{Reference Type}: Journal Article
{Title}: Rituximab for auto-immune alveolar proteinosis, a real life cohort study.
{Author}: Soyez B;Borie R;Menard C;Cadranel J;Chavez L;Cottin V;Gomez E;Marchand-Adam S;Leroy S;Naccache JM;Nunes H;Reynaud-Gaubert M;Savale L;Tazi A;Wemeau-Stervinou L;Debray MP;Crestani B;
{Journal}: Respir Res
{Volume}: 19
{Issue}: 1
{Year}: 04 2018 25
暂无{DOI}: 10.1186/s12931-018-0780-5
{Abstract}: 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.<BR>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.<BR>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.<BR>These data do not support rituximab as a second line therapy for patients with refractory aPAP.