Pulmonary Alveolar Proteinosis and Whole Lung Lavage. Case Report
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Abstract
Pulmonary Alveolar Proteinosis (PAP) is a disease that belongs to the large number of interstitial lung pathologies, with a low incidence and prevalence worldwide. It consists of an accumulation of proteinaceous material derived from surfactant, which is generated by different causes (primary, secondary, congenital and unclassified), within which the autoimmune or primary cause is the most frequent. Its treatment of choice is total lung lavage, with improvement depending on the degree of involvement and progression of the disease. Currently, there are new therapies and others that are being studied, but in developing countries they are often not available. We present the case of a 32-year-old female patient who presented with type 1 respiratory failure and debuting with PAP.
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References
Mejía AMH, Alonso MD, Suárez LT et al. Proteinosis alveolar pulmonar. Neumol Cir Torax 2006;65(S3):S15-S23.
Yoon H, Kim JH, Kim YJ, Song JW. Pulmonary alveolar proteinosis in Korea: analysis of prevalence and incidence via a nationwide population-based study. BMC Pulmonary Medicine 2020;20(1):34. Doi: https://doi.org/10.1186/s12890-020-1074-5.
Rioseco SP. Proteinosis alveolar pulmonar. Rev Chil Enferm Respir 2019;35(3):207-18. Doi: http://dx.doi.org/10.4067/S0717-73482019000300207.
Suzuki T, Trapnell BC. Pulmonary Alveolar Proteinosis Syndrome. Clin Chest Med 2016;37(3):431-40. Doi: 10.1016/j.ccm.2016.04.006.
Tabaj G, Enghelmayer J, Ernst G, Zorrilla L, Scarinci M. Proteinosis alveolar pulmonar: una puesta al día. RAMR 2014;3:282-288.
Inoue Y, Trapnell BC, Tazawa R et al. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan. Am J Respir Crit Care Med 2008;177(7): 752-62. Doi: 10.1164/rccm.200708-1271OC.
Kumar A, Abdelmalak B, Inoue Y, Culver DA. Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach. Lancet Respir Med 2018;6(7):554-65. Doi: 10.1016/S2213-2600(18)30043-2.
Salvaterra E, Campo I. Pulmonary alveolar proteinosis: from classification to therapy.
Breathe (Sheff) 2020;16(2):200018. Doi: 10.1183/20734735.0018-2020.
Kelly A, McCarthy C. Pulmonary Alveolar Proteinosis Syndrome. Semin Respir Crit Care Med 2020;41(2):288-98. Doi: 10.1055/s-0039-3402727.
Soyez B, Borie R, Menard C et al. Rituximab for autoimmune alveolar proteinosis, a real life cohort study. Respiratory Research 2018;19:74. Doi: 10.1186/s12931-018-0780-5.
Awab A, Khan MS, Youness HA. Whole lung lavage-technical details, challenges and management of complications. J Thorac Dis 2017;9(6):1697-706. Doi: 10.21037/jtd.2017.04.10.
Silva A, Moreto A, Pinho C et al. Bilateral whole lung lavage in pulmonary alveolar proteinosis--a retrospective study. Rev Port Pneumol 2014;20:254-9.
Campo I, Luisetti M, Griese M et al. Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures. Orphanet J Rare Dis 2016;11:115.
Rodríguez Portal JA. Treatment of Adult Primary Alveolar Proteinosis. Arch
Bronconeumol 2015;51(7):344-9. Doi: 10.1016/j.arbres.2015.02.003.