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Pulmonary Alveolar Proteinosis Single Center Case Series Experience
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A5005 - Pulmonary Alveolar Proteinosis Single Center Case Series Experience
Author Block: M. Sierra-Ruiz1, A. C. Chee2, M. S. Parikh1, S. Fernandez-Bussy3, K. Fayez4, A. Majid2; 1Thoracic Surgery and Interventional Pulmonology, Beth Israel Deaconess Medical Center, Boston, MA, United States, 2Thoracic Surgery and Interventional Pulmonology, Beth Israel Deaconess Medical Center, boston, MA, United States, 3Interventional pulmonology, Clinica Alemana de Chile, Santiago de Chil, Chile, 4Interventional pulmonology, Tulane hospital, Tulane, LA, United States.
Background: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by intra-alveolar surfactant accumulation. While some patients with PAP spontaneously improve or even undergo disease remission, the majority of patients develop persistent or progressive disease. Although many therapies have been used to treat PAP, whole lung lavage (WLL) remains the gold standard treatment. In this study, we evaluated the efficacy and complications of WLL treatment.
Methods: This was a retrospective case series of 22 patients; we included 13 patients who had WLL procedures of PAP from 2004 to 2017 in Beth Israel Medical Deaconess center.
Results: 61% (n=8) of patients had secondary PAP while 38% (n=5) had primary PAP. Among patients with secondary PAP, 67% (n=6) were exposed to toxic inhalations, 11%(n=1) had infectious and 11% (n=1) had hematologic diseases. The mean age at the time of diagnosis was 43 years and 53% (n=6) of patients were current or past-smokers. Diagnosis was made with surgical lung biopsy in 38%(n=5) ; transbronchial lung biopsy in 32% (n=5) ;and with clinical presentation, radiographic imaging and bronchoalveolar lavage in 30%(n=3). Exertional dyspnea was reported in all patients. Median disease severity score (DSS) was 5 (IQR 3-5) before and 2 (IQR 2-3) following WLL at a median follow up of 12 days. Median number of WLL needed were 2 (IQR 2-5): 38% (n=5) required bilateral procedure. 38 % (n=5) of patients required more that 2 WLL. There were 32 procedures Complication rate per procedure was 21%. 23% (n=3) had pneumothorax, 7% (n=1) had bronchitis (7%), 7% (n=1) had hypotension and 7%(n=1) had transient hypoxemia. One patient died due to tension pneumothorax, followed by pulseless electrical activity after double lumen intubation.Conclusions: Although WLL remains the most common and effective therapy for PAP, a substantial number of patients required more than 2 therapies to control their symptoms and was associated with increased complication rate.
Author Block: M. Sierra-Ruiz1, A. C. Chee2, M. S. Parikh1, S. Fernandez-Bussy3, K. Fayez4, A. Majid2; 1Thoracic Surgery and Interventional Pulmonology, Beth Israel Deaconess Medical Center, Boston, MA, United States, 2Thoracic Surgery and Interventional Pulmonology, Beth Israel Deaconess Medical Center, boston, MA, United States, 3Interventional pulmonology, Clinica Alemana de Chile, Santiago de Chil, Chile, 4Interventional pulmonology, Tulane hospital, Tulane, LA, United States.
Background: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by intra-alveolar surfactant accumulation. While some patients with PAP spontaneously improve or even undergo disease remission, the majority of patients develop persistent or progressive disease. Although many therapies have been used to treat PAP, whole lung lavage (WLL) remains the gold standard treatment. In this study, we evaluated the efficacy and complications of WLL treatment.
Methods: This was a retrospective case series of 22 patients; we included 13 patients who had WLL procedures of PAP from 2004 to 2017 in Beth Israel Medical Deaconess center.
Results: 61% (n=8) of patients had secondary PAP while 38% (n=5) had primary PAP. Among patients with secondary PAP, 67% (n=6) were exposed to toxic inhalations, 11%(n=1) had infectious and 11% (n=1) had hematologic diseases. The mean age at the time of diagnosis was 43 years and 53% (n=6) of patients were current or past-smokers. Diagnosis was made with surgical lung biopsy in 38%(n=5) ; transbronchial lung biopsy in 32% (n=5) ;and with clinical presentation, radiographic imaging and bronchoalveolar lavage in 30%(n=3). Exertional dyspnea was reported in all patients. Median disease severity score (DSS) was 5 (IQR 3-5) before and 2 (IQR 2-3) following WLL at a median follow up of 12 days. Median number of WLL needed were 2 (IQR 2-5): 38% (n=5) required bilateral procedure. 38 % (n=5) of patients required more that 2 WLL. There were 32 procedures Complication rate per procedure was 21%. 23% (n=3) had pneumothorax, 7% (n=1) had bronchitis (7%), 7% (n=1) had hypotension and 7%(n=1) had transient hypoxemia. One patient died due to tension pneumothorax, followed by pulseless electrical activity after double lumen intubation.Conclusions: Although WLL remains the most common and effective therapy for PAP, a substantial number of patients required more than 2 therapies to control their symptoms and was associated with increased complication rate.
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