Proteinosis alveolar pulmonar: reporte de caso en paciente con linfoma no hodgkin mesentérico / Pulmonary alveolar proteinosis: report of a case in patient with history of mesenteric non-hodgkin lymphoma

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.

Proteinosis alveolar pulmonar / Pulmonary alveolar proteinosis

Resumen La proteinosis alveolar pulmonar (PAP) es una enfermedad pulmonar difusa, infrecuente, secundaria a una alteración en la homeostasis del surfactante. Se presenta el caso de una mujer de 69 años que ingresó a sala de internación por disnea progresiva hasta clase funcional III, de tres meses de evolución, asociada a tos no productiva. Se constató insuficiencia respiratoria tipo I. Como hallazgos en tomografía de tórax se evidenció engrosamiento del intersticio pulmonar intra e interlobulillar, opacidades en vidrio esmerilado y áreas con tendencia a la consolidación bilateral. Se realizó biopsia pulmonar con diagnóstico histológico de PAP y se efectuó tratamiento con lavado pulmonar total, logrando mejoría clínica. Se destaca la necesidad de tener presente diagnósticos diferenciales de insuficiencia respiratoria e infiltrados pulmonares en el contexto de la pandemia por COVID-19, incluidas las entidades muy poco prevalentes como lo es la PAP.

Abstract Pulmonary alveolar proteinosis (PAP) is a rare, diffuse pulmonary disease due to abnormal surfactant homeostasis. We present the case of a 69-year-old woman who was admitted to the hospital for progressive dyspnea with marked limitation in activity, and non-productive cough, of three months of evolution. Type I respiratory failure was confirmed. Chest tomography findings were interlobular and intralobular septal thickening, ground glass opacities and bilateral consolidation. Histological diagnosis was made and whole-lung lavage was performed with clinical improvement. We highlight the need to keep in mind differential diagnoses of respiratory failure and pulmonary infiltrates during COVID-19 pandemic, even rare entities such as PAP.

Clinical characteristics of pulmonary alveolar proteinosis / 中南大学学报(医学版)

OBJECTIVES@#Pulmonary alveolar proteinosis (PAP) is a rare disease with non-specific and various clinical manifestations, often leading to misdiagnosis. This study aims to raise the awareness of this disease via summarizing the clinical characteristics, diagnosis, and therapy of PAP.@*METHODS@#We retrospectively analyzed clinical data of 25 hospitalized cases of PAP during 2008 and 2019 in the Department of Respiratory and Critical Care Medicine of the Second Xiangya Hospital of Central South University.@*RESULTS@#Cough with unkown reason and dyspnea were common clinical manifastations of PAP. Five patients had a history of occupational inhalational exposure. Sixteen patients had typical image features including ground-glass opacification of alveolar spaces and thickening of the interlobular and intralobular septa, in typical shapes called crazy-paving and geographic pattern. Fourteen patients underwent pulmonary function tests, revealing a reduction in the diffusing capacity for carbon monoxide. The positive rate of transbronchial biopsy was 95%. Five patients received the whole lung lavage and the symptoms and imaging fcauters significantly relieved after five-years follow-up.@*CONCLUSIONS@#PAP is characterized by radiographic pattern and pathology. Transbronchial lung biopsy is effective to make diagnosis of PAP. The whole lung lavage remains a efficient therapy.

Patrón crazy paving en una mujer joven: hallazgo inesperado y tratamiento no tradicional en un caso de proteinosis alveolar pulmonar / Crazy paving computed tomography pattern in a young woman: unexpected findings and non-traditional treatment in a pulmonary alveolar proteinosis case

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.

Proteinosis alveolar pulmonar / Pulmonary alveolar proteinosis an update

La Proteinosis Alveolar Pulmonar (PAP) es una enfermedad poco frecuente, caracterizada por la acumulación de material lipoproteico derivado del surfactante pulmonar al interior de los alvéolos por una falla de depuración de este material por los macrófagos alveolares, siendo la causa más frecuente de esta disfunción la acción bloqueadora producida por anticuerpos anti factor estimulante de colonias de granulocitos y macrófagos (GM-CSF) lo que lleva a un deterioro del intercambio gaseoso. La evolución es variable abarcando desde la resolución espontánea hasta la insuficiencia respiratoria grave y la muerte. Se describen tres formas de PAP: Genética, secundaria y autoinmune (antes primaria o idiopática) siendo esta última la más frecuente en adultos. Clínicamente, se manifiesta por disnea, tos seca e hipoxemia que pueden ser progresivas. En la radiografía de tórax se encuentran opacidades bilaterales y la tomografía computarizada de tórax de alta resolución (TACAR) muestra vidrio esmerilado con sobre posición de engrosamiento septal intra e interlobulillar, patrón conocido como "crazy paving". El diagnóstico se basa en la clínica y en el lavado broncoalveolar con material PAS positivo. La biopsia quirúrgica es confirmatoria. El tratamiento clásico es el lavado pulmonar total (LPT) para remover el contenido alveolar. Otras alternativas son la administración de GM-CSF subcutáneo o inhalado, plasmaferesis y rituximab, cuyos resultados son variables. Diferentes autores han modificado la forma del LPT y combinado los diferentes métodos de tratamiento con el fin de obtener resultados más rápidos y efectivos.

Pulmonary Alveolar Proteinosis (PAP) is a rare disease characterized by the accumulation of surfactant derived lipoproteinaceous material filling the alveoli, secondary to failure of its clearance by macrophages. Most of the patients are adults that have auto antibodies directed to Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF). The evolution is towards disturbed gaseous exchange with a wide spectrum of disease from spontaneous recovery to death. There are three forms of PAP: genetic, secondary and autoimmune. Symptoms are scarce and patients may present with dyspnea, dry cough and hypoxemia. Chest X ray shows bilateral opacities and thorax CT depicts ground glass opacities surrounded by septal widening, the so called "crazy paving" pattern. Diagnosis is made on clinical and radiological grounds and confirmed by PAS positive staining of bronchoalveolar lavage material or surgical lung biopsy. Accepted treatment is whole lung lavage (WLL) with saline. Alternatives are subcutaneous or inhaled GM-CSF, Plasmapheresis or Rituximab, and even modification of the method of WLL and combination of different manner of treatment.

Characterization and outcomes of pulmonary alveolar proteinosis in Brazil: a case series / Proteinose alveolar pulmonar: caracterização e desfechos em uma série de casos no Brasil

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.

Secondary pulmonary alveolar proteinosis in hematologic malignancies

Pulmonary alveolar proteinosis [PAP], characterized by deposition of intra-alveolar PAS positive protein and lipid rich material, is a rare cause of progressive respiratory failure first described by Rosen et al. in 1958. The intra-alveolar lipoproteinaceous material was subsequently proven to have been derived from pulmonary surfactant in 1980 by Singh et al. Levinson et al. also reported in 1958 the case of 19-year-old female with panmyelosis afflicted with a diffuse pulmonary disease characterized by filling of the alveoli with amorphous material described as "intra-alveolar coagulum". This is probably the first reported case of PAP in relation to hematologic malignancy. Much progress has been made on PAP first described by Rosen which is currently classified as idiopathic or primary or autoimmune PAP. Idiopathic PAP occurs as a result of auto-antibodies directed against granulocyte-macrophage colony stimulating factor [GM-CSF] impeding the surfactant clearing function of alveolar macrophages leading to progressive respiratory failure. Whole lung lavage and GM-CSF therapy has improved outcomes in patients with idiopathic PAP. Despite major advancement in the management of hematologic malignancy and its complications, little is known about the type of PAP first described by Levinson and now known as secondary PAP; a term also used when PAP occurs due to other causes such as occupational dusts. In this article we review and analyze the limited literature available in secondary PAP due to hematologic malignancies and present a case of PAP associated with chronic lymphocytic leukemia successfully treated with bendamustine and rituximab

Proteinosis alveolar: reporte de caso y revisión de literatura / Alveolar proteinosis: case report and literature review

La proteinosis alveolar es una rara enfermedad, sobre la que se han ido conociendo progresivamente sus características y su etiopatología, lo que ha permitido tener un poco más de claridad sobre ella. En general son pocos los casos reportados en el mundo. Por este motivo es de suma importancia realizar el reporte de caso y revisar la literatura actual, donde se encontraron nuevas opciones terapéuticas con resultados adecuados que permitieron manejar a los pacientes sin respuesta al tratamiento convencional. Es necesario considerar que el 25 por ciento de los casos tiene resolución espontánea, pero otro alto porcentaje requiere tratamiento convencional con lavado pulmonar total que muchas veces no mejora el pronóstico de la enfermedad. El caso reportado se asoció a diabetes y a infección por Mycobacterium tuberculosis, por lo que se puede considerar que el diagnóstico de proteinosis alveolar primaria presenta en este tipo de pacientes una relación directa con las patologías asociadas.

Alveolar proteinosis is a rare disease. Its etiology, pathology, and manifestations are becoming increasingly more apparent. Few cases have been reported, which highlights the importance of reporting this case and reviewing current literature. New therapeutic options are being introduced with adequate outcomes thereby making it possible to manage patients who were previously unresponsive to conventional treatment. 25 percent of cases resolve spontaneously, while most patients require conventional treatment with total lung lavage that often does not improve prognosis. The case reported in this article was associated to Mycobacterium tuberculosis infection and diabetes. Primary alveolar proteinosis can have a direct relationship to these concomitant conditions.

Procedimento e complicações anestésicas no manejo de lavagem pulmonar total em paciente obeso com proteinose alveolar pulmonar: relato de caso / Anaesthetic, procedure and complications management of serial whole lung lavage in an obese patient with pulmonary alveolar proteinosis: case report / Procedimiento y complicaciones anestésicas en el manejo del lavado pulmonar total en paciente obeso con proteinosis alveolar pulmonar: relato de caso

JUSTIFICATIVA E OBJETIVOS: O primeiro caso de proteinose alveolar pulmonar (PAP) foi descrito por Rose em 1958, mas ainda é um distúrbio raro. PAP é caracterizada pela deposição de material lipoproteico secundário ao processamento anormal de surfactantes pelos macrófagos. Os pacientes podem ter dispneia progressiva e tosse, às vezes acompanhadas pelo agravamento da hipóxia, e seu curso pode variar de deterioração progressiva a melhora espontânea. Muitas terapias foram usadas, incluindo antibióticos, drenagem postural e ventilação com pressão positiva intermitente com acetilcisteína, heparina e soro fisiológico em aerossol. Atualmente, a base do tratamento é a lavagem pulmonar total (LPT). A LPT, embora seja geralmente bem-tolerada, pode estar associada a algumas complicações. RELATO DE CASO: Relatamos um caso de PAP grave durante o procedimento anestésico e as complicações no manejo da proteinose alveolar pulmonar em um paciente que havia sido submetido a múltiplas e alternadas lavagens de um dos pulmões ao longo de sete anos (os últimos três em nosso hospital), com melhora dos sintomas depois de cada tratamento.

BACKGROUND AND OBJECTIVES: The first case of Pulmonary Alveolar Proteinosis (PAP) was described by Rose in 1958, but it is still a rare disorder. PAP is characterized by deposition of lipoproteinaceous material secondary to abnormal processing of surfactant by macrophages. Patients may suffer from progressive dyspnea and cough that at times is accompanied by worsening hypoxia and its course can vary from progressive deterioration to spontaneous improvement. Many therapies have been used to treat PAP including antibiotics, postural drainage, and intermittent positive pressure breathing with aerosolized Acetylcysteine, heparin and saline. At present, the mainstay of treatment is whole lung lavage (WLL). Although generally well tolerated, WLL can be associated with some complications. CASE REPORT: We report a case of severe PAP through the anaesthetic, procedure and complications management of pulmonary alveolar proteinosis in one patient who has undergone multiple, alternating, single-lung lavages over the past seven years, the last three in our hospital, with improvements in her symptoms following each therapy.

JUSTIFICATIVA Y OBJETIVOS: El primer caso de proteinosis alveolar pulmonar (PAP) fue descrito por Rose en 1958, pero todavía continúa siendo un trastorno raro. La PAP se caracteriza por la deposición de material lipoproteico secundario al procesamiento anormal de surfactantes por los macrófagos. Los pacientes pueden tener disnea progresiva y tos, a veces seguidas de un agravamiento de la hipoxia, y su curso puede variar de deterioración progresiva a una mejoría espontánea. A lo largo de los años, muchas terapias han sido usadas para tratar la PAP, incluyendo antibióticos, drenaje postural y ventilación con presión positiva intermitente con acetilcisteína, heparina y suero fisiológico en aerosol. Hoy por hoy, la base del tratamiento es el lavado pulmonar total (LPT), descrito por primera vez por Ramirez-Rivera y col. Existen tres variantes de la enfermedad: congénita, secundaria y adquirida. Las recientes investigaciones sugieren que en la forma más común (PAP adquirida [idiopática]), la autoinmunidad contra el factor estimulador de las colonias de granulocitos y macrófagos (FEC-GM) pulmonares desempeña un importante papel. El factor recombinante que estimula las colonias de granulocitos y macrófagos parece beneficiarse con un subconjunto de pacientes adultos con PAP y puede representar una alternativa en el tratamiento de la enfermedad, pero la tasa de éxito todavía no es suficiente para substituir la LPT. La LPT, aunque sea generalmente bien tolerada, puede estar asociada con algunas complicaciones. RELATO DE CASO: Relatamos un caso de PAP grave durante el procedimiento anestésico y las complicaciones en el manejo de la proteinosis alveolar pulmonar en un paciente que había sido sometido a múltiples y alternados lavados de uno de los pulmones a lo largo de siete años (los últimos tres en nuestro hospital), con una mejoría de los síntomas después de cada tratamiento.

Experience with treatment of pulmonary alveolar proteinosis from a tertiary care centre in north India.

Background. Pulmonary alveolar proteinosis (PAP) is a disorder characterised by accumulation of lipids and proteins in the alveoli, with the resultant symptoms ranging from indolent subclinical disease to progressive respiratory failure. Methods. We retrospectively studied five patients with PAP managed at our center between January 2007 and April 2010, with whole lung lavage (WLL) and/or subcutaneous granulocyte macrophage-colony stimulating factor (GM-CSF) therapy. Patients undergoing WLL under general anaesthesia were supplemented with three months of GM-CSF therapy. Pre- and post-lavage symptom assessment was performed with a 10-point, symptom-based visual analogue scale. Results. Their mean age was 37.6±7.0 years; there were four males. Diagnosis of PAP [idiopathic (n=3); secondary to Nocardia (n=1)] was established by surgical lung biopsy in four patients who presented with respiratory failure. Three patients with idiopathic PAP (n=3) were treated with a combination of GM-CSF and WLL; one patient with secondary PAP was treated with antibiotics alone. In another patient transbronchial lung biopsy was used to diagnose PAP and GM-CSF alone was administered. All patients were followed up for a median period of two years (range 0.5-3 years). Significant improvement was achieved in all the patients with therapeutic WLL and/or GM-CSF. Conclusions. Whole lung lavage appeared to be an effective and safe therapy in patients with PAP. Efficacy of simultaneous administration of GM-CSF and WLL in the treatment of PAP merits further study.

Anestesia para lavagem pulmonar em paciente pediátrico portador de proteinose alveolar pulmonar / Anesthesia for lung lavage in pediatric patient with pulmonary alveolar proteinosis / Anestesia para el lavado pulmonar en paciente pediátrico portador de proteinosis alveolar pulmonar

JUSTIFICATIVA E OBJETIVOS: A proteinose alveolar pulmonar (PAP) é um transtorno raro, descrito inicialmente em 1958. A lavagem pulmonar total (LPT), proposta na década de 1960 ainda é o tratamento de escolha. Diversas técnicas foram descritas para realizar a lavagem pulmonar em pediatria, no entanto, todas apresentam limitações e riscos. RELATO DO CASO: Paciente de 6 anos e 8 meses, sexo feminino, 25 kg com o diagnóstico de proteinoise alveolar pulmonar submetida a lavagem pulmonar total por fibrobroncoscopia lobar sequencial sob anestesia geral em ventilação espontânea.

BACKGROUND AND OBJECTIVES: Pulmonary Alveolar Proteinosis (PAP) is a rare disorder first described in 1958. The Whole-Lung Lavage (WLL) proposed in the 1960s, remains the treatment of choice. Several techniques have been described to perform lung lavage in pediatric patients; however, all have limitation and risks. CASE REPORT: Female patient, aged 6 years and 8 months, 25 kg, diagnosed with pulmonary alveolar proteinoisis, who underwent whole-lung lavage by sequential lobar fiberoptic bronchoscopy under general anesthesia and spontaneous ventilation.

JUSTIFICATIVA Y OBJETIVOS: La proteinosis alveolar pulmonar (PAP) es un transtorno raro, descrito inicialmente en 1958. El lavado pulmonar total (LPT), propuesto en la década de 1960, es actualmente el tratamiento de elección. Diversas técnicas fueron descritas para realizar el lavado pulmonar en pediatría pero sin embargo, todas tienen limitaciones y riesgos. RELATO DEL CASO: Paciente de seis años y ocho meses, del sexo femenino, 25 kg con el diagnóstico de proteinosis alveolar pulmonar, que fue sometida al lavado pulmonar total por fibrobroncoscopia lobar secuencial bajo anestesia general en ventilación espontánea.

Continuous positive airway pressure ventilation during whole lung lavage for treatment of alveolar proteinosis -A case report and review of literature

Pulmonary alveolar proteinosis [PAP] is a rare disease that affects young population usually in the age group of 20-40 years, characterized by the deposition of lipoproteinacious material in the alveoli secondary to abnormal processing of surfactant by macrophages. We report a case of a 15-year-old female who had history of cough with sputum for 3 days along with fever. She was seen in another hospital and was treated as a case of pneumonia where she received antibiotic but with no improvement. Computerized tomography [CT] chest showed diffuse interlobular septal thickening in the background of ground glass opacity giving a picture of crazy paving pattern which was consistent with the diagnosis of PAP. The patient was scheduled to undergo, first right-sided whole lung lavage [WLL] under general anesthesia. Endobronchial intubation using left sided 37 Fr double lumen tube. Continuous positive airway pressure [CPAP] as described in our previously published report was connected to the right lumen of the endobronchial tube. CPAP ventilation was used during the suctioning of lavage fluid phase in order to improve oxygenation. WLL was done using 5 L of warm heparinized saline [500 i.u/litre]. The same procedure was repeated on the left side using 6 L of heparinized normal saline solution. In conclusion, anesthesia in alveolar proteinosis for patients undergoing WLL is challenging to the anesthesiologist. It requires meticulous preoperative preparation with antibiotics, mucolytics and chest physiotherapy. Also it requires careful intraoperative monitoring and proper oxygenation especially during the suctioning phase of the lavaged fluid. With this second case report of successful anesthetic management using the modified CPAP system we recommend with confidence the application of CPAP ventilation to improve oxygenation during WLL

Pulmonary alveolar proteinosis.

Pulmonary alveolar proteinosis is a rare cause of respiratory distress in neonates. We present a 4-month-old infant who presented with progressive respiratory distress since birth and failure to thrive. He was initially treated as a case of diffuse alveolar disease but on open lung biopsy was diagnosed as pulmonary alveolar proteinosis. The child expired at 7 months of age.

Disnea progresiva e infiltrados pulmonares alveolares: acerca de un caso de Proteinosis Alveolar Pulmonar / Progresive Dyspnea, alveolar pulmonar infiltrates about a case of alveolar proteinosis

La evaluación de un paciente con síntomas respiratorios subagudos o crónicos como tos y disnea y con presencia de infiltrados pulmonares difusos requiere un protocolo de investigación para establecer su etiología y tratamiento específico. Se presenta un caso poco frecuente de paciente con enfermedad difusa del parénquima pulmonar, en quien por una serie de exámenes, incluyendo baciloscopías seriadas, radiografía de tórax, tomografía axial computarizada de alta resolución de tórax, espirometría, broncoscopía y biopsia por toracotomía abierta se diagnosticó Proteinosis Alveolar. El paciente ha tenido buena respuesta a los lavados pulmonares....

Whole lung lavage: the salvage therapy for pulmonary alveolar proteinosis.

A 53-year-old school teacher presented with progressive exertional breathlessness and dry cough of three months duration. His diagnosis was confirmed as pulmonary alveolar proteinosis on open lung biopsy. In about three months, the disease progressed to life threatening respiratory failure. He was subjected to whole lung lavage (WLL) as a salvage therapy. The technical details of WLL performed on this patient are described. At six months follow up, he was clinically and functionally stable and leading a near normal life.

Proteinose alveolar pulmonar: série de quatro casos / Pulmonary alveolar proteinosis: four cases

OBJETIVO: Apresentar a evolução de quatro casos de proteinose alveolar pulmonar atendidos na Faculdade de Medicina da Universidade Estadual de Londrina, enfocando a importância da lavagem pulmonar total como tratamento de escolha. MÉTODOS: Trata-se de um estudo retrospectivo de quatro pacientes, sendo três do gênero feminino, com idades de 22 a 34 anos, e histórias semelhantes de dispnéia progressiva e tosse seca. O diagnóstico final foi realizado por biópsia pulmonar a céu aberto. A lavagem pulmonar total foi realizada em três pacientes em centro cirúrgico, com anestesia geral e sonda de duplo lúme. RESULTADOS: Um paciente apresentou regressão espontânea da proteinose alveolar pulmonar, não sendo necessária a lavagem pulmonar. Nos outros três casos, o número de lavagens variou: uma única lavagem unilateral com remissão completa do quadro bilateralmente, três lavagens sem melhora significativa e quatro procedimentos intercalados com períodos de melhora. CONCLUSÃO: Constatamos em nossa casuística que a lavagem pulmonar se mostrou eficiente, apesar de alguns pacientes apresentarem certa resistência ao procedimento, enquanto que outros podem ter remissão completa da doença.

OBJECTIVE: The aim of this study was to present the evolution of four patients presenting pulmonary alveolar proteinosis and treated at the State University of Londrina School of Medicine. We focus on the importance of whole-lung lavage as the treatment of choice. METHODS: A retrospective study of four patients, three females and one male, 22 to 34 years old, presenting similar histories of progressive dyspnea and dry cough. The final diagnosis was established through open-lung biopsy. Three of the patients underwent whole-lung lavage in the Department of Surgery. The procedures were performed under general anesthesia and using a double-lumen endotracheal tube. RESULTS: One patient presented spontaneous regression of the pulmonary alveolar proteinosis without the need for whole-lung lavage. In the other three cases, the number of lavages varied: in one patient, a single unilateral lavage resulted in complete remission of the bilateral process; in another patient, three lavages yielded no significant improvement; in the remaining patient, four lavages provided intervening periods of transient improvement. CONCLUSION: In the cases evaluated, whole-lung lavage proved an efficient treatment for pulmonary alveolar proteinosis. Although some patients presented a certain resistance to the procedure, it might lead to complete remission of the disease in others.

Fosfolipoproteinosis alveolar (10 casos 12 años de experiencia) / Alveolar phospholipoproteinosis (10 cases 12 years of experience)

Por tratarse de una entidad poco frecuente, 12 años (septiembre de 1991 y septiembre de 2003) han sido necesarios en nuestra institución para diagnosticar, 10 pacientes con Proteinosis alveolar (PA), y realizar un total de 24 lavados pulmonares totales y como parte de la terapéutica en estos pacientes. De este grupo de pacientes el más joven un varón de 26 años de edad y un solo paciente corresponde al sexo femenino siendo del grupo de pacientes la que más tardíamente manifiesta la existencia de su enfermedad (57 años). Esta entidad obedece a la acumulación de fosfolípidos a nivel de las unidades respiratorias del pulmón, que inundan las mismas, llevando a un compromiso progresivo de la transferencia gaseosa por bloqueo alveolo-capilar, alteración de la mecánica ventilatoria, compromiso del retroceso elástico y distensibilidad pulmonar, situaciones que con el tiempo, llevan al paciente a insuficiencia respiratoria aguda, llegando a comprometer su vida. En esta publicación comentaremos nuestra experiencia con 10 pacientes con diagnóstico confirmado de proteinosis alveolar, y haremos una revisión de las experiencias e investigaciones recientemente publicadas en la literatura médica mundial.

Proteinosis alveolar pulmonar / lung alveolar proteinosis

El primer caso de proteinosis alveolar pulmonar (PAP) fue reportado en 1958 por Rosen, es una rara enfermedad caracterizada por la acumulación de material proteinaceo dentro de alvéolo. Tal como sucede con muchas enfermedades infrecuentes, la experiencia acumulada con PAP es diseminada, con únicamente 139 pacientes en la serie más grande que ha sido reportada, a nuestro conocimiento 332 casos reportados en la literatura médica. La edad promedio es 42 años (rango de 21 a 67 años), la mayoría son varones, fumadores, no conocida relación con HIV. Algunas veces asociado con enfermedades sistémicas como cáncer de mama, mielodisplasia, enfermedad del pericardio, enfermedad inflamatoria del intestino. Los síntomas más comunes al momento del diagnóstico fue la dísnea y tos (79 por ciento). El examen físico reveló crepitantes en 50 por ciento, sibilantes en 25 por ciento, y cianosis en 21 por ciento. La Rx tórax mostró un patrón acinar en la mayoría de pacientes (62.5 por ciento), este infiltrado fue difuso con distribución perihiliar. La espirometria y volúmenes pulmonares reveló leve defecto ventilatorio restrictivo con desproporcionada disminución de la capacidad de difusión de CO. Levemente hipoxémicos con gradiente aumentada. DHL levemente elevada. Un temprano reporte de Summers sobre 93 pacientes sugiere una mortalidad de 39.7 por ciento debido a insuficiencia respiratoria hipóxica o enfermedades complicantes durante un período de 17 años. En contraste, recientes experiencias de ambos la Clínica Mayo y de Japón sugieren una mortalidad de 8.8 por ciento y 0 por ciento respectivamente. Hay también incertidumbre acerca de los métodos de diagnóstico y tratamiento para PAPA. En tempranas series de Davidson y Macleod el diagnóstico por toracotomía o por trocar de biopsia; mientras la más reciente experiencia de asamoto reporta suceso diagnóstico por broncoscopía en 89.7 por ciento de 68 casos. El rol de la broncoscopía en el establecimiento del diagnóstico es controversial. Lavado pulmonar total fue necesario en el manejo médico por un período de 8.4 años (rango de 5 meses a 21 años); siendo su indicación más importante para lavado pulmonar total la dísnea y la hipoxemia. En promedio el lavado fue realizado 3.3 veces por paciente (2 a 8). El volumen promedio de cada lavado es 11.8 mas/menos 3.68 L...

The clinical characteristics of pulmonary alveolar proteinosis: experience at Seoul National University Hospital, and review of the literature

Pulmonary alveolar proteinosis is such an extremely rare disease in Korea, that only a few cases have been reported. Meanwhile five cases were experienced at Seoul National University Hospital over ten years since 1987. We summarized the clinical characteristics and courses of them. Seven cases reported in the literature were included to add data about clinical characteristics and courses although only a few case reports mentioned patient's course. Middle aged male patients were mainly affected. No association with particular environmental or occupational exposure was identified. Dyspnea on exertion was the main symptom. Bilateral crackles were consistent, and bilateral parahilar hazy infiltrations on plain chest radiograph and ground glass opacity on high-resolution CT were characteristic. Superimposed infection was not identified in any patient at the time of diagnosis. Decreased diffusing capacity and hypoxia were present in almost every case. Whole lung lavage proved to be an effective therapeutic measure. The response to treatment was good. Long-term course of the disease, e.g. recurrence rate, is not yet known.