Proteinosis alveolar

Carlos Bula G.; Ernesto Santiago H.; Brenda Martínez O.; Melisa Reyes; Ricardo Pabón R.

doi:10.30789/rcneumologia.v29.n1.2017.239

Alveolar proteinosis

Proteinosis alveolar

Published 2017-08-01

Open | Download

PDF (Spanish) FLIP

Issue

Vol. 29 No. 1 (2017)

Section

Presentación de casos

How to Cite

Bula G. C, Santiago H. E, Martínez O. B, Reyes M, Pabón R. R. Alveolar proteinosis.

rev. colomb. neumol. [Internet]. 2017 Aug. 1 [cited 2025 Jun. 3];29(1):55-62. Disponible en: https://doi.org/10.30789/rcneumologia.v29.n1.2017.239

DOI

https://doi.org/10.30789/rcneumologia.v29.n1.2017.239

Dimensions

PlumX

license

Ninguna publicación, nacional o extranjera, podrá reproducir ni traducir sus artículos ni sus resúmenes sin previa autorización escrita del editor; sin embargo los usuarios pueden descargar la información contenida en ella, pero deben darle atribución o reconocimiento de propiedad intelectual, deben usarlo tal como está, sin derivación alguna.

Carlos Bula G.

Hospital Fundación Universidad Metropolitano (FHUM). Barranquilla

Ernesto Santiago H.

Clínica General del Norte. Barranquilla

Brenda Martínez O.

Fundación Hospital Universidad Metropolitana de Barranquilla. Barranquilla

Melisa Reyes

Universidad Libre de Barranquilla.Barranquilla,

Ricardo Pabón R.

Fundación Universitaria Hospital Metropolitano, Barranquilla

Show authors biography

Carlos Bula G.,

Médico Neumólogo. Coordinador Departamento de Neumología y Docente de Postgrado Hospital Fundación Universidad Metropolitano (FHUM). Barranquilla,Colombia.

Ernesto Santiago H.,

Médico Internista Neumólogo: Clínica General del Norte. Barranquilla, Colombia.

Brenda Martínez O.,

Médico Internista: Fundación Hospital Universidad Metropolitana de Barranquilla. Barranquilla, Colombia

Melisa Reyes,

Residente de tercer año de Medicina Interna. Universidad Libre de Barranquilla.Barranquilla, Colombia.

Ricardo Pabón R.,

Residente de segundo año de Medicina Interna FHUM. Fundación Universitaria Hospital Metropolitano, Barranquilla, Colombia.

alveolar proteinosis

dyspnea

total pulmonary lavage

Pulmonary alveolar proteinosis is a rare disease characterized by accumulation of surfactant within the alveolar space, which leads to a deranged gas exchange. Its clinical course is variable, ranging from spontaneous resolution to respiratory failure and death. Three forms have been described: genetic, secondary, and primary or idiopathic. The latter is the most common form in the adult population. The pathophysiology is given by an autoimmune process against the granulocyte-macrophage colony-stimulating factor (GM-CSF); this process conditions a functional impairment of macrophages that leads to accumulation of surfactant within the alveoli, as well as pulmonary and extrapulmonary infectious manifestations, often due to unusual germs. The bulk of data yielded by image studies, clinical-radiological dissociation, and bronchoalveolar lavage material is usually enough for reaching diagnosis, although histopathology is sometimes required for confirmation. Recommended treatment is total pulmonary lavage. The mortality is low and is fundamentally related to infectious complications. We describe a case of pulmonary alveolar proteinosis that required confirmation by histopathologic study of thoracoscopic lung biopsy. We also offer a review of the literature.

Article visits 5354 | PDF visits 2934

Downloads

Download data is not yet available.

Rosen SH, Castleman B, Liebbow AA. Pulmonary alveolar proteinosis. N Engl J Med. 1958; 258:1124-42.
Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis:progress in the first 44 years. Am J Respir Crit Care Med.2002;166:215-35.
Larson RK, Gordinier R. Pulmonary alveolar proteinosis: report of six cases, review of the literature, and formulation of a new theory. Ann Intern Med. 1965;62:292-312.
Kumar V, Abbas AK, Fausto N, Aster JC. Robbins y Cotran.Patología estructural y funcional, 7a ed. Ed. Elsevier; 2007.p. 745-6.
Borie R, Danel C, Debray MP, Taille C, Dombret MC, Aubier M, et al. Pulmonary alveolar proteinosis. Eur Respir Rev.2011; 20:98-107.
Inoue Y, Trapnell BC, Tazawa R, Arai T, Takada T, Hizawa N. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan. Am J Respir Crit Care Med. 2008;177: 752–62.
Teja K, Cooper PH, Squires JE, Schnatterly PT. Pulmonary alveolar proteinosis in four siblings. N Engl J Med.1981;305(23): 1390-2.
Suzuki T, Sakagami T, Young LR, Carey BC, Wood RE,Luisetti M, et al. Hereditary pulmonary alveolar proteinosis:pathogenesis, presentation, diagnosis, and therapy. Am J Respir Crit Care Med. 2010; 182(10): 1292-304.
Schiller V, Aberle DR, Aberle AM. Pulmonary alveolar proteinosis. Occurrence with metastatic melanoma to lung. Chest.1989; 95(2): 466-7.
Bedrossian CW, Luna MA, Conklin RH, Miller WC. Alveolar proteinosis as a consequence of immunosuppression. A hypothesis based on clinical and pathologic observations. Hum Pathol. 1980;11(5 Suppl):527-35.
Ruben FL, Talamo TS. Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome. Am J Med. 1986;80(6):1187-90.
Keller CA, Frost A, Cagle PT, Abraham JL. Pulmonary alveolar proteinosis in a painter with elevated pulmonary concentrations of titanium. Chest. 1995;108(1):277-80.
Chan ED, King TE. Diagnosis and treatment of pulmonary alveolar proteinosis in adults. Uptodate. [Sede web]. Jun, 2013.Waltham, Massachusetts. com/
Kitamura T, Tanaka N, Watanabe J, et al. Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colonystimulating factor. J Exp Med. 1999;190:875-80.
Paine R, Preston AM, Wilcoxen S, Jin H, Siu BB, Morris SB, et al. Granulocyte-macrophage colony-stimulating factor in the innate immune response to Pneumocystis carinii pneumonia in mice. J Immunol. 2000; 164(5): 2602-9.
Kumar V, Abbas AK, Fausto N, Aster JC. Robbins y Cotran. Patología estructural y funcional, 7a ed. Ed. Elsevier; 2007. p. 745-6.
Rodríguez J, Rodríguez E, Sánchez A. Proteinosis alveoloar.Respuesta al tratamiento con factor estimulante de colonias de granulocitos y macrófagos por vía inhalada. Arch Bronconeumol. 2009;45(3):150-2.
Bonella F, Bauer PC, Griese M, Ohshimo S, Guzman J, Costabel U. Pulmonary alveolar proteinosis: new insights from a single-center cohort of 70 patients. Respir Med.2011;105(12):1908-16.
Leth S, Bendstrup E, Vestergaard H, Hilberg O. Autoimmune pulmonary alveolar proteinosis: treatment options in year 2013. Respirology. 2013;18(1):82.
Lin FC, Chang GD, Chern MS, Chen YC,Chang SC. Clinical significance of anti-GMCSF antibodies in idiopathic pulmonary alveolar proteinosis. Thorax. 2006;61:528-34.
Trapnell BC, Uchida K. Pulmonary alveolar proteinosis. Eur Respir Mon. 2009;46(12):208-24.
Huizar I, Kavuru M. Alveolar proteinosis syndrome: pathogenesis, diagnosis and management. Curr Opin Pulm Med.2009;15(5):491-8.
Chan ED, King TE. Diagnosis and treatment of pulmonary alveolar proteinosis in adults. Uptodate. [Sede web]. Jun, 2013.Waltham, Massachusetts.
Vancheeswaran R, Morgan C, Wells A. Pulmonary alveolar proteinosis. Investigation and management –A review. Clin Pulmonary Med. 2007;14(6):360-4.
Kavuru MS, Malur A, Marshall I, Barna BP, Meziane M, Huizar I, et al. An open-label trial of rituximab therapy in pulmonary alveolar proteinosis. Eur Respir J. 2011;38:1361-7.
Tazawa R, Trapnell BC, Inoue Y, Arai T, Takada T, Nasuhara Y, et al. Inhaled granulocyte/macrophage-colony stimulating factor as therapy for pulmonary alveolar proteinosis. Am J Respir Crit Care Med. 2015;181(12):1345-54.
Beccaria M, Luisetti M, Rodi G, Corsico A, Zoia MC, Colato S, et al. Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis. Eur Respir J. 2004;23(4):526-31.
Luisetti M, Rodi G, Perotti C, Campo I, Mariani F, Pozzi E, et al. Plasmapheresis for treatment of pulmonary alveolar proteinosis. Eur Respir J. 2009;33(5):1220-2.
Rodríguez Robles AO, Ángel Ortega M, Valenciano López C, Araújo J, Ortiz Barraza E, Pérez Rodríguez. Proteinosis alveolar y lavado broncoalveolar terapéutico(LBA-T).Tratamiento solo o combinado.Patología Respiratoria.2014;17(2).