Perfil de pacientes evaluados con el Test de Capacidad de Difusión de Monóxido de Carbono: Una experiencia chilena / Profile of patients evaluated with the Carbon Monoxide Diffusion Capacity Test: A Chilean experience

ANTECEDENTES: La prueba de capacidad de difusión de monóxido de carbono (DLCO) es una evaluación de función pulmonar rutinaria y no invasiva clínicamente útil para determinar el estado de la función pulmonar en pacientes con trastornos crónicos como la enfermedad pulmonar intersticial difusa (EPID). OBJETIVO: Describir el perfil sociodemográfico y clínico de usuarios de la prueba DLCO en Valdivia, Chile. Materiales y Métodos: Estudio observacional, retrospectivo, de base documental. A partir de registros de 490 pacientes que se realizaron la prueba DLCO entre 2017 y 2019, se describen características sociodemográficas, clínicas y reporte de consumo de cigarrillo como cigarrillos/d e índice paquetes/año (IPA), comparando por sexo. RESULTADOS: La mayor proporción de evaluados fueron mujeres (61%), con una edad mediana de 65 años, mayor en mujeres (66 vs 64 años; p = 0,0361). La mayoría consultaron por EPID (54,5%). Según estado nutricional, 38% presentó preobesidad y 24,7% obesidad I (24,7%), destacando que 40,5% de las mujeres y 33% de los hombres se encontraron en alguna categoría de obesidad. Entre quienes reportaron información de consumo de cigarrillos (n = 346, 70,6%), 14,7% (n = 51) eran consumidores actuales, con un consumo mediano de 10 cigarrillos/d, sin diferencias por sexo. Entre exfumadores (n = 144; 50% hombres/mujeres) hubo significativamente menor consumo diario (5 vs 15; p = 0,0300) y de IPA (7 vs 18; p = 0,0083) en mujeres. CONCLUSIONES: En usuarios de DLCO el principal diagnóstico de consulta fue EPID. Destacó alta frecuencia de obesidad y tabaquismo, sin diferencia de consumo por sexo en fumadores actuales, pero sí en exfumadores.

BACKGROUND: The carbon monoxide diffusion capacity test (DLCO) is a clinically useful, routine, non-invasive lung function assessment to determine the status of lung function in patients with chronic disorders such as interstitial lung disease (ILD). AIM: To describe the sociodemographic and clinical profile of users of the DLCO test in Valdivia, Chile. MATERIALS AND METHODS: Observational, retrospective, documentary-based study. From the records of 490 patients who underwent the DLCO test between 2017 and 2019, sociodemographic and clinical characteristics and reports of cigarette consumption are described, such as cigarettes/d and pack-year index (PYI), comparing by sex. Results: The highest proportion of those evaluated were women (61%), with a median age of 65 years, higher in women (66 vs. 64 years; p = 0.0361). The majority consulted for ILD (54.5%). According to nutritional status, 38% presented pre-obesity and 24.7% obesity I (24.7%), highlighting that 40.5% of women and 33% of men were in some category of obesity. Among those who reported information on cigarette consumption (n = 346, 70.6%), 14.7% (n = 51) were current consumers, with a median consumption of 10 cigarettes/d, without differences by sex. Among exsmokers (n = 144; 50% men/women) there was significantly less daily (5 vs 15; p = 0.0300) and IPA (7 vs 18; p = 0.0083) consumption in women. CONCLUSIONS: In DLCO users, the main consultation diagnosis was ILD. High frequency of obesity and smoking stood out, with no difference in consumption by sex in current smokers, but yes in ex-smokers.

Fisiología respiratoria: fisiología de las enfermedades pulmonares restrictivas / Pathophysiology of pulmonary restrictive diseases

Las enfermedades restrictivas comprenden un grupo heterogéneo de trastornos que se caracterizan por una alteración en la distensibilidad pulmonar, generada por enfermedades del parénquima o intersticio pulmonar o por problemas extrapulmonares (alteraciones de la caja torácica o enfermedades neuromusculares). Presentan un patrón característico en las pruebas de función pulmonar, relación VEF1/ FVC normal o aumentada con CVF disminuida, VEF1 disminuido leve o normal y capacidad pulmonar total disminuida (CPT). Su manejo es complejo debido a la dificultad para establecer el diagnóstico diferencial, por lo que se recomienda una derivación precoz a un especialista en enfermedades respiratorias y el enfrentamiento por un equipo multidisciplinario.

Restrictive diseases comprise a heterogeneous group of disorders characterized by an alteration in lung compliance, generated by diseases of the lung parenchyma or interstitium, as well as by extrapulmonary problems (abnormalities of the rib cage or neuromuscular diseases). They present a characteristic pattern in pulmonary function tests, with decreased FVC (forced vital capacity), slightly decreased or normal FEV1 (forced expiratory volume in 1 second), normal or increased FEV1/FVC ratio, and decreased total lung capacity. Its management is complex due to the difficulty in establishing the differential diagnosis, so early referral to a specialist in respiratory diseases and confrontation by a multidisciplinary team is recommended.

Esophageal manometry in systemic sclerosis: findings and association with clinical manifestations

SUMMARY INTRODUCTION Systemic sclerosis (SSC) is an autoimmune disorder that affects several organs of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. OBJECTIVES To relate the profile of changes in esophageal electromanometry (EM), the profile of skin involvement, interstitial pneumopathy (ILD), and esophageal symptoms in SSC patients. METHODS This is an observational, cross-sectional study carried out at the SSC outpatient clinic of the Hospital de Clínicas of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially enrolled, from 04/12/2014 to 06/25/2015. They were submitted to the usual investigations according to the clinical picture. The statistical analysis was descriptive in percentage, means, and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high-resolution tomography, and esophageal symptoms. RESULTS 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% had ILD. 24.3% presented the diffuse form of SSC. No association was found between manometric changes and clinical manifestations (cutaneous, pulmonary, and gastrointestinal symptoms). CONCLUSION The present study confirms that esophageal motility alterations detected by EM are frequent in SSC patients, but may not be related to cutaneous extension involvement, the presence of ILD, or the gastrointestinal complaints of patients.

RESUMO INTRODUÇÃO A esclerose sistêmica (ES) é uma doença autoimune que afeta vários órgãos de etiologia desconhecida, caracterizada por dano vascular e fibrose da pele e órgãos. Entre os órgãos envolvidos estão o esôfago e o pulmão. OBJETIVOS Relacionar o perfil das alterações na eletromanometria (ME), o perfil de acometimento da pele, a pneumopatia intersticial (PI) e os sintomas esofágicos em pacientes com ES. MÉTODO Trata-se de um estudo observacional, transversal, realizado no ambulatório de SSC do Hospital das Clínicas da Universidade Federal de Uberlândia. Após aprovação pelo Comitê de Ética e assinatura dos termos de consentimento, 50 pacientes foram inicialmente convidados, de 04/12/2014 a 25/06/2015. Eles foram submetidos às investigações usuais de acordo com o quadro clínico. A análise estatística foi descritiva em porcentagem, média e desvio padrão. O teste Qui-quadrado foi utilizado para avaliar a relação entre ME, tomografia de alta resolução e sintomas esofágicos. RESULTADOS 91,9% dos pacientes apresentaram alterações manométricas. 37,8% tinham envolvimento do corpo esofágico e do esfíncter esofágico inferior. 37,8% tinham IP. 24,3% apresentaram a forma difusa da ES. Não há associação entre alterações manométricas e manifestações clínicas (sintomas cutâneos, pulmonares e gastrointestinais). CONCLUSÃO O presente estudo confirma que as alterações da motilidade esofágica detectadas pela EM são frequentes em pacientes com SSC, mas podem não estar relacionadas ao envolvimento cutâneo, à de DPI ou às queixas gastrointestinais dos pacientes.

Translation and cultural adaptation of the King's Brief Interstitial Lung Disease health status questionnaire for use in Brazil / Tradução e adaptação cultural do King's Brief Interstitial Lung Disease health status questionnaire

ABSTRACT Objective: To translate the King's Brief Interstitial Lung Disease (K-BILD) questionnaire to Portuguese and culturally adapt it for use in Brazil. The K-BILD quantifies the health status of patients with ILD. Methods: The process involved the following steps: authorization from the author of the original (English-language) questionnaire; translation of the questionnaire to Portuguese by three translators, working independently; merging of the translations by a committee of specialists; back-translation of the questionnaire to English; revision and readjustment of the back-translation by the committee of specialists; evaluation by the original author; revision of the back-translation; cognitive debriefing (verification of the clarity and acceptability of the Portuguese-language version in the target population-i.e., patients with ILD); and finalization of the Portuguese-language version. Results: In the cognitive debriefing step, 20 patients with ILD were interviewed. After the interviews, the clarity and acceptability index of each question was ≥ 0.8, which is considered acceptable. Conclusions: The Portuguese-language version of K-BILD appears to be easily administered to and understood by patients with ILD in Brazil. To our knowledge, this is the only instrument in Brazilian Portuguese that is designed to evaluate the impact that ILD has on the various aspects of the lives of those it affects.

RESUMO Objetivo: Traduzir e adaptar culturalmente o King's Brief Interstitial Lung Disease (K-BILD) health status questionnaire, utilizado para mensurar o estado de saúde em pacientes com doença pulmonar intersticial (DPI), para a língua portuguesa falada no Brasil. Métodos: As seguintes etapas foram realizadas: autorização do autor do instrumento original; preparação; tradução do K-BILD da língua inglesa para a língua portuguesa falada no Brasil por três tradutores independentes; unificação das traduções por um comitê de especialistas; retradução do português para o inglês; revisão e readequação da retradução pelo comitê de especialistas; avaliação do autor do original; revisão da versão em inglês; desdobramento cognitivo (observação da clareza e aceitabilidade do questionário traduzido na população-alvo); e elaboração da versão final em português. Resultados: Na etapa de desdobramento cognitivo foram entrevistados 20 pacientes com DPI. Após todas as entrevistas, o índice de clareza e aceitabilidade das questões foi igual ou maior que 0,8, indicando um índice de clareza aceitável para todas as questões. Conclusões: A versão do K-BILD mostrou ser compreensível e de fácil aplicação. Até onde sabemos, esse é atualmente o único instrumento disponível em português falado no Brasil dirigido à avaliação do impacto da DPI em vários aspectos da vida desses pacientes.

Ventilation distribution as a contributor to the functional exercise capacity in patients with systemic sclerosis-associated interstitial lung disease without pulmonary hypertension

Phenotypic differences have been described between patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and SSc-associated pulmonary hypertension, including performance differences in the 6-min walk test (6MWT). Moreover, the correlations between the 6MWT and traditional pulmonary function tests (PFTs) are weak, indicating the need to search for new parameters that explain exercise performance. Thus, our objective was to evaluate the impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N2SBW) test and peripheral muscle dysfunction on the exercise capacity in patients with SSc-ILD and limited involvement of the pulmonary parenchyma. In this cross-sectional study, 20 women with SSc-ILD and 20 matched controls underwent PFTs (including spirometry, diffusing capacity for carbon monoxide (DLco), and the N2SBW test) and performed the 6MWT and knee isometric dynamometry. The 6-min walking distance (6MWD, % predicted) was strongly correlated with the phase III slope of the single-breath nitrogen washout (phase III slopeN2SBW) (r=−0.753, P<0.0001) and reasonably correlated with the forced vital capacity (FVC) (r=0.466, P=0.008) and DLco (r=0.398, P=0.011). The peripheral oxygen saturation (SpO2) during exercise was not significantly correlated with any of the pulmonary or muscle function parameters. The phase III slopeN2SBW was the only predictive variable for the 6MWD, whereas quadriceps strength and FVC/DLco were predictive variables for SpO2. Ventilation distribution heterogeneity is one factor that contributes to a lower 6MWD in SSc-ILD patients. In addition, muscle dysfunction and abnormal lung diffusion at least partly explain the decreased SpO2 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.

Lung volumes and airway resistance in patients with a possible restrictive pattern on spirometry / Volumes pulmonares e resistência das vias aéreas em pacientes com possível padrão restritivo à espirometria

ABSTRACT Objective: Many patients with proportional reductions in FVC and FEV1 on spirometry show no reduction in TLC. The aim of this study was to evaluate the role that measuring lung volumes and airway resistance plays in the correct classification of patients with a possible restrictive pattern on spirometry. Methods: This was a prospective study involving adults with reduced FVC and FEV1, as well as an FEV1/FV(C) ratio within the predicted range. Restrictive lung disease (RLD) was characterized by TLC below the 5th percentile, as determined by plethysmography. Obstructive lung disease (OLD) was characterized by high specific airway resistance, significant changes in post-bronchodilator FEV1, or an FEF25-75% < 50% of predicted, together with a high RV/TLC ratio. Nonspecific lung disease (NLD) was characterized by TLC within the predicted range and no obstruction. Combined lung disease (CLD) was characterized by reduced TLC and findings indicative of airflow obstruction. Clinical diagnoses were based on clinical suspicion, a respiratory questionnaire, and the review of tests of interest. Results: We included 300 patients in the study, of whom 108 (36%) were diagnosed with RLD. In addition, 120 (40%) and 72 (24%) were diagnosed with OLD/CLD and NLD, respectively. Among the latter, 24 (33%) were clinically diagnosed with OLD. In this sample, 151 patients (50.3%) were obese, and obesity was associated with all patterns of lung disease. Conclusions: Measuring lung volumes and airway resistance is often necessary in order to provide an appropriate characterization of the pattern of lung disease in patients presenting with a spirometry pattern suggestive of restriction. Airflow obstruction is common in such cases.

RESUMO Objetivo: Muitos pacientes com redução proporcional de CVF e VEF1 na espirometria não têm CPT reduzida. O objetivo deste estudo foi avaliar o papel da medida dos volumes pulmonares e da resistência das vias aéreas para a classificação correta de pacientes com possível restrição à espirometria. Métodos: Estudo prospectivo de adultos com CVF e VEF1 reduzidos e relação VEF1/CV(F) na faixa prevista. Distúrbio ventilatório restritivo (DVR) foi definido por CPT < 5º percentil por pletismografia. Distúrbio ventilatório obstrutivo (DVO) foi caracterizado por resistência específica de vias aéreas elevada, resposta significativa do VEF1 pós-broncodilatador e/ou um FEF25-75% < 50% do previsto associado a uma relação VR/CPT elevada. Distúrbio ventilatório inespecífico (DVI) foi caracterizado por CPT na faixa prevista e ausência de obstrução. Distúrbio ventilatório combinado (DVC) foi caracterizado por CPT reduzida e achados indicativos de obstrução ao fluxo aéreo. Os diagnósticos clínicos foram baseados em suspeita clínica, um questionário respiratório e revisão de exames de interesse. Resultados: Foram incluídos 300 pacientes no estudo, dos quais 108 (36%) tiveram diagnóstico de DVR, enquanto 120 (40%) foram diagnosticados com DVO ou DVC e 72 (24%) com DVI. Destes últimos, 24 (33%) tinham diagnóstico clínico de DVO. Nesta amostra, 151 pacientes (50,3%) eram obesos, e isso se associou com todos os padrões de distúrbios funcionais. Conclusões: Medidas dos volumes pulmonares e da resistência das vias aéreas são frequentemente necessárias para a caracterização adequada do tipo de distúrbio funcional em casos com possível restrição à espirometria. A obstrução ao fluxo aéreo é comum nesses casos.

Identifying decreased diaphragmatic mobility and diaphragm thickening in interstitial lung disease: the utility of ultrasound imaging / Identificação da diminuição da mobilidade diafragmática e do espessamento diafragmático na doença pulmonar intersticial: utilidade da ultrassonografia

Objective: To investigate the applicability of ultrasound imaging of the diaphragm in interstitial lung disease (ILD). Methods: Using ultrasound, we compared ILD patients and healthy volunteers (controls) in terms of diaphragmatic mobility during quiet and deep breathing; diaphragm thickness at functional residual capacity (FRC) and at total lung capacity (TLC); and the thickening fraction (TF, proportional diaphragm thickening from FRC to TLC). We also evaluated correlations between diaphragmatic dysfunction and lung function variables. Results: Between the ILD patients (n = 40) and the controls (n = 16), mean diaphragmatic mobility was comparable during quiet breathing, although it was significantly lower in the patients during deep breathing (4.5 ± 1.7 cm vs. 7.6 ± 1.4 cm; p < 0.01). The patients showed greater diaphragm thickness at FRC (p = 0.05), although, due to lower diaphragm thickness at TLC, they also showed a lower TF (p < 0.01). The FVC as a percentage of the predicted value (FVC%) correlated with diaphragmatic mobility (r = 0.73; p < 0.01), and an FVC% cut-off value of < 60% presented high sensitivity (92%) and specificity (81%) for indentifying decreased diaphragmatic mobility. Conclusions: Using ultrasound, we were able to show that diaphragmatic mobility and the TF were lower in ILD patients than in healthy controls, despite the greater diaphragm thickness at FRC in the former. Diaphragmatic mobility correlated with ILD functional severity, and an FVC% cut-off value of < 60% was found to be highly accurate for indentifying diaphragmatic dysfunction on ultrasound.

Objetivo: Investigar a aplicabilidade da ultrassonografia do diafragma na doença pulmonar intersticial (DPI). Métodos: Por meio da ultrassonografia, pacientes com DPI e voluntários saudáveis (controles) foram comparados quanto à mobilidade diafragmática durante a respiração profunda e a respiração tranquila, à espessura diafragmática no nível da capacidade residual funcional (CRF) e da capacidade pulmonar total (CPT) e à fração de espessamento (FE, espessamento diafragmático proporcional da CRF até a CPT). Foram também avaliadas correlações entre disfunção diafragmática e variáveis de função pulmonar. Resultados: Entre os pacientes com DPI (n = 40) e os controles (n = 16), a média da mobilidade diafragmática foi comparável durante a respiração tranquila, embora tenha sido significativamente menor nos pacientes durante a respiração profunda (4,5 ± 1,7 cm vs. 7,6 ± 1,4 cm; p < 0,01). Os pacientes apresentaram maior espessura diafragmática na CRF (p = 0,05), embora tenham também apresentado, devido à menor espessura diafragmática na CPT, menor FE (p < 0,01). A CVF em porcentagem do previsto (CVF%) correlacionou-se com a mobilidade diafragmática (r = 0,73; p < 0,01), e um valor de corte < 60% da CVF% apresentou alta sensibilidade (92%) e especificidade (81%) na identificação de mobilidade diafragmática reduzida. Conclusões: Com a ultrassonografia, foi possível demonstrar que a mobilidade diafragmática e a FE estavam mais reduzidas nos pacientes com DPI do que nos controles saudáveis, apesar da maior espessura diafragmática na CRF nos pacientes. A mobilidade diafragmática correlacionou-se com a gravidade funcional da DPI, e um valor de corte < 60% da CVF% mostrou ser altamente acurado na identificação da disfunção diafragmática por ultrassonografia.

Usefulness of the second heart sound for predicting pulmonary hypertension in patients with interstitial lung disease / Utilidade da segunda bulha cardíaca na predição de hipertensão pulmonar em portadores de doenças intersticiais pulmonares

CONTEXT AND OBJECTIVE: P2 hyperphonesis is considered to be a valuable finding in semiological diagnoses of pulmonary hypertension (PH). The aim here was to evaluate the accuracy of the pulmonary component of second heart sounds for predicting PH in patients with interstitial lung disease. DESIGN AND SETTING: Cross-sectional study at the University of Brasilia and Hospital de Base do Distrito Federal. METHODS: Heart sounds were acquired using an electronic stethoscope and were analyzed using phonocardiography. Clinical signs suggestive of PH, such as second heart sound (S2) in pulmonary area louder than in aortic area; P2 > A2 in pulmonary area and P2 present in mitral area, were compared with Doppler echocardiographic parameters suggestive of PH. Sensitivity (S), specificity (Sp) and positive (LR+) and negative (LR-) likelihood ratios were evaluated. RESULTS: There was no significant correlation between S2 or P2 amplitude and PASP (pulmonary artery systolic pressure) (P = 0.185 and 0.115; P= 0.13 and 0.34, respectively). Higher S2 in pulmonary area than in aortic area, compared with all the criteria suggestive of PH, showed S = 60%, Sp= 22%; LR+ = 0.7; LR- = 1.7; while P2> A2 showed S= 57%, Sp = 39%; LR+ = 0.9; LR- = 1.1; and P2 in mitral area showed: S= 68%, Sp = 41%; LR+ = 1.1; LR- = 0.7. All these signals together showed: S= 50%, Sp = 56%. CONCLUSIONS: The semiological signs indicative of PH presented low sensitivity and specificity levels for clinically diagnosing this comorbidity.

RESUMO CONTEXTO E OBJETIVO: Hiperfonese de P2 tem sido considerada como achado valoroso no diagnóstico semiológico de hipertensão pulmonar (HP). O objetivo foi de avaliar a acurácia do componente pulmonar da segunda bulha cardíaca em predizer HP nos pacientes portadores de doenças intersticiais pulmonares. TIPO DE ESTUDO E LOCAL: Estudo transversal na Universidade de Brasília e Hospital de Base do Distrito Federal. MÉTODOS: Os sons cardíacos foram adquiridos com estetoscópio eletrônico e analisados por fonocardiografia. Os sinais clínicos sugestivos de HP, como B2 mais intensamente audível em área pulmonar que aórtica, P2 > A2 na área pulmonar e P2 presente em área mitral foram confrontados com parâmetros cardiográficos no exame de Doppler sugestivos de HP. Sensibilidade (S), especificidade (E), razões de verossimilhança positiva (RV+) e negativa (RV-) foram avaliados. RESULTADOS: Não houve correlação significativa entre amplitude de B2 e P2 e a PSAP (pressão sistólica arterial pulmonar) (P = 0,185 e 0,115; P = 0,13 e 0,34; respectivamente). A análise da presença de B2 mais intensa na área pulmonar que aórtica, quando comparada a todos os critérios sugestivos de HP, mostrou S = 60%; E = 22%; RV+ = 0,7; RV- = 1,7; enquanto P2 > A2 mostrou: S = 57%; E = 39%; RV+ = 0,9; RV- = 1,1; e P2 no foco mitral mostrou: S = 68%; E = 41%; RV+ = 1,1; RV- = 0,7. Todos os sinais juntos mostraram S = 50%; E = 56%. CONCLUSÃO: Os sinais semiológicos indicativos de HP apresentam baixos valores de especificidade e sensibilidade para diagnóstico clínico dessa comorbidade.

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.

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.

Pneumonia intersticial associada à esclerose sistêmica: avaliação da função pulmonar no período de cinco anos / Systemic sclerosis-associated interstitial pneumonia: evaluation of pulmonary function over a five-year period

OBJETIVO: Avaliar as alterações da função pulmonar em portadores de pneumonia intersticial associada à esclerose sistêmica no intervalo de cinco anos. MÉTODOS: Foi realizado um estudo longitudinal no qual foram avaliados 35 pacientes não tabagistas com esclerose sistêmica e sem história de doença pulmonar prévia. Na primeira avaliação, realizada na época do diagnóstico da pneumonia intersticial, os pacientes foram submetidos à TCAR, espirometria e medida de DLCO. Os pacientes foram subdivididos em dois grupos de acordo com a presença ou não de faveolamento na TCAR. Aproximadamente cinco anos após a primeira avaliação, os pacientes foram submetidos a espirometria e medida da DLCO apenas. RESULTADOS: Dos 35 pacientes, 34 eram mulheres, com média de idade de 47,6 anos. A média de tempo entre as duas avaliações foi de 60,9 meses. O faveolamento foi demonstrado por TCAR em 17 pacientes. Na amostra total, após cinco anos do diagnóstico, CVF, VEF1 e DLCO reduziram significativamente (81,3 ± 18,2 por cento vs. 72,1 ± 22,2 por cento; 79,9 ± 17,8 por cento vs. 72,5 ± 20,6 por cento; e 74,0 ± 20,5 por cento vs. 60,7 ± 26,8 por cento, respectivamente; p = 0.0001 para todos), enquanto a relação VEF1/CVF aumentou significativamente (98,5 ± 7,2 por cento vs. 101,9 ± 7,8 por cento; p = 0,008). No mesmo período, os valores de CVF, VEF1 e DLCO foram significativamente menores nos pacientes com faveolamento do que naqueles sem faveolamento na TCAR (p = 0,0001). CONCLUSÕES: Na esclerose sistêmica com doença pulmonar intersticial associada, a detecção de faveolamento na TCAR é determinante para predizer uma deterioração acelerada da função pulmonar.

OBJECTIVE: To evaluate alterations in pulmonary function in patients with systemic sclerosis-associated interstitial pneumonia over a five-year period. METHODS: This was a longitudinal study involving 35 nonsmoking patients with systemic sclerosis and without a history of lung disease. At the first evaluation, performed at the time of the diagnosis of interstitial pneumonia, the patients were submitted to HRCT, spirometry, and measurement of DLCO. The patients were subdivided into two groups by the presence or absence of honeycombing on the HRCT scans. Approximately five years after the first evaluation, the patients were submitted to spirometry and measurement of DLCO only. RESULTS: Of the 35 patients, 34 were women. The mean age was 47.6 years. The mean time between the two evaluations was 60.9 months. Honeycombing was detected on the HRCT scans in 17 patients. In the sample as a whole, five years after the diagnosis, FVC, FEV1 and DLCO significantly decreased (81.3 ± 18.2 percent vs. 72.1 ± 22.2 percent; 79.9 ± 17.8 percent vs. 72.5 ± 20.6 percent; and 74.0 ± 20.5 percent vs. 60.7 ± 26.8 percent, respectively; p = 0.0001 for all), and the FEV1/FVC ratio significantly increased (98.5 ± 7.2 percent vs. 101.9 ± 7.8 percent; p = 0.008). In the same period, FVC, FEV1, and DLCO values were significantly lower in the patients with honeycombing on the HRCT scans than in those without (p = 0.0001). CONCLUSIONS: In systemic sclerosis-associated interstitial lung disease, the detection of honeycombing on HRCT is crucial to predicting accelerated worsening of pulmonary function.

Hypoxemia and Arrhythmia during Daily Activities and Six-minute Walk Test in Fibrotic Interstitial Lung Diseases

We performed 24-hr monitoring of pulse oximetric saturation (SpO2) with ECG and six-minute walk test (6MWT) in 19 patients with fibrotic interstitial lung diseases (ILD) to investigate; 1) The frequency and severity of hypoxemia and dysrhythmia during daily activities and 6MWT, 2) safety of 6MWT, and 3) the parameters of 6MWT which can replace 24-hr continuous monitoring of SpO2 to predict hypoxemia during daily activities. All patients experienced waking hour hypoxemia, and eight of nineteen patients spent > 10% of waking hours in hypoxemic state. Most patients experienced frequent arrhythmia, mostly atrial premature contractions (APCs) and ventricular premature contractions (VPCs). There were significant correlation between the variables of 6MWT and hypoxemia during daily activities. All of the patients who desaturated below 80% before 300 meters spent more than 10% of waking hour in hypoxemia (P = 0.018). In contrast to waking hour hypoxemia, SpO2 did not drop significantly during sleep except in the patients whose daytime resting SpO2 was already low. In conclusion, patients with fibrotic ILD showed significant period of hypoxemia during daily activities and frequent VPCs and APCs. Six-minute walk test is a useful surrogate marker of waking hour hypoxemia and seems to be safe without continuous monitoring of SpO2.

Sono e doenças pulmonares crônicas: pneumopatias intersticiais difusas, asma brônquica e DPOC / Sleep and chronic lung diseases: diffuse interstitial lung diseases, bronchial asthma, and COPD

As doenças pulmonares crônicas podem ser agravadas por inúmeros fatores e comorbidades, inclusive pelos distúrbios respiratórios do sono. Embora alterações na qualidade de vida de pacientes com doenças pulmonares crônicas sejam normalmente determinadas a partir de variáveis diurnas, as alterações fisiopatológicas no sono pioram a qualidade de sono e interferem na história natural dessas doenças. Alterações da arquitetura do sono parecem ser um mecanismo comum entre essas doenças. Durante o sono, as vias respiratórias inferiores estão mais interligadas às vias respiratórias superiores, quando as alterações de resistências das vias aéreas superiores durante o sono somam-se às graves alterações resistivas de vias aéreas inferiores devido a asma e DPOC. Além disso, surgem complexas interações mecânicas e ventilatórias. O reconhecimento dessas interações possibilita uma melhor avaliação das exacerbações e da evolução dessas doenças.

Chronic lung diseases can be aggravated by various factors and comorbidities, including sleep-disordered breathing. Although changes in the quality of life of patients with chronic lung disease are usually related to daytime variables, the physiological changes in sleep impair the quality of sleep and interfere with the natural history of the disease. Alterations in sleep architecture appear to be a common mechanism in these diseases. During sleep, the upper and lower airways are more interconnected: changes in upper airway resistance during sleep are added to the severe resistive alterations in the lower airways due to asthma and COPD. In addition, there are complex mechanical and ventilatory interactions. The recognition of these interactions allows better assessment of the exacerbations and the progression of chronic lung diseases.

Desaturation - distance ratio: a new concept for a functional assessment of interstitial lung diseases

INTRODUCTION: The functional evaluation has become increasingly important in the understanding and management of patients with interstitial lung diseases. The cardiopulmonary exercise test and the six-minute walk test (6MWT), through their isolated variables, have been used to do this evaluation, with some limitations. OBJECTIVES: We proposed a new composite index (desaturation distance ratio using continuous peripheral oxygen saturation (SpO2) and the distance walked as a more reliable tool for doing a functional evaluation of these patients. METHODS: 6MWT was performed by interstitial lung diseases patients and controls. Analyzed parameters were walked distance and desaturation area (DAO2), obtained by taking the difference between maximal SpO2 possible (100 percent) and patient's SpO2 every 2 seconds. desaturation distance ratio was calculated using the ratio between DAO2 and distance walked. RESULTS: Forty-nine interstitial lung diseases patients and 11 control subjects completed the protocol. The mean (SD) age was 60 (12) years and 65 (9) years, respectively (p:NS). Data obtained from 6MWT showed a significant statistical difference between interstitial lung diseases patients and controls: mean walked distance (430 and 602 meters, respectively); SpO2 minimal maintained at least 10 seconds - SpO2 min (85 percent and 94 percent, respectively), and median desaturation distance ratio (10 and 2.5, respectively). A correlation analysis, considering interstitial lung diseases patients, revealed the best correlation between desaturation distance ratio and DLco (r= - 0.72; p<0.001), being the correlation between SpO2 min and DLco of 0.61 (p<0.001) and among walked distance and DLco of 0.58 (p<0.05). CONCLUSION: Desaturation distance ratio is a promising concept and a more reliable physiologic tool to assess pulmonary diseases characterized by involvement of the alveolar-capillary membrane, such as interstitial lung diseases.

Relevância da lesão da unidade epitélio/membrana basal epitelial no remodelamento arquitetural precoce de pneumonias intersticiais idiopáticas / Relevance of the epithelial cell/epithelial basement membrane unity lesion in idiopathic interstitial pneumonias early achitectural

Foi feito um estudo retrospectivo qualitativo e quantitativo, por técnicas histoquímicas e imuno-histoquímicas, do remodelamento arquitetural precoce em padrões histológicos de pneumonia intersticial idiopática em 81 biopsias pulmonares a céu aberto realizadas no Hospital das Clínicas da FMUSP. A forma de remodelamento dependeu da extensão da lesão da unidade epitélio-membrana basal e da configuração espacial do ácino pulmonar, obedecendo a mesma seqüência básica de eventos morfológicos observada na cicatrização de feridas. Todos padrões histológicos apresentaram as várias formas de remodelamento, cuja magnitude se correlacionou com seus respectivos cursos clínicos naturais / Early architectural remodelling in idiopathic interstitial pneumonia histologic patterns were retrospectively studied in 81 open lung biopsies performed at the Hospital das Clínicas da FMUSP. Morphological parameters were qualitative and quantitatively analysed after histochemical and immunohistochemical procedures. The type of remodelling depended on the injury extension to the epithelial cell/epithelial basement membrane unity and on the acinar spatial configuration, obeying the basic sequence of morphological events observed in wound healing. All histologic patterns showed the different types of remodelling, which amounts correlated with their respective natural clinical histories...