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

• Published in: Annals of clinical and laboratory science Vol. 44; no. 4; pp. 405 - 409
• 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
• ISSN: 1550-8080

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.