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Clin Respir J ; 17(10): 1077-1081, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37300395

ABSTRACT

INTRODUCTION: Autoimmune pulmonary alveolar proteinosis (aPAP) is a disease caused by IgG antibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). Whole lung lavage (WLL) allows to remove the lipo-proteinaceous material accumulated by the poor clearance of alveolar surfactant. However, it is a complex technique that is not exempt from complications, and in some cases, the patients are refractory, requiring the performance of several WLLs spaced apart in time. MATERIALS AND METHODS: We present the clinical, functional, and radiological evolution after 24 months of follow-up of a patient diagnosis of aPAP refractory to WLL, with performed three therapeutic WLLs spaced 16 and 36 months and serious potentially fatal complications in the last one. RESULTS AND DISSCUSION: After 24 months, no adverse effects have appeared and the great clinical, functional and radiological response is maintained. The patient has been successfully treated with inhaled recombinant human GM-CSF sargramostim.


Subject(s)
Autoimmune Diseases , Pulmonary Alveolar Proteinosis , Humans , Pulmonary Alveolar Proteinosis/diagnostic imaging , Pulmonary Alveolar Proteinosis/drug therapy , Granulocyte-Macrophage Colony-Stimulating Factor/therapeutic use , Follow-Up Studies , Administration, Inhalation , Autoimmune Diseases/drug therapy
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