Pulmonary alveolar proteinosis: a long way to correct diagnosis: problems of diagnostics and therapy in routine practice

Pulmonary alveolar proteinosis: a long way to correct diagnosis: problems of diagnostics and therapy in routine practice

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 mea...

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Bibliographic Details
Published in:Annals of clinical and laboratory science Vol. 44; no. 4; p. 405
Main Authors: Ilkovich, Yulia M, Ariel, Boris M, Novikova, Lubov N, Bazhanov, Andrej A, Dvorakovskaya, Ivetta V, Ilkovich, Mikhail M
Format: Journal Article
Language:English
Published: United States 2014
Subjects:
Adult
Bronchoalveolar Lavage - methods
Female
Humans
Longitudinal Studies
Male
Middle Aged
Physical Examination
Pulmonary Alveolar Proteinosis - diagnosis
Pulmonary Alveolar Proteinosis - physiopathology
Pulmonary Alveolar Proteinosis - therapy
Retrospective Studies
Tomography Scanners, X-Ray Computed
Treatment Outcome
lung
alveolar proteinosis
whole lung lavage
surfactant
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Summary: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.
ISSN:1550-8080