Evaluation of the impact of a pulmonary rehabilitation program on exercise capacity in patients with lymphangioleiomyomatosis

Introdução: A linfangioleiomiomatose (LAM) é uma neoplasia de baixo grau, frequentemente associada à redução na capacidade de exercício, secundária a múltiplos fatores incluindo alteração de troca gasosa, limitação ventilatória e hiperinsuflação dinâmica (HD). A reabilitação pulmonar (RP) tem benefícios bem estabelecidos em diversas doenças pulmonares crônicas, porém não foi estudada na LAM. Objetivos: Avaliar o impacto de um programa de RP, comparativamente a um grupo controle, em portadoras de LAM, nos seguintes parâmetros: capacidade de exercício (objetivo primário), HD, dispneia, nível de atividade física diária, qualidade de vida, ansiedade e depressão, função pulmonar e força muscular. Metodologia: Ensaio clínico, controlado, não-randomizado, incluindo 21 pacientes com LAM no grupo RP e 19 no grupo controle. A RP teve duração de 3 meses, compreendendo 24 sessões de uma hora de duração (30 minutos de exercício aeróbico e 30 minutos de treinamento de força muscular). A avaliação inicial incluiu um teste de exercício cardiopulmonar (TECP) máximo incremental. As seguintes variáveis foram avaliadas antes e após a RP ou observação (grupo controle): capacidade de exercício, através do tempo até o limite da tolerância (Tlim) no teste de exercício cardiopulmonar (TECP) com carga constante; distância percorrida e dessaturação de oxigênio no teste de caminhada de 6 minutos (TC6M); dispneia (escala de dispneia do Medical Research Council modificada - mMRC, Índice de Dispneia Basal - BDI, e Índice Transicional de Dispneia - TDI); nível de atividade física diária (pedômetro); qualidade de vida relacionada à saúde (Questionário Respiratório de St George\'s, SGRQ); ansiedade e depressão (Escala Hospitalar de Ansiedade e Depressão, HADS); provas de função pulmonar (PFP) e força muscular (uma repetição máxima, 1 RM). Resultados: Não houve diferença nas características basais entre os grupos RP e controle em relação à: idade (45 ± 11 vs. 40 ± 9 anos, p = 0,21), VEF1 (74 ± 30 vs. 70 ± 27% pred, p = 0,67), DLCO (67 ± 33 vs. 64 ± 30% pred, p = 0,79), carga máxima (77 ± 33 vs. 76 ± 35 W, p = 0,93) e O2 pico (17 ± 5 vs. 16 ± 4 ml/ kg/ min; p = 0,52) no TECP incremental. O grupo RP apresentou melhora comparativamente ao grupo controle em (expressos em mediana [intervalo interquartil]): Tlim (169 s [2 - 303 s] vs. -33 s [-129 - 39 s], p = 0,001) e O2 (11% [2 - 26%] vs. -2% [-7 - 5% pred], p = 0,001) no TECP com carga constante, mMRC (0 [-1 - 0] vs. 0 [0 - 1], p < 0,001), TDI (3 [2 - 3] vs. 0 [-2 - 0], p < 0,001), números de passos diários (752 [-694 - 1814] vs. -138 [-830 - 208], p= 0,02), SGRQ (-8 [-16 - 2] vs. 2 [-4 - 5], p = 0,002, distância caminhada no TC6M (59 m [13 - 81] vs. 20 [-12 - 30], p = 0,002) e 1 RM para todos os grupamentos musculares treinados (ex. quadríceps 39% [20 - 70%] vs. 4% [0 - 17%], p <0,001). Houve uma tendência de melhora nos sintomas de depressão e HADS total. HD, dessaturação ao exercício, sintomas de ansiedade e PFP não melhoraram após RP. Houve correlação moderada entre o aumento do Tlim e as variações da mMRC, do O2 de pico no TECP com carga constante, do Borg dispneia isotime e do Borg de pernas isotime. Conclusões: A RP está associada à melhora na capacidade de exercício, dispneia, nível de atividade física diária, qualidade de vida relacionada à saúde e força muscular em pacientes com LAM. O principal mecanismo sugerido é adaptação da musculatura periféricaIntroduction: Lymphangioleiomyomatosis (LAM) is a low-grade neoplasm, which is frequently associated with reduced exercise capacity, secondary to multiple factors including gas exchange impairment, ventilatory limitation and dynamic hyperinflation (DH). Pulmonary rehabilitation (PR) has proven benefits in many chronic pulmonary diseases but it was not evaluated in LAM. Objectives: To evaluate the impact of a PR program in women with LAM, when compared to a control group, in the following parameters: exercise capacity (primary outcome), DH, dyspnea, daily physical activity, quality of life, anxiety and depression, lung function and muscle strength. Methods: A non-randomized controlled clinical trial that included 21 LAM patients in the PR group and 19 in the control group. The PR program lasted 3 months, comprising 24 sessions of 1 hour (30 minutes of aerobic exercise and 30 minutes of muscle strength training). The initial evaluation included a maximum incremental cardiopulmonary exercise test (CPET). The following variables were assessed before and after PR or observation (control group): exercise capacity using the tolerable limit duration (Tlim) in constant work rate (CWR) exercise testing; walking distance and oxygen desaturation (six-minute walk test, 6MWT), dyspnea (Modified Medical Research Council Dyspnea Scale -mMRC; Basal Dyspnea Index - BDI, and Transitional Dyspnea Index -TDI); daily physical activity (pedometer); health-related quality of life (St George\'s Respiratory Questionnaire, SGRQ); anxiety and depression (Hospital Anxiety and Depression Scale, HADS); pulmonary function tests (PFT) and muscle strength (one-repetition maximum, 1RM). Results: There was no difference in baseline characteristics between the PR and control groups related to age (45 ± 11 vs. 40 ± 9 years, p = 0.12), FEV1 (74 ± 30 vs. 70 ± 27% pred, p = 0.67), DLCO (67 ± 33 vs. 64 ± 30% pred, p = 0.79), maximum work rate (77 ± 33 vs. 76 ± 35 W, p = 0.93) and peak O2 (17 ± 5 vs. 16 ± 4 ml/ kg/ min; p = 0.52) in incremental CPET. The PR group had a significant improvement when compared to the control group in (expressed in median [interquartile range]): Tlim (169 s [2 - 303 s] vs. -33 s [-129 - 39 s], p = 0.001) and O2 (11% [2 - 26%] vs. -2% [-7 - 5% pred], p = 0.001) in CWR exercice testing, mMRC (0 [-1 - 0] vs. 0 [0 - 1], p< 0.001), ( TDI (3 [2 - 3] vs.0 [-2 - 0], p< 0.001), daily steps (752 [-694 - 1814] vs. -138 [-830 - 208], p= 0.02), SGRQ (-8 [-16 - 2] vs. 2 [-4 - 5], p = 0.002), walking distance in 6MWT (median 59 m [13 - 81] vs. 20 [-12 - 30], p = 0.002) and 1 RM for all muscle groups trained (ex. quadriceps 39% [20 - 70%] vs. 4% [0 - 17%], p <0.001). There was a trend towards improvement in depression symptoms and total HADS. DH, desaturation during exercise, anxiety symptoms and PFT did not improve after PR. There was a moderate correlation between increased Tlim and variations of mMRC, peak O2 in CWR exercise testing, Borg dyspnea isotime and Borg leg discomfort isotime. Conclusions: PR is associated with improvements in exercise capacity, dyspnea, daily physical activity, health-related quality of life and muscle strength in patients with LAM. The main mechanism suggested is peripheral muscles adaptatio

Pneumomediastinum, subcutaneous emphysema, and pneumothorax after a pulmonary function testing in a patient with bleomycin-induced interstitial pneumonitis

Spontaneous pneumomediastinum is an uncommon event, the clinical picture of which includes retrosternal chest pain, subcutaneous emphysema, dyspnea, and dysphonia. The pathophysiological mechanism involved is the emergence of a pressure gradient between the alveoli and surrounding structures, causing alveolar rupture with subsequent dissection of the peribronchovascular sheath and infiltration of the mediastinum and subcutaneous tissue with air. Known triggers include acute exacerbations of asthma and situations that require the Valsalva maneuver. We described and documented with HRCT scans the occurrence of pneumomediastinum after a patient with bleomycin-induced interstitial lung disease underwent pulmonary function testing. Although uncommon, the association between pulmonary function testing and air leak syndromes has been increasingly reported in the literature, and lung diseases, such as interstitial lung diseases, include structural changes that facilitate the occurrence of this complication

Lung cysts in chronic paracoccidioidomycosis

On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung parenchyma and a well-defined interface with the normal adjacent lung. The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. In a retrospective analysis of the HRCT findings in 50 patients diagnosed with chronic paracoccidioidomycosis, we found lung cysts in 5 cases (10%), indicating that patients with paracoccidioidomycosis can present with lung cysts on HRCT scans. Therefore, paracoccidioidomycosis should be included in the differential diagnosis of cystic lung diseases

Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features

OBJECTIVE: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). METHODS: This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD). RESULTS: Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. CONCLUSIONS: In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria