title: Pulmonary alveolar proteinosis: a long way to correct diagnosis: problems of diagnostics and therapy in routine practice.
abstract: Pulmonary Alveolar Proteinosis is a rare disease with unknown etiology that is due to an abnormal surfactant metabolism. Retrospective analysis of a consecutive series of 68 patients with PAP who were seen at Pulmonology Clinic of Pavlov State Medical University from 1977-2013 was performed. The mean time since first abnormalities were found until diagnosis establishment was 34 months. During that time, most patients were treated with antibiotics, tuberculostatics, glucocorticoids, and immunosupressants. The most effective PAP treatment method was found to be whole lung lavage procedure: 82% of patients showed improvement after its implementation. Delayed diagnosis and incorrect administration of antibiotics, tuberculostatics etc. reduce the probability of a long symptom-free period after lung lavage and spontaneous resolution of the disease.
authors.affiliation: Pulmonology Clinic of Pavlov State Medical University, St. Petersburg.
authors.forename: Yulia M
authors.initials: YM
authors.lastname: Ilkovich
authors.affiliation: St. Petersburg City Mortem Bureau, St. Petersburg Institute of Phthysiopulmonology, St. Petersburg, Russia arielboris@rambler.ru.
authors.forename: Boris M
authors.initials: BM
authors.lastname: Ariel
authors.affiliation: Pulmonology Clinic of Pavlov State Medical University, St. Petersburg.
authors.forename: Lubov N
authors.initials: LN
authors.lastname: Novikova
authors.affiliation: Pulmonology Clinic of Pavlov State Medical University, St. Petersburg.
authors.forename: Andrej A
authors.initials: AA
authors.lastname: Bazhanov
authors.affiliation: Pulmonology Clinic of Pavlov State Medical University, St. Petersburg.
authors.forename: Ivetta V
authors.initials: IV
authors.lastname: Dvorakovskaya
authors.affiliation: Pulmonology Clinic of Pavlov State Medical University, St. Petersburg.
authors.forename: Mikhail M
authors.initials: MM
authors.lastname: Ilkovich
mesh_terms: D000328:Adult; D018893:Bronchoalveolar Lavage / Q000379:methods; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D010808:Physical Examination; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D012189:Retrospective Studies; D015898:Tomography Scanners, X-Ray Computed; D016896:Treatment Outcome
keywords: alveolar proteinosis; lung; surfactant; whole lung lavage