Predicting Outcome in Patients with Anti-GBM Glomerulonephritis

Daalen, Emma E. van; Jennette, J. Charles; McAdoo, Stephen P.; Pusey, Charles D.; Alba, Marco A.; Poulton, Caroline J.; Wolterbeek, Ron; Nguyen, Tri Q.; Goldschmeding, Roel; Alchi, Bassam; Griffiths, Meryl; Zoysa, Janak R de; Vincent, B.; Bruijn, Jan A.; Bajema, Ingeborg M.

doi:10.2215/cjn.04290417

Cited by 82 publications

(83 citation statements)

References 18 publications

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“…Patients with pulmonary involvement were more likely to receive a standard regimen: 61 (82%) patients versus 76 (60%) in patients with isolated glomerulonephritis (p < 0.001) ( Figure 2). [7][8][9][10][11][12][13][14][15] PLEX sessions. The proportion of patients receiving PLEX and the number of sessions was significantly higher in patients with pulmonary involvement.…”

Section: Therapeutic Managementmentioning

confidence: 99%

“…A combination of plasma exchange (PLEX), corticosteroids (CST), and cyclophosphamide (CYC) has improved survival, particularly when the lungs are involved [9][10][11], and some observational studies have suggested the efficacy of rituximab (RTX) as an adjunct to CYC or alone [12,13]. However, kidney outcome is still very poor, with a one-year dialysis-free survival rate ranging from 16 to 37% despite intensive therapy [14][15][16]. For older patients in whom isolated glomerulonephritis is most common, the benefit of intensive treatment remains controversial in the case of anuria or dialysis dependence, particularly regarding a hypothetical increased infectious risk.…”

Section: Introductionmentioning

confidence: 99%

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Severe Infection in Anti-Glomerular Basement Membrane Disease: A Retrospective Multicenter French Study

Caillard

Vigneau

Hazzan

et al. 2020

JCM

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In patients presenting with anti-glomerular basement membrane (GBM) disease with advanced isolated kidney involvement, the benefit of intensive therapy remains controversial due to adverse events, particularly infection. We aim to describe the burden of severe infections (SI) (requiring hospitalization or intravenous antibiotics) and identify predictive factors of SI in a large cohort of patients with anti-GBM disease. Among the 201 patients (median [IQR] age, 53 [30–71] years) included, 74 had pulmonary involvement and 127 isolated glomerulonephritis. A total of 161 SI occurred in 116 patients during the first year after diagnosis. These infections occurred during the early stage of care (median [IQR] time, 13 [8–19] days after diagnosis) with mainly pulmonary (45%), catheter-associated bacteremia (22%) and urinary tract (21%) infections. In multivariable analysis, positive ANCA (HR [95% CI] 1.62 [1.07−2.44]; p = 0.02) and age at diagnosis (HR [95% CI] 1.10 [1.00–1.21]; p = 0.047) remained independently associated with SI. Age-adjusted severe infection during the first three months was associated with an increased three-year mortality rate (HR [95% CI] 3.13 [1.24–7.88]; p = 0.01). Thus, SI is a common early complication in anti-GBM disease, particularly in the elderly and those with positive anti-neutrophil cytoplasmic antibodies (ANCA). No significant association was observed between immunosuppressive strategy and occurrence of SI.

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Section: Therapeutic Managementmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Severe Infection in Anti-Glomerular Basement Membrane Disease: A Retrospective Multicenter French Study

Caillard

Vigneau

Hazzan

et al. 2020

JCM

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“…Other subsequent studies with larger cohorts of patients confirmed these findings (Cui et al , ; van Daalen et al , ), supporting use of TPE for anti‐GBM disease with renal impairment not requiring dialysis at presentation and in all cases of DAH.…”

Section: Tpe In Non‐haematological Autoimmune Disordersmentioning

confidence: 64%

The role of plasma exchange in the management of autoimmune disorders

Zanatta

Cozzi²,

Marson

et al. 2019

Br J Haematol

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Summary Therapeutic plasma exchange (TPE) has been mainly used in the treatment of autoimmune diseases. The main mechanisms of action of TPE include the removal of circulating autoantibodies, immune complexes, complement components, cytokines and adhesion molecules, along with sensitization of antibody‐producing cells to immunosuppressant agents. TPE is useful in autoimmune haematological, renal, rheumatic and neurological diseases, and is recommended for acute disorders, together with relapsed or worsened chronic diseases that are often unresponsive to conventional treatments. The American Society for Apheresis and the British Society of Haematology have published guidelines on the clinical use of apheresis procedures, indicating the different levels of efficacy of TPE. Based on the evidence from current literature and our personal experience, this review discusses the indications and the suggested regimens for TPE in autoimmune haematological and non‐haematological disorders.

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“…Fourthly, kidney biopsy, and laboratory data were lacking in the database. Previous studies have suggested that no patient with 100% glomerular crescents and dialysis dependence at presentation recovered kidney function, and so current guidelines do not recommend treatment in these cases [27,28]. Furthermore, studies have also demonstrated that those patients with higher serum creatinine (5.7 mg/dL or higher) and reduced proportion of normal glomeruli on kidney biopsy have poor renal outcomes [2,13].…”

Section: Discussionmentioning

confidence: 99%

Inpatient Burden and Mortality of Goodpasture’s Syndrome in the United States: Nationwide Inpatient Sample 2003–2014

Kaewput

Boonpheng

Ungprasert

et al. 2020

JCM

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Background: Goodpasture’s syndrome is a rare, life-threatening, small vessel vasculitis. Given its rarity, data on its inpatient burden and resource utilization are lacking. We conducted this study aiming to assess inpatient prevalence, mortality, and resource utilization of Goodpasture’s syndrome in the United States. Methods: The 2003–2014 National Inpatient Sample was used to identify patients with a principal diagnosis of Goodpasture’s syndrome. The inpatient prevalence, clinical characteristics, in-hospital treatment, end-organ failure, mortality, length of hospital stay, and hospitalization cost were studied. Multivariable logistic regression was performed to identify independent factors associated with in-hospital mortality. Results: A total of 964 patients were admitted in hospital with Goodpasture’s syndrome as the principal diagnosis, accounting for an overall inpatient prevalence of Goodpasture’s syndrome among hospitalized patients in the United States of 10.3 cases per 1,000,000 admissions. The mean age of patients was 54 ± 21 years, and 47% were female; 52% required renal replacement therapy, whereas 39% received plasmapheresis during hospitalization. Furthermore, 78% had end-organ failure, with renal failure and respiratory failure being the two most common end-organ failures. The in-hospital mortality rate was 7.7 per 100 admissions. The factors associated with increased in-hospital mortality were age older than 70 years, sepsis, the development of respiratory failure, circulatory failure, renal failure, and liver failure, whereas the factors associated with decreased in-hospital mortality were more recent year of hospitalization and the use of therapeutic plasmapheresis. The median length of hospital stay was 10 days. The median hospitalization cost was $75,831. Conclusion: The inpatient prevalence of Goodpasture’s syndrome in the United States is 10.3 cases per 1,000,000 admissions. Hospitalization of patients with Goodpasture’s syndrome was associated with high hospital inpatient utilization and costs.

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Predicting Outcome in Patients with Anti-GBM Glomerulonephritis

Abstract: This article contains a podcast at https://www.asn-online.org/media/podcast/CJASN/2017_11_21_CJASNPodcast_18_1_v.mp3.

Cited by 82 publications

References 18 publications

Severe Infection in Anti-Glomerular Basement Membrane Disease: A Retrospective Multicenter French Study

Severe Infection in Anti-Glomerular Basement Membrane Disease: A Retrospective Multicenter French Study

The role of plasma exchange in the management of autoimmune disorders

Inpatient Burden and Mortality of Goodpasture’s Syndrome in the United States: Nationwide Inpatient Sample 2003–2014

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