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Open Access 01-12-2018 | Research

Rituximab for auto-immune alveolar proteinosis, a real life cohort study

Authors: Berenice Soyez, Raphael Borie, Cedric Menard, Jacques Cadranel, Leonidas Chavez, Vincent Cottin, Emmanuel Gomez, Sylvain Marchand-Adam, Sylvie Leroy, Jean-Marc Naccache, Hilario Nunes, Martine Reynaud-Gaubert, Laurent Savale, Abdellatif Tazi, Lidwine Wemeau-Stervinou, Marie-Pierre Debray, Bruno Crestani

Published in: Respiratory Research | Issue 1/2018

Abstract

Background

Whole lung lavage is the current standard therapy for pulmonary alveolar proteinosis (PAP) that is characterized by the alveolar accumulation of surfactant. Rituximab showed promising results in auto-immune PAP (aPAP) related to anti-GM-CSF antibody.

Methods

We aimed to assess efficacy of rituximab in aPAP in real life and all patients with aPAP in France that received rituximab were retrospectively analyzed.

Results

Thirteen patients were included. No patients showed improvement 6 months after treatment, but, 4 patients (30%) presented a significant decrease of alveolar-arterial difference in oxygen after 1 year. One patient received lung transplantation and one patient was lost of follow-up within one year. Although a spontaneous improvement cannot be excluded in these 4 patients, improvement was more frequent in patients naïve to prior specific therapy and with higher level of anti-GM-CSF antibodies evaluated by ELISA. No serious adverse event was evidenced.

Conclusions

These data do not support rituximab as a second line therapy for patients with refractory aPAP.

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Title: Rituximab for auto-immune alveolar proteinosis, a real life cohort study
Authors: Berenice Soyez
Raphael Borie
Cedric Menard
Jacques Cadranel
Leonidas Chavez
Vincent Cottin
Emmanuel Gomez
Sylvain Marchand-Adam
Sylvie Leroy
Jean-Marc Naccache
Hilario Nunes
Martine Reynaud-Gaubert
Laurent Savale
Abdellatif Tazi
Lidwine Wemeau-Stervinou
Marie-Pierre Debray
Bruno Crestani
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: Respiratory Research / Issue 1/2018
Electronic ISSN: 1465-993X
DOI: https://doi.org/10.1186/s12931-018-0780-5

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