Lung Magnetic Resonance Imaging for Prediction of Progression in Patients With Nonidiopathic Pulmonary Fibrosis Interstitial Lung Disease: A Pilot Study.

PURPOSE: Correlate magnetic resonance imaging (MRI) parameters at baseline with disease progression in nonidiopathic pulmonary fibrosis interstitial lung disease (ILD). MATERIALS AND METHODS: Prospective observational cohort study, in which patients with non-idiopathic pulmonary fibrosis ILD underwent MRI at baseline (1.5 T). T2-weighted images (T2-WI) were acquired by axial free-breathing respiratory-gated fat-suppressed "periodically rotated overlapping parallel lines with enhanced reconstruction" and T1-weighted images (T1-WI) by coronal end-expiratory breath-hold fat-suppressed "volumetric interpolated breath-hold examination" sequences, before and at time points T1, T3, T5, and T10 minutes after gadolinium administration. After MRI segmentation, signal intensity values were extracted by dedicated software. Percentage of the ILD volume and a ratio between signal intensity of ILD (SIILD) and normal lung (SInormal lung) were calculated for T2-WI; percentage of signal intensity (%SI) at each time point, time to peak enhancement, and percent relative enhancement of ILD in comparison with normal lung (%SIILD/normal lung) were calculated for T1-WI. MRI parameters at baseline were correlated with diagnosis of disease progression and variation in percent predicted forced vital capacity (%FVC) and diffusing capacity of the lung for carbon monoxide after 12 months. RESULTS: Comprehensive MRI evaluation (T2-WI and T1-WI) was performed in 21 of the 25 patients enrolled (68% females; mean age: 62.6 y). Three of the 24 patients who completed follow-up fulfilled criteria for disease progression. Baseline T2-WI SIILD/SInormal lung was higher for the progression group (P = 0.052). T2-WI SIILD/SInormal lung and T1-WI %SIILD/normal lung at T1 were positively correlated with the 12-month variation in %FVC (r = 0.495, P = 0.014 and r = 0.489, P= 0.034, respectively). CONCLUSIONS: Baseline MRI parameters correlate with %FVC decline after 12 months.

Spirometry for the diagnosis of airway obstruction in patients with risk factors for COPD: the GOLD and lower limit of normal criteria.

OBJECTIVE: The identification of persistent airway obstruction is key to making a diagnosis of COPD. The GOLD guidelines suggest a fixed criterion-a post-bronchodilator FEV1/FVC ratio < 70%-to define obstruction, although other guidelines suggest that a post-bronchodilator FEV1/FVC ratio < the lower limit of normal (LLN) is the most accurate criterion. METHODS: This was an observational study of individuals ≥ 40 years of age with risk factors for COPD who were referred to our pulmonary function laboratory for spirometry. Respiratory symptoms were also recorded. We calculated the prevalence of airway obstruction and of no airway obstruction, according to the GOLD criterion (GOLD+ and GOLD-, respectively) and according to the LLN criterion (LLN+ and LLN-, respectively). We also evaluated the level of agreement between the two criteria. RESULTS: A total of 241 individuals were included. Airway obstruction was identified according to the GOLD criterion in 42 individuals (17.4%) and according to the LLN criterion in 23 (9.5%). The overall level of agreement between the two criteria was good (k = 0.67; 95% CI: 0.52-0.81), although it was lower among the individuals ≥ 70 years of age (k = 0.42; 95% CI: 0.12-0.72). The proportion of obese individuals was lower in the GOLD+/LLN+ category than in the GOLD+/LLN- category (p = 0.03), as was the median DLCO (p = 0.04). CONCLUSIONS: The use of the GOLD criterion appears to be associated with a higher prevalence of COPD. The agreement between the GOLD and LLN criteria also appears to be good, albeit weaker in older individuals. The use of different criteria to define airway obstruction seems to identify individuals with different characteristics. It is essential to understand the clinical meaning of discordance between such criteria. Until more data are available, we recommend a holistic, individualized approach to, as well as close follow-up of, patients with discordant results for airway obstruction.

Current Practices in Home Mechanical Ventilation for Chronic Obstructive Pulmonary Disease: A Real-Life Cross-Sectional Multicentric Study.

Purpose: Home mechanical ventilation (HMV) use in chronic obstructive pulmonary disease (COPD) is becoming increasingly widespread. The aim of this study was to provide an accurate description of the current practices and clinical characteristics of COPD patients on HMV in Portugal. Methods: The study was designed as a cross-sectional, multicenter real-life study of COPD patients established on HMV for at least 30 days. Data related to clinical characteristics, adaptation and ventilatory settings were collected. Results: The study included 569 COPD patients on HMV from 15 centers. The majority were male, with a median age of 72 years and a high prevalence of obesity (43.2%) and sleep apnea (45.8%). A high treatment compliance was observed (median 8h/day), 48.7% with inspiratory positive airway pressure ≥20 cmH2O and oronasal masks were the preferred interface (91.7%). There was an equal distribution of patients starting HMV during chronic stable condition and following an exacerbation. Patients in stable condition were initiated in the outpatient setting in 92.3%. Despite the differences in criteria and setting of adaptation and a slightly lower BMI in patients starting HMV following an exacerbation, we found no significant differences regarding age, gender, ventilation pressures, time on HMV, usage, severity of airflow obstruction or current arterial blood gas analysis (ABGs) in relation to patients adapted in stable condition. Conclusion: Patients were highly compliant with the therapy. In agreement with most recent studies and recommendations, there seems to be a move towards higher ventilation pressures, increased use of oronasal masks and an intent to obtain normocapnia. This study shows that chronic hypercapnic and post exacerbation patients do not differ significantly regarding patient characteristics, physiological parameters or ventilatory settings with one exception: chronic hypercapnic patients are more often obese and, subsequently, more frequently present OSA.

Spirometry for the diagnosis of airway obstruction in patients with risk factors for COPD: the GOLD and lower limit of normal criteria / Espirometria para o diagnóstico de obstrução das vias aéreas em pacientes com fatores de risco para DPOC: os critérios GOLD e limite inferior da normalidade

ABSTRACT Objective: The identification of persistent airway obstruction is key to making a diagnosis of COPD. The GOLD guidelines suggest a fixed criterion-a post-bronchodilator FEV1/FVC ratio < 70%-to define obstruction, although other guidelines suggest that a post-bronchodilator FEV1/FVC ratio < the lower limit of normal (LLN) is the most accurate criterion. Methods: This was an observational study of individuals ≥ 40 years of age with risk factors for COPD who were referred to our pulmonary function laboratory for spirometry. Respiratory symptoms were also recorded. We calculated the prevalence of airway obstruction and of no airway obstruction, according to the GOLD criterion (GOLD+ and GOLD−, respectively) and according to the LLN criterion (LLN+ and LLN−, respectively). We also evaluated the level of agreement between the two criteria. Results: A total of 241 individuals were included. Airway obstruction was identified according to the GOLD criterion in 42 individuals (17.4%) and according to the LLN criterion in 23 (9.5%). The overall level of agreement between the two criteria was good (k = 0.67; 95% CI: 0.52-0.81), although it was lower among the individuals ≥ 70 years of age (k = 0.42; 95% CI: 0.12-0.72). The proportion of obese individuals was lower in the GOLD+/LLN+ category than in the GOLD+/LLN− category (p = 0.03), as was the median DLCO (p = 0.04). Conclusions: The use of the GOLD criterion appears to be associated with a higher prevalence of COPD. The agreement between the GOLD and LLN criteria also appears to be good, albeit weaker in older individuals. The use of different criteria to define airway obstruction seems to identify individuals with different characteristics. It is essential to understand the clinical meaning of discordance between such criteria. Until more data are available, we recommend a holistic, individualized approach to, as well as close follow-up of, patients with discordant results for airway obstruction.

RESUMO Objetivo: A identificação de obstrução persistente das vias aéreas é fundamental para o diagnóstico de DPOC. As diretrizes da GOLD sugerem um critério fixo - relação VEF1/CVF pós-broncodilatador < 70% - para definir obstrução, embora outras diretrizes sugiram que a relação VEF1/CVF pós-broncodilatador < o limite inferior da normalidade (LIN) é o critério mais preciso. Métodos: Estudo observacional com indivíduos ≥ 40 anos de idade com fatores de risco para DPOC encaminhados ao nosso laboratório de função pulmonar para espirometria. Também foram registrados sintomas respiratórios. Calculamos a prevalência de obstrução e de ausência de obstrução das vias aéreas segundo o critério GOLD (GOLD+ e GOLD−, respectivamente) e segundo o critério LIN (LIN+ e LIN−, respectivamente). Avaliamos também o grau de concordância entre os dois critérios. Resultados: Foram incluídos 241 indivíduos. Obstrução das vias aéreas foi identificada segundo o critério GOLD em 42 indivíduos (17,4%) e segundo o critério LIN em 23 (9,5%). A concordância global entre os dois critérios foi boa (k = 0,67; IC95%: 0,52-0,81), embora tenha sido menor entre os indivíduos ≥ 70 anos de idade (k = 0,42; IC95%: 0,12-0,72). A proporção de obesos foi menor na categoria GOLD+/LIN+ do que na categoria GOLD+/LIN− (p = 0,03), assim como a mediana de DLCO (p = 0,04). Conclusões: A utilização do critério GOLD parece estar associada a uma maior prevalência de DPOC. A concordância entre os critérios GOLD e LIN também parece ser boa, embora seja mais fraca em indivíduos mais velhos. A utilização de diferentes critérios para definir obstrução das vias aéreas parece identificar indivíduos com diferentes características. É essencial compreender o significado clínico da discordância entre esses critérios. Até que mais dados estejam disponíveis, recomendamos uma abordagem holística e individualizada e também um acompanhamento cuidadoso dos pacientes com resultados discordantes para obstrução das vias aéreas.

Angiosarcoma of the lung.

Angiosarcoma is a rare malignant vascular tumor. Pulmonary involvement is usually attributable to metastasis from other primary sites, primary pulmonary angiosarcoma therefore being quite uncommon. We report a case of angiosarcoma with pulmonary involvement, probably primary to the lung, which had gone untreated for more than two years. We describe this rare neoplasm and its growth, as well as the extensive local invasion and hematogenous metastasis at presentation. We also discuss its poor prognosis.

Angiosarcoma of the lung / Angiossarcoma pulmonar

Angiosarcoma is a rare malignant vascular tumor. Pulmonary involvement is usually attributable to metastasis from other primary sites, primary pulmonary angiosarcoma therefore being quite uncommon. We report a case of angiosarcoma with pulmonary involvement, probably primary to the lung, which had gone untreated for more than two years. We describe this rare neoplasm and its growth, as well as the extensive local invasion and hematogenous metastasis at presentation. We also discuss its poor prognosis.

O angiosarcoma é um tumor vascular maligno. O envolvimento pulmonar é geralmente atribuído à metástase de outros sítios primários, sendo o angiossarcoma pulmonar primário extremamente raro. Relatamos um caso de angiossarcoma com envolvimento pulmonar, provavelmente primário no pulmão com mais de dois anos de evolução. Descrevemos seu crescimento e sua extensa invasão local e hematogênica na apresentação. Documentamos ainda seu mau prognóstico.