title: Plasmapheresis for treatment of pulmonary alveolar proteinosis.
abstract: 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 microg mL(-1) to 156 microg 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 microg 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.
authors.affiliation: Clinica Malattie Apparato Respiratorio, Fondazione IRCCS Policlinico San Matteo, Università di Pavia, Pavia, Italy. m.luisetti@smatteo.pv.it
authors.forename: M
authors.initials: M
authors.lastname: Luisetti
authors.forename: G
authors.initials: G
authors.lastname: Rodi
authors.forename: C
authors.initials: C
authors.lastname: Perotti
authors.forename: I
authors.initials: I
authors.lastname: Campo
authors.forename: F
authors.initials: F
authors.lastname: Mariani
authors.forename: E
authors.initials: E
authors.lastname: Pozzi
authors.forename: B C
authors.initials: BC
authors.lastname: Trapnell
mesh_terms: D000328:Adult; D018893:Bronchoalveolar Lavage; D006801:Humans; D008297:Male; D010956:Plasmapheresis; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis / Q000628:therapy*