Outcomes of patients with Goodpasture syndrome: A nationwide cohort-based study from the French Society of Hemapheresis

Huart, Antoine; Josse, Anne-Gaëlle; Chauveau, Dominique; Korach, Jean-Michel; Heshmati, Farhad; Bauvin, Eric; Cointault, Olivier; Kamar, Nassim; Ribes, David; Pourrat, Jacques; Faguer, Stanislas

doi:10.1016/j.jaut.2016.05.015

Cited by 53 publications

(62 citation statements)

References 14 publications

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“…The discovery of anti-GBM antibodies and their pathogenicity provide the rationale for treatment with plasma exchange and immunosuppressive agents, particularly cyclophosphamide and corticosteroids (8). This therapy combined with better supportive care and earlier diagnosis has improved the extremely poor outcome of patients with anti-GBM GN over the past decades (9)(10)(11)(12). Despite these improvements, the rapid deterioration caused by the disease still causes ESRD, requiring dialysis at presentation in approximately 55% of patients (9).…”

Section: Introductionmentioning

confidence: 99%

Predicting Outcome in Patients with Anti-GBM Glomerulonephritis

Daalen¹,

Jennette²,

McAdoo

et al. 2017

CJASN

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Section: Introductionmentioning

confidence: 99%

Predicting Outcome in Patients with Anti-GBM Glomerulonephritis

Daalen¹,

Jennette²,

McAdoo

et al. 2017

CJASN

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“…Although the reason remains unclear, we speculated that this is because of the increasing awareness and widespread availability of diagnostic tests around that time [4,13]. Previous studies have suggested that, in addition to genetic factors, environmental factors can also trigger the development of GS, such as cigarette smoking, inhaled hydrocarbons, or potential infectious triggers damaging the alveolar basement membrane and exposing type IV collagen epitopes [3,4,6,[14][15][16][17][18]. Future studies are needed to assess if these factors play an important role in the trends of inpatient prevalence of GS in the United States.…”

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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“…In the series of 122 French patients, three predictive factors of death within the first 12 months were identified: being aged > 60 years, a low number of plasmapheresis sessions and the requirement for an alternative immunosuppressive agent because of refractory or relapsing anti-GBM disease [14]. New data indicate that preexisting chronic interstitial pneumonia is an independent factor of a poor prognosis in anti-GBM disease.…”

Section: Discussionmentioning

confidence: 99%

Diffuse Alveolar Haemorrhage Complicated by Pulmonary Embolism—Problems with Treatment

Fijołek¹,

Wiatr²,

Jakubowska³

et al. 2017

Advances in Respiratory Medicine

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Diffuse alveolar haemorrhage (DAH) refers to a clinical syndrome resulting from injury of the alveolar capillaries, arterioles and venules leading to red blood cel accumulation in the distal air spaces. The conditions associated with DAH and underlying disease determine the prognosis and the treatment regimen. The coexistence of DAH with venous thromboembolism (VTE) is a seroius problem for clinicians and poses a challenge in the therapeutic management. We describe a young patient who developed massive DAH in the course of anti-glomerular basement membrane (anti-GBM) disease (formerly called Goodpasture's syndrome) complicated by pulmonary embolism (PE).

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Outcomes of patients with Goodpasture syndrome: A nationwide cohort-based study from the French Society of Hemapheresis

Cited by 53 publications

References 14 publications

Predicting Outcome in Patients with Anti-GBM Glomerulonephritis

Predicting Outcome in Patients with Anti-GBM Glomerulonephritis

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

Diffuse Alveolar Haemorrhage Complicated by Pulmonary Embolism—Problems with Treatment

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