral corticosteroids and cytotoxic drugs, often with anti coagulants and antiplatelet agents, were used with some benefit [6,7,20]. More recently, better results have been reported with pulse méthylprednisolone therapy [9,22] and plasmapheresis [8,9], The therapeutic experience in crescentic GN in children was recently reviewed with the inference that specific treatment favorably affected the course of the disorder [12]. We cannot draw any conclu sions from our observations since in most cases, the treat ment was started late when irreversible glomerular dam age could have been present and a standard regimen was not employed.…”

Section: Discussionmentioning

confidence: 99%

“…A wide variety of disorders may be associated with such a histology [1], The prognosis is generally poor [ 1,3,4,7,12], although the possibility of recovery is considered to be higher in poststreptococcal and postinfectious cases [2,5,7,13]. The role of immuno suppressive and other specific therapy is not clearly estab lished, and the long-term outcome in patients who re cover is uncertain [8,9].…”

Section: Introductionmentioning

confidence: 99%

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Crescentic Glomerulonephritis in Children: A Review of 43 Cases

et al. 1992

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Forty-three children with crescentic glomerulonephritis (GN), having large crescents in more than 50% of the glomeruli, were observed during a period of 22 years. There were 17 boys and 26 girls between the ages of 3.5 and 14 years (mean 8.7 ± 2.6). Thirty-one patients (72%) presented with acute nephritic features and increasing renal insufficiency (rapidly progressive GN) whereas 12 had an insidious onset with nephrotic syndrome, or rarely with nonspecific symptoms. Eleven patients had evidence of poststreptococcal GN and 6 an underlying systemic disorder. Renal biopsy showed large crescents in > 80% of the glomeruli in 38 cases (100% in 28) which were predominantly fibrocel-lular or fibrous in 80% of the patients. Nineteen patients (44%) were treated with prednisolone, cyclophosphamide and dipyridamole; in addition, 8 were also given anticoagulants. Six patients received pulse doses of corticosteroids. In 23 patients, there was inexorable progression of renal failure, 14 showed partial improvement but subsequently had varying degrees of renal insufficiency and in 6, there was recovery of renal function with normal levels of serum creatinine. Of the latter, 4 had received immunosuppressive anticoagulant therapy and 2 only supportive care. Of 11 patients with poststreptococcal crescentic GN, 7 progressed to end-stage renal disease and 2 developed chronic renal insufficiency. Our findings confirm the poor outcome of crescentic GN in children, irrespective of the underlying etiology. In a small proportion of cases, the disorder may have an insidious onset and a slowly progressive course, but an equally grave prognosis.

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“…Laboratory diagnosis in these patients may have high plasma creatinine, marked Glomerular filtration, and proteinuria [2]. Some patients may not have hematuria the reason of which is not known [3]. Choosing medications for renal insufficiency patients vary individually with underlying causes, renal function and response to the drugs and the results were inconsistent.…”

Section: Introductionmentioning

confidence: 99%

Therapeutic Response of Natural Adrenocorotropin in Treatment of Proteinuria due to ANCA Negative Pauci-Immune Crescentic Glomerulonephritis

Swan¹,

Kyaw²,

Aye³

2018

UNOAJ

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Long-term follow-up of aggressively treated idiopathic rapidly progressive glomerulonephritis

Cited by 34 publications

References 18 publications

Chances of Renal Recovery for Dialysis-Dependent ANCA-Associated Glomerulonephritis

Chances of Renal Recovery for Dialysis-Dependent ANCA-Associated Glomerulonephritis

Crescentic Glomerulonephritis in Children: A Review of 43 Cases

Therapeutic Response of Natural Adrenocorotropin in Treatment of Proteinuria due to ANCA Negative Pauci-Immune Crescentic Glomerulonephritis

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