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J Autoimmun. 2016 Sep;73:24-9. doi: 10.1016/j.jaut.2016.05.015. Epub 2016 Jun 4.

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

Author information

1
CHU de Toulouse, Département de Néphrologie et Transplantation d'Organes, Centre de Référence des maladies rénales rares, Hôpital Rangueil, Université Paul Sabatier - Toulouse III, Toulouse, France.
2
Clinique du Pont de Chaumes, Service de Néphrologie, Montauban, France.
3
CHU de Toulouse, Département de Néphrologie et Transplantation d'Organes, Centre de Référence des maladies rénales rares, Hôpital Rangueil, Université Paul Sabatier - Toulouse III, Toulouse, France; INSERM U1048 (équipe 12), Institut des Maladies Métaboliques et Cardiovasculaires (I2MC), Toulouse, France.
4
Centre Hospitalier Général, Unité de Réanimation, Châlons en Champagne, France.
5
AP-HP, Hôpital Cochin, Unité de médecine transfusionnelle, Paris, France.
6
INSERM U1027, Faculté de Médecine de Purpan, Toulouse, France.
7
CHU de Toulouse, Département de Néphrologie et Transplantation d'Organes, Centre de Référence des maladies rénales rares, Hôpital Rangueil, Université Paul Sabatier - Toulouse III, Toulouse, France; INSERM U1048 (équipe 12), Institut des Maladies Métaboliques et Cardiovasculaires (I2MC), Toulouse, France. Electronic address: faguer.s@chu-toulouse.fr.

Abstract

The overall and renal outcomes of patients with Goodpasture syndrome (GS), a rare autoimmune disorder characterized by circulating anti-GBM antibodies and rapidly progressive glomerulonephritis and/or pulmonary hemorrhage, have mostly been reported in small-sized cohorts or by aggregating patients receiving a variety of therapies that include aggressive (i.e., combined plasma exchanges, corticosteroids, and cyclophosphamide) and less aggressive (i.e., either plasma exchanges or immunosuppressive drugs, or no treatment). To address the prognosis of GS patients with relatively homogeneous management including plasma exchanges, we conducted a multicenter retrospective study on GS patients included in the registry of the French Society of Hemapheresis. 122 patients were included (kidney alone (n = 28), lung alone (n = 5), or combined involvement (n = 89)). All 122 patients received plasma exchanges (median number of sessions: 13 [9-17]), either alone (n = 8) or associated with combined corticosteroids and oral or IV cyclophosphamide (n = 101) or with corticosteroids alone (n = 12) or cyclophosphamide alone (n = 2). One-year survival was 86.9%. 7/16 patients died from severe infection. In multivariate analyses (Cox's regression model), being aged <60 years, and number of plasma exchanges were correlated to overall survival. The use of alternative immunosuppressive drugs (because of refractory or relapsing GS) was correlated to mortality at one year. Superiority of oral cyclophosphamide compared to intravenous intake was close to significant. Using a logistic regression model, renal survival in patients alive at 1 year was only predicted by serum creatinine <500 μmol/L at presentation. This large series describes the predictive factors for overall and renal survival of GS patients treated by plasma exchanges. Interventional studies that compare oral and intravenous cyclophosphamide, as well as testing new immunosuppressive therapies, are warranted.

KEYWORDS:

Anti-GBM antibodies; Cyclophosphamide; Goodpasture syndrome; Outcome; Plasma exchange

PMID:
27267459
DOI:
10.1016/j.jaut.2016.05.015
[Indexed for MEDLINE]

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