Long term follow-up in patients with pulmonary alveolar proteinosis

Ref ID: 18403

Author:

Yulia Ilkovich, Lubov Novikova, Olga Baranova, Ivetta Dvorakovskaya,
Andrey Bazhanov.

Author address:

Research Institute of Pulmonology, Pavlov State Medical
University, St. Petersburg, Russian Federation

Full conference title:

European Respiratory Congress

Abstract:

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by
respiratory failure due to surfactant accumulation within the lung. PAP is currently
treated by whole lung lavage (WLL).
Since 1977, our cohort is 52 (35 male, 17 female) idiopathic PAP patients. Median
age – 37±9 y.o. Most patients are current or former smokers (64%) and have
occupational history (55%). Most patients have moderate impairments in functional
parameters (mean DLCO – 65±15% pred., mean pulmonary artery systolic
pressure – 30±6 mm Hg).
The majority of patients (88.4%) received WLL therapy. 3 patients (5.8%) had
segmental lavage, 3 patients (5.8%) – N-acetylcysteine (NAC) as monotherapy.
The average number of WLL procedures was 2 (1-7). 34.6% received only one
WLL and demonstrated long-term improvement after the procedure.
5-years survival rate is 100%. Spontaneous resolution was observed in 2 patients
(3.8%) on NAC treatment. In most cases the course of PAP was slowly progressive.
8 patients (15.4%) had rapid progressive deterioration with respiratory failure despite
repeated WLL. Predictors of fast progression were estimated. No associations
with gender, age, smoking status were found. Long antibiotics and corticosteroids
(CS) intake due to incorrect diagnoses before PAP diagnostic (p<0.05) and more than 3 years since first symptoms occurred till PAP diagnostic (p<0.05) correlated with progressive deterioration despite repeated WLL treatments. Besides, long CS intake increased the risk of secondary infections: pneumonia in 3 patients (5.8%), tuberculosis in 3 patients (5.8%), aspergillosis - 1 patient (1.9%). Avoiding of delayed diagnosis/incorrect treatment increases the probability of long symptom-free period after WLL.

Abstract Number: P638

Conference Year: 2011

Link to conference website: http://www.ers-education.org/ersMade/abstract_print_11/files/Abstract_book_2011.pdf

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