Esclerose lateral amiotrófica é uma doença neuromuscular progressiva que resulta em fraqueza muscular, redução dos volumes pulmonares, tosse ineficaz, retenção de secreção e insuficiência respiratória. Medidas como a capacidade vital, pressão inspiratória e pressão expiratória máximas, pressão inspiratória máxima nasal, pico de fluxo de tosse e oximetria de pulso são recomendados para monitorar a função respiratória. Os pacientes devem ser acompanhados por uma equipe multidisciplinar, buscando melhorias na qualidade de vida e melhores estratégias para lidar com os sintomas respiratórios. A abordagem de cuidados respiratórios inclui técnicas de desobstrução das vias respiratórias, tosse assistida mecanicamente e ventilação mecânica não invasiva. Vacinação e suporte farmacológico também são recomendados. Até o momento, não existem provas suficientes que suportam o treinamento muscular inspiratório e a estimulação diafragmática.

Amyotrophic lateral sclerosis is a progressive neuromuscular disease, resulting in respiratory muscle weakness, reduced pulmonary volumes, ineffective cough, secretion retention, and respiratory failure. Measures as vital capacity, maximal inspiratory and expiratory pressures, sniff nasal inspiratory pressure, cough peak flow and pulse oximetry are recommended to monitor the respiratory function. The patients should be followed up by a multidisciplinary team, focused in improving the quality of life and deal with the respiratory symptoms. The respiratory care approach includes airway clearance techniques, mechanically assisted cough and noninvasive mechanical ventilation. Vaccination and respiratory pharmacological support are also recommended. To date, there is no enough evidence supporting the inspiratory muscle training and diaphragmatic pacing.

OBJECTIVE: Asthma may cause systemic repercussions due to its severity and the effects of treatment. Our objective was to compare posture, balance, functional capacity, and quality of life (QOL) according to the severity of disease, as assessed by pulmonary function levels. METHOD: This cross-sectional study evaluated fifty individuals with asthma. We compared two groups of adult individuals who were divided according to the median of the forced expiratory volume in one second (FEV1) as follows: group A  =  FEV1>74% predicted; group B  =  FEV1<74% predicted. All patients underwent the following tests: spirometry, whole-body plethysmography, diffusing capacity for carbon monoxide (DLco), respiratory muscle strength, posture assessment, stabilometry, six-minute walking distance (6MWD), and QOL. RESULTS: All pulmonary function variables exhibited statistically significant differences between the two groups, except for the DLco. The maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and 6MWD were lower in group B. The maximal mediolateral velocity and the mediolateral displacement were significantly different, while the postural changes and QOL were similar between the groups. CONCLUSIONS: In adult individuals with asthma, the pulmonary function is associated with balance control in the mediolateral direction but does not influence the postural changes or QOL.

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% vs. 72,1 ± 22,2%; 79,9 ± 17,8% vs. 72,5 ± 20,6%; e 74,0 ± 20,5% vs. 60,7 ± 26,8%, respectivamente; p = 0.0001 para todos), enquanto a relação VEF1/CVF aumentou significativamente (98,5 ± 7,2% vs. 101,9 ± 7,8%; 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% vs. 72.1 ± 22.2%; 79.9 ± 17.8% vs. 72.5 ± 20.6%; and 74.0 ± 20.5% vs. 60.7 ± 26.8%, respectively; p = 0.0001 for all), and the FEV1/FVC ratio significantly increased (98.5 ± 7.2% vs. 101.9 ± 7.8%; 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.