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

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

Others:: Service de Pneumologie A [Paris] ; AP-HP - Hôpital Bichat - Claude Bernard [Paris] ; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP); Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)) ; Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM); LabEx Inflamex; Université Sorbonne Paris Cité (USPC); CHU Pontchaillou [Rennes]; Service de Pneumologie = Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon] ; CHU Tenon [AP-HP] ; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU); Centre Hospitalier Universitaire [Grenoble] (CHU); Department of Pneumology [Lyon] ; Hospices Civils de Lyon (HCL); Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy); Pathologies Respiratoires : Protéolyse et Aérosolthérapie ; Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM); Centre Hospitalier Régional Universitaire de Tours (CHRU Tours); COMUE Université Côte d'Azur (2015-2019) (COMUE UCA); Hôpital Pasteur [Nice] (CHU); Université Pierre et Marie Curie - Paris 6 (UPMC); Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P) ; Université Paris 13 (UP13)-UFR SMBH; Microbes évolution phylogénie et infections (MEPHI) ; Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS); Université Paris-Sud - Paris 11 - Faculté de médecine (UP11 UFR Médecine) ; Université Paris-Sud - Paris 11 (UP11); Centre de référence des Histiocytoses ; Centre de référence des Histiocytoses; Service de pneumologie. Centre de compétence des maladies pulmonaires rares ; Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille); Département de Radiologie [Bichat] (DR- Bichat) ; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris] ; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP); Fondation du Souffle (FR)

Description

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

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