Vol 4-2 Mini Review

Pulmonary Alveolar Proteinosis: Own Experience of Diagnosis and Treatment

Boris M. Ariel1,2*, Ivetta V. Dvorakovskaya1,3, Ludmila N. Novikova3, Mikhail M. Ilkovich3

1St. Petersburg Research Institute of Phthisiopulmonology, Health Ministry of Russia, St. Petersburg, Russia

2St. Petersburg City Mortem Bureau, St. Petersburg, Russia

3Pulmonology Clinic of Pavlov State Medical University, St. Petersburg, Russia

Pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease with severe impairment of respiratory function caused by some genetic abnormalities of surfactant production and utilization. One of the clue moments in the pathogenesis of this disease which can lead to respiratory failure and death is the pulmonary fibrosis development occurring occasionally in patients with moderate and severe PAP. According to our own experience in 1977-2018 whole and segmental lung lavage remains the first-line treatment of PAP; 70 patients (82%) had shown an improvement after this treatment. A noticeable improvement was achieved in 67 patients (79%), and no serious complications were observed. The 5-year survival rate reached 100%. Nevertheless, delayed diagnosis and incorrect administration of antibiotics and tuberculostatics reduce the probability of a long symptom-free period after lung lavage and resolution of the disease.

DOI: 10.29245/2572-9411/2019/2.1172 Read More