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INTRODUCCIÓN: Las opacidades pulmonares en receptores de trasplante de precursores hematopoyéticos (TPH) representan un desafío diagnóstico y son una causa de morbimortalidad. Existen grandes discrepancias con respecto a la sensibilidad diagnóstica del lavado broncoalveolar (LBA), sus complicaciones, y los factores asociados a la identificación microbiológica. OBJETIVO: Conocer la utilidad del estudio microbiológico del LBA en el diagnóstico, modificación de la conducta médica y estimar las complicaciones y mortalidad asociada al procedimiento, en receptores de TPH con opacidades pulmonares. PACIENTES Y MÉTODOS: Estudio de cohorte, retrospectivo, en adultos receptores de TPH a los que se les realizó una broncoscopía con LBA por presentar opacidades pulmonares, en el Hospital Italiano de Buenos Aires entre el 01/01/2011 y el 31/12/2020. RESULTADOS: De los 189 procedimientos analizados, en 79 se logró un hallazgo microbiológico (41,8%) y 122 permitieron modificar la conducta médica (64,6%). En 11 casos se observaron complicaciones graves dentro de las 12 horas (5,8%) de efectuado el LBA. La mortalidad intrahospitalaria fue de 16,8% (N = 21/125). El valor de neutrófilos en sangre previo al LBA (p = 0,037) y la presencia de nódulos pulmonares como lesión tomográfica predominante (p = 0,029) se asociaron independientemente al hallazgo microbiològico global. CONCLUSIONES: Nuestra investigación apoya la realización del LBA como herramienta diagnóstica en pacientes que reciben un TPH y presentan opacidades pulmonares.

BACKGROUND: Lung opacities are a cause of morbimortality in bone marrow transplant patients, and represent a diagnostic challenge. There are large discrepancies regarding the diagnostic sensitivity of bronchoalveolar lavage (BAL), its complications, and the factors associated with microbiological detection. AIM: To know the usefulness of the microbiological study of BAL in the diagnosis, in the modification in medical behavior and to estimate the complications and associated mortality of this diagnostic procedure in patients transplanted with hematopoietic progenitor cells with pulmonary opacities. METHODS: Retrospective cohort study in bone marrow transplant adult patients who underwent bronchoscopy with BAL due to lung opacities at Hospital Italiano de Buenos Aires between 01/01/2011 and 12/31/2020. RESULTS: Of the 189 BAL analyzed, 79 presented a microbiological detection (41.8%) and 122 allowed to modify the medical behavior (64.6%). Severe complications were observed within 12 hours after the procedure in11 cases (5.8%). In-hospital mortality was 16,8% (N = 21/125). The value of blood neutrophils prior to bronchoalveolar lavage (p = 0.037) and the presence of pulmonary nodules as the predominant tomographic lesion (p = 0.029) were independently associated with global microbiological detection. CONCLUSION: Our research supports the performance of BAL as a diagnostic tool in bone marrow transplant patients with lung opacities.

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Objetivos: Exponer en base a un caso clínico una revisión de literatura reciente sobre Proteinosis alveolar pulmonar (PAP). Presentación del caso: Revisión de ficha clínica electrónica de paciente de sexo masculino de 76 años con antecedente de linfoma no Hodgkin (LNH) mesentérico, estirpe B de tipo folicular, quien acude en forma reiterada a servicios de urgencia por cuadro de dos meses de evolución de fiebre, compromiso del estado general y tos. Al examen físico destaca crépitos en hemitórax derecho. Se realizó Tomografía computada (TC) de tórax que mostró opacidades pulmonares en vidrio esmerilado periféricas, con engrosamiento septal liso y algunas bandas retráctiles subpleurales. Se manejó ambulatoriamente con Azitromicina por una semana. Sin respuesta, evoluciona con baja de peso y diaforesis nocturna. Nueva TC de tórax en enero 2021, muestra nuevos focos de "empedrado" periféricos extensos, descrito como "crazy paving", focos de vidrio esmerilado difusos extensos, sin condensación y con resolución de bandas retráctiles. Estudio infeccioso negativo. Se realiza lavado broncoalveolar (LBA) con estudio histológico de líquido que muestra proceso inflamatorio crónico con abundantes macrófagos y material proteináceo. Discusión: Tras el descarte de patología infecciosa, se orientó el estudio hacia otras causas de enfermedad parenquimatosa pulmonar. Así, resulta fundamental la descripción correcta del patrón imagenológico tomográfico y el LBA que resultaron compatibles con PAP. Conclusión: La PAP es una patología infrecuente, pero una historia clínica adecuada, el planteamiento de diagnósticos diferenciales de neumonía de lenta resolución, asociado el reconocimiento del patrón radiológico característico y el estudio histológico con LBA permiten realizar un diagnóstico certero, con gran implicancia terapéutica.

Objective: To present a review of recent literature on pulmonary alveolar proteinosis (PAP) based on a clinical case. Presentation of the case: Review of electronic clinical record of a 76 years-old masculine patient with history of mesenteric Non-Hodgkin Lymphoma (NHL) follicular-type lineage B, who repeatedly attended the emergency services due to a two-month history of symptoms of fever, compromised general condition and cough. Physical examination revealed crepitus in the right hemithorax. Chest computed tomography (CT) was performed, which showed peripheral ground-glass pulmonary opacities, with smooth septal thickening and some subpleural retractile bands. He was managed on an outpatient basis with Azithromycin for one week. No response; evolves with weight loss and night diaphoresis. New chest CT in January 2021, shows new extensive peripheral "cobblestone" foci, described as "crazy paving", extensive diffuse ground glass foci, without condensation and with resolution of retractile bands. Negative infectious study. Bronchoalveolar lavage (BAL) was performed with a histological study of the fluid showing a chronic inflammatory process with abundant macrophages and proteinaceous material. Discussion: After ruling out infectious pathology, the study was oriented towards other causes of pulmonary parenchymal disease. Thus, the correct description of the tomographic imaging pattern and the BAL that were compatible with PAP are essential. Conclusion: PAP is an infrequent pathology, but an adequate clinical history, the approach to differential diagnoses of slowly resolving pneumonia, associated with the recognition of the characteristic radiological pattern and the histological study with BAL allow an accurate diagnosis to be made, with great therapeutic implications.

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Pulmonary alveolar proteinosis is an unusual diffuse lung disease characterized by abnormal accumulation of pulmonary surfactant and lipoproteins in the alveolar space, which impairs gas exchange with a variable clinical course, ranging from an asymptomatic clinical presentation to severely affected respiratory failure. A 16-year-old girl with diagnosis of pulmonary alveolar proteinosis presented to our hospital for therapeutic lung lavage after a recent history of progressive dyspnea, respiratory distress, declining lung function measurements, and worsening radiographic abnormalities. We obtained baseline pulmonary function tests and laboratory measurements before and after therapeutic bilateral lung lavage.

La proteinosis alveolar pulmonar es una enfermedad pulmonar difusa caracterizada por la acumulación anormal de surfactante y lipoproteínas en el espacio alveolar, lo cual empeora el intercambio gaseoso y lleva a un curso variable desde una presentación clínica asintomática hasta una falla respiratoria grave. Se presenta a una adolescente de 16 años con este diagnóstico que fue remitida a nuestro Hospital para la realización de un lavado pulmonar total luego de una historia de disnea progresiva, dificultad respiratoria, caída de la función pulmonar y anormalidades radiológicas. Se le realizaron estudios de función pulmonar y mediciones de laboratorio antes y después del lavado pulmonar terapéutico. Luego, la paciente presentó una inmediata mejoría tanto de los síntomas, radiografía de tórax y mediciones de estudios de función pulmonar como en el intercambio gaseoso. El lavado pulmonar total continúa siendo el estándar de oro para el tratamiento de la proteinosis alveolar pulmonar en casos graves.

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Introduction: Pulmonary alveolar proteinosis (PAP) is a rare disease caused by accumulation of proteinaceous material in the alveolar spaces. The most common mechanism implied is the development of antibodies directed to granulocyte macrophage colony stimulating factor (GM-CSF). which inhibits activation of alveolar macrophage, thereby causing accumulation of surfactant. Standard treatment is considered to be whole lung lavage (WLL). Object: We present the case of a 47 years old woman referred for lung transplantation because of combined lung fibrosis and emphysema. Diagnose was change to respiratory failure secondary to PAP. We performed a single cession bilateral WLL under venous-venous ECMO. After a 5 years follow up, the patients remains asymptomatic and with normal pulmonary physiology. Conclusions: The case highlights the importance of careful examination of previous diagnoses, in particular of cases referred for lung transplantation and provides factibility of performing single session bilateral WLL without causing right heart failure.

Introducción: la proteinosis alveolar es una enfermedad rara caracterizada por la acumulación de material proteináceo en los espacios alveolares. El principal mecanismo implicado es el desarrollo de anticuerpos contra el factor estimulante de colonias de granulocitos y macrófagos (GM-CSF), que impide la activación del macrófago alveolar y genera acumulación de surfactante. Su tratamiento estándar es el lavado pulmonar total (LPT). Objetivo: presentar el caso de una mujer de 47 años derivada para trasplante pulmonar por sospecha de combinación de fibrosis y enfisema. Se realizó el diagnóstico insuficiencia respiratoria por proteinosis alveolar pulmonar severa y se realizó tratamiento con lavado pulmonar total bilateral, secuencial y en una sola sesión bajo ECMO veno-venoso. A 5 años del tratamiento, la paciente se encuentra asintomática y con fisiología pulmonar normal. Conclusiones: el caso resalta la importancia de evaluar cuidadosamente el diagnóstico de todos los pacientes, en especial los que son derivados a trasplante pulmonar y muestra la factibilidad de realizar el lavado pulmonar total bajo ECMO veno-venoso sin producir claudicación del ventrículo derecho.

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El patrón llamado 'crazy paving' en tomografia computada de tórax (TAC) puede deberse a diferentes condiciones siendo una de ellas la Proteinosis Alveolar Pulmonar (PAP), rara condición que puede llevar a insuficiencia respiratoria y a menudo, a la muerte. Presentamos el caso de una mujer joven con una historia de un año de evolución de disnea progresiva y tos seca que consultó por un cuadro de aparición brusca de fiebre, calofríos, malestar general y falla respiratoria hipoxémica severa (PaO2 = 51,9 mmHg con FiO2 = 0,50) en la cual la TAC de tórax mostraba un patrón de empedrado o 'crazy paving' que significó un desafío diagnóstico resuelto finalmente con una biopsia pulmonar quirúrgica que mostró una PAP. Ante el fracaso del tratamiento tradicional de Lavado Pulmonar Total (LPT) se usó una aproximación terapéutica novedosa consistente en una serie de 4 lavados lobares con un perfluorocarbono, Perflubron (PFC) bajo anestesia local seguido por 5 sesiones de Plasmaféresis. Casi inmediatamente después de este tratamiento la paciente evidenció mejoría radiológica y funcional. La PaO2 fue de 89,9 mmHg respirando aire ambiental y la CVF y el VEF1 aumentaron alcanzado respectivamente el 77 y el 75% de sus valores normales de referencia. Dadas las características químicas y físicas del PFC, pensamos que es una alternativa válida al LPT en estos casos.

Crazy paving computed tomography pattern may be due to a number of causes, one of them being Pulmonary Alveolar Proteinosis, a rare condition leading to respiratory failure and often to death. We present the case of a young woman with a one-year history of progressive dyspnea and dry cough, who consulted for an acute onset of fever, chills, malaise and severe hypoxemic respiratory failure (PaO2 = 51.9 mmHg; FiO2 = 0.50) with a 'crazy paving' pattern on chest CT. This diagnostic challenge was resolved by a surgical lung biopsy that showed a pulmonary alveolar proteinosis. Taking into account that the traditional treatment using whole lung lavage had already failed in this patient, a novel therapeutic approach was settled. A series of 4 lobar lavages with a perfluorocarbon (Perflubron) under local anesthesia followed by 5 plasmapheresis sessions were carried out. The patient showed radiographic and functional improvement almost immediately after this treatment. PaO2 was 89.9 mmHg breathing room air and FVC and FEV1 increased to reach 77 and 75% respectively of their normal reference values. Because of its chemical and physical properties we think this novel therapeutic approach should be a valuable alternative to saline solution for whole lung lavage in these cases.

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OBJECTIVE: Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by the alveolar accumulation of surfactant, which is composed of proteins and lipids. PAP is caused by a deficit of macrophage activity, for which the main treatment is whole-lung lavage (WLL). We report the experience at a referral center for PAP in Brazil. METHODS: This was a retrospective study involving patients with PAP followed between 2002 and 2016. We analyzed information regarding clinical history, diagnostic methods, treatments, and outcomes, as well as data on lung function, survival, and complications. RESULTS: We evaluated 12 patients (8 of whom were women). The mean age was 41 ± 15 years. Most of the patients were diagnosed by means of BAL and transbronchial biopsy. The mean number of WLLs performed per patient was 2.8 ± 2.5. One third of the patients never underwent WLL. Four patients (33.3%) had associated infections (cryptococcosis, in 2; nocardiosis, in 1; and tuberculosis, in 1), and 2 (16.6%) died: 1 due to lepidic adenocarcinoma and 1 due to complications during anesthesia prior to WLL. When we compared baseline data with those obtained at the end of the follow-up period, there were no significant differences in the functional data, although there was a trend toward an increase in SpO2. The median follow-up period was 45 months (range, 1-184 months). The 5-year survival rate was 82%. CONCLUSIONS: To our knowledge, this is the largest case series of patients with PAP ever conducted in Brazil. The survival rate was similar to that found at other centers. For symptomatic, hypoxemic patients, the treatment of choice is still WLL. Precautions should be taken in order to avoid complications, especially opportunistic infections.

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OBJECTIVES: To establish the reference values, diagnostic accuracy, and effect of various factors on cell count in intubated preterm neonates subjected to nonbronchoscopic bronchoalveolar lavage. STUDY DESIGN: This prospective, cross-sectional, blinded study included preterm neonates ventilated for any reason who underwent nonbronchoscopic bronchoalveolar lavage if they had not previously received postnatal antibiotics or steroids. Lavage was performed before surfactant replacement, if any. A gentle ventilation policy was applied. Pneumonia was diagnosed using clinical criteria, without considering cell count. Investigators performing cell counts were blinded to the clinical data. RESULTS: There were 276 neonates enrolled; 36 had congenital or ventilator-associated pneumonia. In the 240 noninfected babies, median neutrophil count increased significantly after the first 2 days of ventilation (day 1, 2 cells per field [IQR, 0.0-9.5 cells per field]; day 2, 2 cells per field [IQR, 0-15 cells per field]; day 3, 20 cells per field [IQR, 2-99 cells per field]; day 4, 15 cells per field [IQR, 2-96 cells per field]; P < .0001). No significant difference was seen over time in infected babies. Multivariate analysis indicated pneumonia (standardized ß = 0.134; P = .033) and the time spent under mechanical ventilation before nonbronchoscopic bronchoalveolar lavage as factors significantly influencing neutrophil count (standardized ß = 0.143; P = .027). Neutrophil count was correlated with the duration of ventilation (rho = 0.28; P <.001). Neutrophil counts were higher in infected (24 cells/field [IQR, 5-78] cells/field) than in noninfected babies (4 cells/field [IQR, 1-24 cells/field]; P <.001) and had an moderate reliability for pneumonia within the first 2 days of ventilation (area under the curve, 0.745; (95% CI, 0.672-0.810; P = .002). CONCLUSIONS: We provide reference values for airway neutrophil counts in ventilated preterm neonates. Bronchoalveolar lavage neutrophils significantly increase after 2 days of ventilation. Neutrophil count has moderate accuracy to diagnose pneumonia, but only within the first 2 days of ventilation.

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ABSTRACT Objective: Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by the alveolar accumulation of surfactant, which is composed of proteins and lipids. PAP is caused by a deficit of macrophage activity, for which the main treatment is whole-lung lavage (WLL). We report the experience at a referral center for PAP in Brazil. Methods: This was a retrospective study involving patients with PAP followed between 2002 and 2016. We analyzed information regarding clinical history, diagnostic methods, treatments, and outcomes, as well as data on lung function, survival, and complications. Results: We evaluated 12 patients (8 of whom were women). The mean age was 41 ± 15 years. Most of the patients were diagnosed by means of BAL and transbronchial biopsy. The mean number of WLLs performed per patient was 2.8 ± 2.5. One third of the patients never underwent WLL. Four patients (33.3%) had associated infections (cryptococcosis, in 2; nocardiosis, in 1; and tuberculosis, in 1), and 2 (16.6%) died: 1 due to lepidic adenocarcinoma and 1 due to complications during anesthesia prior to WLL. When we compared baseline data with those obtained at the end of the follow-up period, there were no significant differences in the functional data, although there was a trend toward an increase in SpO2. The median follow-up period was 45 months (range, 1-184 months). The 5-year survival rate was 82%. Conclusions: To our knowledge, this is the largest case series of patients with PAP ever conducted in Brazil. The survival rate was similar to that found at other centers. For symptomatic, hypoxemic patients, the treatment of choice is still WLL. Precautions should be taken in order to avoid complications, especially opportunistic infections.

RESUMO Objetivo: A proteinose alveolar pulmonar (PAP) é uma doença rara, caracterizada pelo acúmulo alveolar de substância surfactante, composta por proteínas e lipídios. É causada por um déficit de atividade macrofágica, cujo principal tratamento é a lavagem pulmonar total (LPT). Relatamos a seguir a experiência de um centro de referência brasileiro em PAP. Métodos: Estudo retrospectivo com a avaliação de pacientes com PAP acompanhados entre 2002 e 2016. Informações sobre história clínica, métodos diagnósticos, tratamentos realizados e desfechos, incluindo dados de função pulmonar, sobrevida e presença de complicações, foram analisados. Resultados: Foram incluídos 12 pacientes (8 mulheres), com média de idade de 41 ± 15 anos. A maioria dos pacientes foi diagnosticada por LBA e biópsia transbrônquica. A média do número de LPT realizadas por paciente foi de 2,8 ± 2,5. Um terço dos pacientes não foi submetido à LPT. Quatro pacientes (33,3%) apresentaram infecções associadas (criptococose, em 2; nocardiose, em 1; e tuberculose, em 1) e houve 2 óbitos (16,6%): 1 por adenocarcinoma lepídico e 1 por complicações na indução anestésica pré-LPT. Não houve diferença significativa entre os dados funcionais; porém houve uma tendência de aumento da SpO2 ao se comparar os dados iniciais aos do final do seguimento. A mediana de seguimento foi de 45 meses (1-184 meses), com taxa de sobrevida em 5 anos de 82%. Conclusões: Pelo que sabemos, esta é a maior série de casos de PAP no Brasil. A taxa de sobrevida foi semelhante à encontrada em outros centros. A LPT ainda é o tratamento de escolha para pacientes sintomáticos e hipoxêmicos. Deve-se atentar para complicações, especialmente infecções oportunistas.

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INTRODUCTION: Pulmonary alveolar proteinosis is a rare, diffuse interstitial lung disease, characterized by alveolar obstruction due to the accumulation of pulmonary surfactant. CLINICAL PRESENTATION: A 30-year-old male with progressively worsening dyspnea and non-productive cough for one year. He was a sugar cane plantation worker and had prior recurrent respiratory infections. Physical exam revealed cyanosis, and bilateral coarse and fine rales. Chest computed tomography showed diffuse crazy paving pattern. Bronchoscopy with bronchoalveolar lavage yielded a foamy, thick whitish material. Cytology revealed lymphocytes and acellular proteinaceous eosinophilic material. Transbronchial biopsy confirmed the diagnosis of pulmonary alveolar proteinosis. Patient met criteria for whole lung lavage, responding favorably to this therapy. CONCLUSION: Pulmonary alveolar proteinosis is a rare lung disease and important to consider due to the diagnostic and therapeutic challenge it represents.

INTRODUCCIÓN: La proteinosis alveolar pulmonar es una enfermedad intersticial difusa poco frecuente, en la cual se produce obstrucción alveolar, debido al acúmulo de surfactante pulmonar. REPORTE DEL CASO: Varón de 30 años de edad, presentó disnea progresiva y tos seca de un año de evolución. Antecedente personal: estibador de caña de azúcar. Presentó infecciones respiratorias recurrentes. Al examen físico se encontró cianosis, crepitantes difusos bilaterales y subcrepitantes en bases pulmonares. En la tomografía torácica con contraste se encontró un patrón de "empedrado loco". Se realizó videobroncoscopia con lavado broncoalveolar, aspirando material lechoso, espumoso y mucoso. Por citología se encontró linfocitos y material eosinofílico proteináceo acelular. El estudio anatomopatológico de la biopsia transbronquial reveló proteinosis alveolar pulmonar. El paciente reunió los criterios para tratamiento con lavado broncoalveolar total. Luego de este procedimiento, evolucionó favorablemente. CONCLUSIÓN: La proteinosis alveolar pulmonar constituye una enfermedad importante a considerar, por el desafío diagnóstico y terapéutico que representa.

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