Enfermedades pulmonares intersticiales. Una perspectiva actual / Update on interstitial lung diseases

Interstitial lung diseases are a broad, diverse, challenging group of diseases, most of them chronic whose prognosis is not good. In the last two decades there have been considerable advances in the knowledge of the epidemiology, pathological and genetic bases and treatment of several of these diseases. This article summarizes and presents updated information about their classification, new knowledge on genetics and treatments in idiopathic pulmonary fibrosis, advances in the diagnosis and management of hypersensitivity pneumonitis and a review of the broad spectrum of interstitial diseases associated with connective tissue diseases. Several clinical trials are currently underway whose results will be available in the coming years and will provide more information and tools to improve the treatment of these patients.

Utilidad de la crio-tecnología para uso diagnóstico y terapéutico en neumología intervencionista: crio-biopsia pulmonar transbronquial y crioterapia / Transbronchial cryobiopsies and cryotherapy in lung diseases

Bronchoscopy cryoprobes are used for palliative treatment of endobronchial obstructions caused by tumors and removal of granulation tissue or foreign bodies. Currently this technology is also used for diagnosis of diffuse interstitial lung disease (ILD). The multidisciplinary team that establishes the clinical, radiological and histopathological correlation in ILD, decides about performing a surgical lung biopsy when the characteristics of the interstitial disease are not similar to Idiopathic Pulmonary Fibrosis (IPF). Although surgical lung biopsy is the gold standard for diagnosis, treatment, and prognosis, transbronchial cryo-biopsy has a high diagnostic yield, low morbidity and mortality rate, low rate of complications and lower cost. It is the diagnostic method of choice in ILD when it is available. Technological improvements with greater freezing power and tensile strength of the cryo probes, allow their use in cryotherapy and cryo-recanalization for occlusive airway tumors.

Aproximación diagnóstica a las enfermedades pulmonares difusas / Diagnostic approach to diffuse pulmonary diseases

Las enfermedades pulmonares difusas representan un grupo de enfermedades que comparten un mismo criterio radiológico, existiendo más de 200 entidades que se presentan como tal. La clínica es fundamental para aproximar el diagnóstico etiológico que muchas veces resulta complejo. Tos y disnea progresiva son los síntomas clínicos característicos de estas enfermedades y se acompañan de la radiografía de tórax con opacidades difusas como método radiológico inicial. El estudio en general es multi- disciplinario incluyendo patrones radiológicos de la tomografía axial de tórax, estudio de función pulmonar, lavado bronquioal- veolar y biopsia pulmonar en algunos casos.

The diffuse lung diseases are a group of conditions that share common radiological criteria. There are over 200 causes. The clinic skill is essential to approximate the etiologic diagnosis, often complicated. Cough and progressive dyspnea are the clinical features of these diseases and are accompanied by chest radiography with diffuse opacities as the initial radiological method. The study is generally multidisciplinary and including radiological patterns in computer tomography of the chest, lung function study, bronchoalveolar lavage and lung biopsy in some cases.

Enfermedad pulmonar intersticial del lactante / Interstitial lung disease in infants

Interstitial lung disease is an heterogeneous group of illnesses. Some of them only affect children under two years old and new classifications have been proposed for this age group. Proper clinical awareness, X-ray studies like thorax high-resolution computed tomography, and lung biopsy are required for diagnosis. Clinically, there is tachipnea, intercostal retractions, rales, interstitial images at X-ray, hypoxemia and restrictive lung disease. Steroids are the most used treatment; in severe cases lung transplantation might be the sole option. Some of them presents a good prognosis; for others it is often fatal when it appears in the neonatal period.

La enfermedad pulmonar intersticial (EPI) constituye un grupo heterogéneo de patologías. Algunas son exclusivas de niños menores de 2 años, para los cuales se ha propuesto un nuevo esquema de clasificación. El diagnóstico requiere de sospecha clínica, estudio radiológico, y en general biopsia pulmonar. Hay presencia de taquipnea, retracciones, crepitaciones, hipoxemia, infiltrados intersticiales y enfermedad restrictiva. El tratamiento de elección son los corticoides, en casos severos el trasplante. Algunas presentan buen pronóstico, otras iniciadas en período neonatal tienen a menudo una evolución fatal.

Interpretation of autoantibody positivity in interstitial lung disease and lung-dominant connective tissue disease / Interpretacao da positividade de autoanticorpos na doenca pulmonar intersticial e colagenose pulmao dominante

The initial evaluation of patients with interstitial lung disease (ILD) primarily involves a comprehensive, active search for the cause. Autoantibody assays, which can suggest the presence of a rheumatic disease, are routinely performed at various referral centers. When interstitial lung involvement is the condition that allows the definitive diagnosis of connective tissue disease and the classical criteria are met, there is little debate. However, there is still debate regarding the significance, relevance, specificity, and pathophysiological role of autoimmunity in patients with predominant pulmonary involvement and only mild symptoms or formes frustes of connective tissue disease. The purpose of this article was to review the current knowledge of autoantibody positivity and to discuss its possible interpretations in patients with ILD and without clear etiologic associations, as well as to enhance the understanding of the natural history of an allegedly new disease and to describe the possible prognostic implications. We also discuss the proposition of a new term to be used in the classification of ILDs: lung-dominant connective tissue disease.

A avaliação inicial de pacientes com doença pulmonar intersticial (DPI) envolve primordialmente a busca ativa e detalhada por uma etiologia. A pesquisa rotineira de autoanticorpos é comum em diferentes centros e permite sugerir a presença de alguma doença do espectro reumatológico. Quando o acometimento pulmonar intersticial é a condição que permite o diagnóstico firmado de uma colagenose bem estabelecida, preenchendo os critérios clássicos, há pouco debate. Entretanto, ainda existe muita discussão sobre o significado, a relevância, a especificidade e o papel fisiopatológico da autoimunidade nos pacientes que tenham prioritariamente acometimento respiratório e apenas algum indício leve ou frustro de colagenose. O propósito dessa revisão foi apresentar o conhecimento atual e discutir possibilidades de interpretação da positividade de autoanticorpos em pacientes com DPI que não tenham associações etiológicas inequívocas, assim como aumentar o entendimento da história natural de uma possível nova doença e descrever possíveis implicações prognósticas. Discutimos ainda a proposição de uma nova terminologia na classificação das DPIs, a colagenose pulmão dominante.

Classificação das pneumonias intersticiais idiopáticas: perspectivas para os próximos dez anos / Classification of idiopathic interstitial pneumonias: the next ten years

A classificação das pneumonias intersticiais idiopáticas pela American Thoracic Society/European Respiratory Society em 2002 incluiu sete entidades clínico-patológicas: fibrose pulmonar idiopática, pneumonia intersticial não específica, pneumonia em organização criptogênica, pneumonia intersticial aguda, bronquiolite respiratória associada a doença pulmonar intersticial, pneumonia intersticial descamativa e pneumonia intersticial linfoide. Todavia, em 2002, muitas áreas de incerteza foram geradas, incluindo a exacerbação aguda da fibrose pulmonar idiopática, a pneumonia intersticial não específica, diretrizes baseadas em evidências para o diagnóstico e gerenciamento da fibrose pulmonar idiopática, assim como as doenças com padrão de fibrose intersticial associada ao tabaco. O objetivo da presente revisão foi propor uma revisão dessa classificação para os próximos dez anos, incluindo o diagnóstico clínico, radiológico e patológico da pneumonia intersticial usual/fibrose pulmonar idiopática; a exacerbação aguda da fibrose pulmonar idiopática, assim como de pneumonia intersticial não específica, doenças pulmonares intersticiais associadas ao tabaco, pneumonia em organização criptogênica e pneumonia intersticial aguda; pneumonias intersticiais idiopáticas raras, como pneumonia intersticial linfoide idiopática e fibroelastose pleuropulmonar idiopática limitada ao lobo superior; padrões histológicos raros, como pneumonia em organização aguda fibrinosa e padrões bronquiolocêntricos das pneumonias intersticiais idiopáticas; e pneumonias intersticiais idiopáticas genéticas, como as pneumonias intersticiais idiopáticas familiares (herdadas) e fibrose pulmonar associada a síndromes hereditárias.

Destaques das diretrizes de doenças pulmonares intersticiais da Sociedade Brasileira de Pneumologia e Tisiologia / Highlights of the Brazilian Thoracic Association guidelines for interstitial lung diseases

As doenças pulmonares intersticiais (DPIs) são afecções heterogêneas, envolvendo um elevado número de condições, cuja abordagem ainda é um grande desafio para o pneumologista. As Diretrizes de DPIs da Sociedade Brasileira de Pneumologia e Tisiologia, publicadas em 2012, foram estabelecidas com o intuito de fornecer aos pneumologistas brasileiros um instrumento que possa facilitar a abordagem dos pacientes com DPIs, padronizando-se os critérios utilizados para a definição diagnóstica das diferentes condições, além de orientar sobre o melhor tratamento nas diferentes situações. Esse artigo teve como objetivo descrever resumidamente os principais destaques dessas diretrizes.

Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.

Doenças pulmonares interticiais: linfangioliomiomatose em questão / Intertitial pulmonary diseases: lymphangioleiomyomatosis in question

As doenças pulmonares intersticiais (DPIs) são conceituadas como distúrbios que acometem o parênquima pulmonar - o endotélio cailar, os alvéolos, o epitélio alveolar e os espaços entreestas estruturas, bem como os tecidos perivasculares e linfáticos - , podendo ser classificadas segundo critérios histopatológicos, distinguindo-se dois grandes grupos: 1. das associadas à inflamação e fibrose; e 2. daquelas com reação granulomatosa predominante na área intersticial u vasculas. A linfangioliomiomatose (LAM) é uma rara DPI, idiópática, e com altas taxas de morbimortalidade, sendo caracterizada por uma multiplicação acelerada de células musculares lisas imaturas em qualquer estrutura pulmonar. No presente artigo apresentar-se-á uma revisão da literatura enfocando a etiopatogenia, a epidemiologia, o quadro clínico, o diagnóstico - procedimentos, critérios e diagnóstico diferencial - o tratamento e o prognóstico da LAM.

The interstitial pulmonary diseases (IPDs) are a range of disorders that affect the pulmonary parenchyma - the capillary endothelium, alveoli, alveolar epithelium and the spaces between thesestructures, as well as the perivascular and lymphatic tissues. The IPDs may be classified according to histopathologic criteria, and are divided into two large groups: 1. those associated with inflammation and fibrosis; and 2. those associated with granulomatous reactions predominantly in the interstitial or vascular area. Lymphangioleiomyomatosis (LAM) ia a rare, idiopathic IPD with high morbimortality rates, which is characterized by an accelerated multiplication of immature smooth muscle cellsin any pulmonary structure. In this article, we present a review of the literature onthe etiopathogenesis, epidemiology, clinical picture, diagnosis and differential diagnosos, treatment, and prognosis of this condition.

Doenças pulmonares intersticiais crônicas na criança: [revisão] / Chronic interstitial lung diseases in children: [review]

As doenças pulmonares intersticiais (DPIs) da criança constituem um grupo heterogêneo de doenças raras que têm sido definidas e classificadas de acordo com as experiências e as pesquisas em adultos. Entretanto, os pneumologistas pediátricos vêm observando que o espectro clínico é mais amplo nas crianças, e que muitas destas doenças evoluem e respondem ao tratamento de forma diferente. Além disso, provavelmente devido a estágios diferentes de desenvolvimento e maturação pulmonares, novas formas clínicas têm sido descritas, principalmente em lactentes, ampliando a classificação nessa faixa etária. A compreensão de que nem a definição nem as classificações estabelecidas se aplicam inteiramente ao grupo pediátrico tem motivado a realização de estudos multicêntricos com o objetivo de estudá-las melhor, unificando as estratégias diagnósticas e terapêuticas. Fizemos a revisão atualizando a conceituação das DPIs no grupo pediátrico, considerando as particularidades desse grupo na utilização do esquema de classificação dessas doenças para adultos e revendo o histórico dos esforços para uma melhor compreensão do problema com os estudos multicêntricos. Foram ressaltadas as diferenças na apresentação clínica, procurando realçar os novos conhecimentos sobre as doenças recém descritas nas crianças pequenas. Alertamos também para a necessidade de ser seguida uma rotina padronizada de investigação laboratorial, radiológica e de processamento das biópsias à luz desses conhecimentos. É importante lembrar que, do grupo das novas doenças descritas, as alterações genéticas do surfactante devem constar também do diagnóstico diferencial das DPIs dos adultos, podendo se apresentar nesse grupo como uma das doenças classificadas como idiopáticas.

Interstitial lung diseases (ILDs) in children constitute a heterogeneous group of rare diseases that have been described and classified according to experiences and research in adults. However, pediatric pulmonologists have observed that the clinical spectrum is broader in children than in adults, and that many of these disorders have different courses and treatment responses. In addition, probably due to the various stages of lung development and maturation, new clinical forms have been described, particularly in infants. This has broadened the classification of ILDs in this age bracket. The understanding that neither the usual definition nor the standard classification of these disorders entirely apply to children has prompted multicenter studies designed to increase knowledge of these disorders, as well as to standardize diagnostic and therapeutic strategies. We have reviewed the conceptualization of ILDs in children, taking into consideration the particularities of this group of patients when using the criteria for the classification of these diseases in adults. We have also made a historical review of several multicenter studies in order to further understanding of the problem. We have emphasized the differences in the clinical presentation, in an attempt to highlight knowledge of newly described entities in young children. We underscore the need to standardize management of laboratory and radiological routines, as well as of lung biopsy processing, taking such knowledge into account. It is important to bear in mind that, among the recently described disorders, genetic surfactant dysfunction, which is often classified as an idiopathic disease in adults, should be included in the differential diagnosis of ILDs.

Análise de 39 casos de pneumonia intersticial crônica idiopática / Analysis of 39 cases of idiopathic chronic interstitial pneumonia

OBJETIVO: Analisar de forma retrospectiva fragmentos de biópsias pulmonares que receberam o diagnóstico de pneumonia intersticial crônica idiopática, à luz da classificação da American Thoracic Society e European Respiratory Society, de 2000. MÉTODOS: A partir da revisão de 252 fragmentos de biópsias pulmonares a céu aberto de pacientes com doença intersticial pulmonar, no período de 1977 a 1999, 39 casos de doença pulmonar intersticial idiopática foram selecionados e reavaliados por dois patologistas, segundo a classificação da American Thoracic Society e European Respiratory Society, de 2000. RESULTADOS: Vinte e oito dos 39 diagnósticos foram mantidos (71,8 por cento). Uma nova entidade patológica, a pneumonia intersticial não específica, foi incluída na reclassificação e houve superposição de padrões em seis casos. Mantiveram o mesmo diagnóstico 28 casos, 4 casos apresentaram associação entre fibrose pulmonar idiopática e organização pneumônica criptogênica, 1 entre organização pneumônica criptogênica e pneumonia intersticial não específica, e 1 entre pneumonia intersticial descamativa e pneumonia intersticial não específica. Todos os casos de fibrose pulmonar idiopática foram confirmados, embora 3 deles estivessem associados a organização pneumônica criptogênica. Os diagnósticos anteriores foram quase todos mantidos na revisão dos espécimes (p > 0,05). CONCLUSÃO: A classificação das doenças pulmonares intersticiais da American Thoracic Society e European Respiratory Society é uma ferramenta útil aos patologistas que lidam com biópsias pulmonares.

OBJECTIVE: To make a retrospective analysis of lung biopsy samples obtained from patients diagnosed with chronic idiopathic interstitial pneumonia, as defined in the American Thoracic Society/European Respiratory Society classification system made public in 2000. METHODS: Samples from 252 open-lung biopsies of patients with interstitial lung disease, all performed between 1977 and 1999, were reviewed, and 39 cases of idiopathic interstitial lung disease were selected and re-evaluated by two pathologists in accordance with the American Thoracic Society/European Respiratory Society classification system. RESULTS: Among those 39 cases, the diagnoses were maintained in 28 (71.8 percent). A new pathologic entity, nonspecific interstitial pneumonia, was included in the reclassification, and overlapping patterns were observed in 6 cases. Of the 28 cases in which the diagnosis of chronic idiopathic interstitial pneumonia remained unchanged, idiopathic pulmonary fibrosis was accompanied by cryptogenic organizing pneumonia in 4, cryptogenic organizing pneumonia was accompanied by nonspecific interstitial pneumonia in 1, and desquamative interstitial pneumonia was accompanied by nonspecific interstitial pneumonia in 1. All cases of idiopathic pulmonary fibrosis were confirmed, although 3 of those were found to be accompanied by cryptogenic organizing pneumonia. Virtually all prior diagnoses were maintained in the review of the biopsy samples (p > 0,05). CONCLUSION: The American Thoracic Society/European Respiratory Society system of classifying interstitial lung disease is a useful tool for pathologists who deal with lung biopsies.

Neumatosis quistica intestinal: a proposito de un caso / Intestinal cystic pnematosis

La neumatosis quística intestinal es una rara patología en nifio que se caracteriza por presencia de múltiples quistes de gas, las que pueden causar neumoperitoneo importante por ruptara de los quistes e por perforación intestinal. Es una de las causas de neumoperitonec benigno. Se reporta un paciente de 12 años de edad con neumatosis quística intestinal asociado a mal rotación intestinal, donde se realizc una laparotomía exploradora por neumoperitoneo sin causa aparente. Se presenta el caso por la poca frecuencia en la edad pediátricas

Interstitial granulomatous pulmonary diseases: a diagnostic approach for the general pathologist

Some kinds of interstitial pneumonia present a histopathological pattern dominated by sarcoid - necrotizing or non-necrotizing - granulomas, which can be divided into two main groups: infectious and non-infectious. The infectious causes include tuberculosis, histoplasmosis, fungi in general, paracoccidioidomycosis, ascaridiasis, echinococcosis and dirophilariosis. The non-infectious causes include histiocytosis-X, hipersensitivity pneumonia, vasculitis, lymphomas, sarcoidosis, and pneumoconioses such as silicosis and berylliosis. The purpose of this review is to provide a practical guideline to enable general pathologists to make the differential diagnosis of granulomatous pulmonary diseases. For this purpose, anatomical-clinical-radiological correlations will be presented and targeted to each diagnosis discussed. Whenever a granulomatous inflammatory process is in progress, the search for infective agents by direct observation, by culture, and by histochemical methods should be mandatory. The histological aspects of infectious granulomas to be analyzed should include their random histo-anatomical location, the type of inflammatory reaction, and necrosis. A panel of complementary reactions (immunohistochemistry and PCR) should identify the infectious agent and, whenever their results and the culture are negative, the possibility of non-infectious granulomatous diseases has to be evaluated. In such cases, the histo-anatomical distribution (bronchocentric, lymphangitic, angiocentric, random), the qualitative characteristics of the lesions (type of necrosis and inflammatory reaction), and the correlation with the X-ray findings will help the diagnosis.

Fibrosis pulmonar idiopática / Idiopathic pulmonary fibrosis

Las enfermedades intersticiales del pulmón han aumentado su frecuencia por la mayor incidencia de factores ambientales desencadenantes pero también porque actualmente se diagnostican e identifican en forma más precisa. Dentro de estas enfermedades, la Fibrosis Pulmonar Idiopática en la más frecuente, pero muchas veces se agrupan dentro de esta entidad varios subtipos nosológicos de evolución diferente. Desde que Liebow subdividió las neumonías intersticiales, se reconocen diferentes patrones de la enfermedad. Actualmente Katzenstein y Myers han redefinido los diferentes subgrupos. Hacemos un repaso de los principales aspectos de estas afecciones y de sus características clínicas, inmunológicas y de otros aspectos diagnósticos relevantes. Insistimos en la importancia de la biopsia pulmonar quirúrgica como elemento definitorio para determinar el subtipo de neumonía intersticial, lo que conlleva un diagnóstico y una actitud terapéutica diferente

Management of interstitial lung disease: an audit at a university teaching hospital in Saudi Arabia.

To assess the management of interstitial lung disease (ILD) in relation to the published guidelines 122 consecutive cases were analyzed. Clinical features and non-invasive laboratory tests led to the diagnosis in nearly one sixth of the patients (16%), mainly CTD and a few miscellaneous disorders. In another sixth the diagnosis was reached by means of a transbronchial lung biopsy, particularly in sarcoidosis. Nearly a third had surgical lung biopsies, which were diagnostic in 98%. The diagnoses were reached in 82 patients (67%) and include: cryptogenic fibrosing alveolitis (20), sarcoidosis (16), connective tissue disease (17) and miscellaneous (29). The remaining third were undiagnosed, and this group had a higher mean age and was much less likely to receive immunosuppressive therapy than any group with a specific diagnosis. It is concluded that while physicians reached a specific diagnosis in most cases of ILD, commonly through a lung biopsy. A sizeable proportion (nearly a third), or remained undiagnosed and those were less likely to be treated and had a poorer prognosis. The availability of less invasive techniques should encourage physicians to obtain a biopsy since this is likely to lead to a more active approach to therapy.

Video thoracoscopic lung biopsy in diffuse interstitial lung diseases.

The present study examined the use of video thoracoscopic lung biopsy (VTLB) in diffuse interstitial lung disease, in comparison with open lung biopsy (OLB). Twenty and fifteen patients underwent VTLB and OLB, respectively, from 1987 to 1997 at the Central Chest Hospital, Thailand. Data in mean (SD). The mean age was 39 years in both groups. VTLB yielded equivalent size of lung tissues, 4.7 (2.32) cm3, and was as diagnostically useful as OLB. Estimated blood loss, 60 (37) mls, and length of pleural drainage, 2.8 (0.5) days, were comparable in either technique. As OLB had been in practice for decades, it took shorter operative time, 64 (11) mins, than VTLB, 105 (30) mins, (p = 0.005). Both VTLB and OLB approaches were safe and not associated with major postoperative complications.

Linfoma de Hodgkin primario de pulmón

Presentamos el caso de una mujer de 75 años, con artritis reumatoidea y síndrome de Sjogren, quien presenta un cuadro clínico de un año de evolución de disnea progresiva hasta el reposo acompañada de tos no productiva. La radiografía y la TAC del tórax mostraron infiltrado interticial bilateral micronodular e infiltrado de ocupación alveolar bibasal, sin la presencia de adenomegalias. Se realizó biopsia pulmonar abierta que fue demostrativa de enfermedades de Hodgkin asociada a cambios de pulmón reumatoideo (neumonia intersticial usual, UPI). No se encontró compromiso linfomatoso extratorácico en los estudios de extensión. Se discute la presentación clínica, radiológica y los hallazgos histológicos de esta infrecuente expresión de la enfermedad de Hodgkin

Enfermedad pulmonar intersticial difusa (EPID) en Colombia

Las EPID, aunque no muy comunes, son más frecuentes en Colombia de lo que en principio se piensa. Las condiciones locales, sin duda, influyen en su distribución y frecuencia y justifican su investigación en nuestro medio. Dadas las características de presentación y los métodos necesarios para el diagnóstico de las EPID, es necesaria la intervención del neumonologo en la mayoría de los casos. La cooperación interdisciplinaria permitirá conocer mejor el espectro de las EPID en nuestros país.

Síndrome de pulmones contraidos y neumonitis intersticial en LES: caso de evolución fatal

Se presentan el primer caso informado en la literatura de una complicación pulmonar infrecuente en pacientes con Lupus Eritematoso Sistémico, " El Síndrome de Pulmones Contraidos". El caso descrito presenta características especiales que se discuten ampliamente y permiten revisar el tema.