Ty-Jour T1 - 治疗肺肺泡蛋白症的血浆血浆JF - 欧洲呼吸期刊Jo - Eur Respir J SP - 1220 LP - 1222 Do - 10.1183 / 09031936.00097508 VL - 33是 - 5 Au - Luisetti，M. Au - Rodi，G. AU - Perotti, C. AU - Campo, I. AU - Mariani, F. AU - Pozzi, E. AU - Trapnell, B. C. Y1 - 2009/05/01 UR - //www.qdcxjkg.com/content/33/5/1220.abstract N2 - Whole lung lavage (WLL) is currently the standard therapy for pulmonary alveolar proteinosis (PAP). Nevertheless, some PAP patients respond poorly to WLL or require it frequently. The present paper reports a patient with autoimmune PAP with persistent disease despite three WLL treatments over 10 months. Plasmapheresis with ten 1.5-L plasma exchanges was performed, which lowered the serum granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibody level from 250 μg·mL−1 to 156 μg·mL−1 but did not improve respiratory impairment. Further WLL therapy was required and transiently effective. Serum GM-CSF autoantibody levels declined progressively, reaching a value of 56 μg·mL−1 80 weeks after completion of plasmapheresis. However, this decrease was not accompanied by clinical improvement and the patient required additional WLL therapy. The results confirm that minor reductions in serum granulocyte-macrophage colony-stimulating factor autoantibody levels from plasmapheresis are not reflected in clinical improvement in the severity of lung disease in pulmonary alveolar proteinosis. ER -