Pulmonary Arterial Hypertension with Features of Venous Involvement: A Detective's Task. / Hipertensão Arterial Pulmonar com Características de Envolvimento Venoso: Um Trabalho Investigativo.

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis are rare types of histopathological substrates within the spectrum of pulmonary arterial hypertension (PAH) with a very poor prognosis. They are characterized by a widespread fibroproliferative process of the small caliber veins and/or capillaries with sparing of the larger veins, resulting in a pre-capillary pulmonary hypertension phenotype. Clinical presentation is unspecific and similar to other PAH etiologies. Definitive diagnosis is obtained through histological analysis, although lung biopsy is not advised due to a higher risk of complications. However, some additional findings may allow a presumptive clinical diagnosis of PVOD, particularly a history of smoking, chemotherapy drug use, exposure to organic solvents (particularly trichloroethylene), low diffusing capacity for carbon monoxide (DLCO), exercise induced desaturation, and evidence of venous congestion without left heart disease on imaging, manifested by a classical triad of ground glass opacities, septal lines, and lymphadenopathies. Lung transplant is the only effective treatment, and patients should be referred at the time of diagnosis due to the rapid progression of the disease and associated poor prognosis. We present a case of a 58-year-old man with PAH with features of venous/capillary involvement in which clinical suspicion, prompt diagnosis, and early referral for lung transplantation were determinant factors for the successful outcome.

A doença veno-oclusiva pulmonar (DVOP) e a hemangiomatose capilar pulmonar são tipos raros de substratos histopatológicos dentro do espectro da hipertensão arterial pulmonar (HAP) com prognóstico muito ruim. Caracterizam-se por um processo fibroproliferativo generalizado das veias e/ou capilares de pequeno calibre com preservação das veias maiores, resultando em um fenótipo de hipertensão pulmonar pré-capilar. A apresentação clínica é inespecífica e semelhante a outras etiologias de HAP. O diagnóstico definitivo é obtido por meio de análise histológica, embora a biópsia pulmonar não seja aconselhada devido ao maior risco de complicações. No entanto, alguns achados adicionais podem permitir um diagnóstico clínico presuntivo de DVOP, especialmente história de tabagismo, uso de drogas quimioterápicas, exposição a solventes orgânicos (particularmente tricloroetileno), baixa capacidade de difusão do monóxido de carbono (DLCO), dessaturação ao esforço e evidências de doença venosa sem doença cardíaca esquerda no exame de imagem, manifestada por uma tríade clássica de opacidades em vidro fosco, linhas septais, e linfadenopatias. O transplante pulmonar é o único tratamento eficaz e os pacientes devem ser encaminhados no momento do diagnóstico, devido à rápida progressão da doença e ao prognóstico ruim. Apresentamos o caso de um homem de 58 anos com HAP com características de envolvimento venoso/capilar em que a suspeita clínica, o pronto diagnóstico e o encaminhamento precoce para transplante pulmonar foram determinantes para um bom desfecho.

Segmentation-Based Analysis of T2- and T1-Weighted Dynamic Magnetic Resonance Images Provides Adequate Observer Agreement in the Evaluation of Interstitial Lung Disease.

OBJECTIVE: The aim of the study is to quantify observer agreement in the magnetic resonance imaging (MRI) classification of inflammatory or fibrotic interstitial lung disease (ILD). METHODS: Our study is a preliminary analysis of a larger prospective cohort. The MRI images of 18 patients with ILD (13 females; mean age, 65 years) were acquired in a 1.5 T scanner and included axial fat-saturated T2-weighted (T2-WI, n = 18) and coronal fat-saturated T1-weighted images before and 1, 3, 5, and 10 minutes after gadolinium administration (n = 16). The MRI studies were evaluated with 2 different methods: a qualitative evaluation (visual assessment and measurement of few regions of interest; evaluations were performed independently by 5 radiologists and 3 times by 1 radiologist) and a segmentation-based analysis with software extraction of signal intensity values (evaluations were performed independently by 2 radiologists and twice by 1 radiologist). Interstitial lung disease was classified as inflammatory or fibrotic, based on previously described imaging criteria. RESULTS: Regarding the qualitative evaluation, intraobserver agreement was excellent (κ = 0.92, P < 0.05) for T2-WI and fair (κ = 0.29, P < 0.05) for T1 dynamic study, while interobserver agreement was moderate (κ = 0.56, P < 0.05) and poor (κ = 0.11, P = 0.18), respectively. In contrast, upon segmentation-based analysis, intraobserver and interobserver agreement were excellent for T2-WI (κ = 0.886, P < 0.001; κ = 1.00, P < 0.001; respectively); for T1-WI, intraobserver agreement was excellent (κ = 0.87, P < 0.05) and interobserver agreement was good (κ = 0.75, P < 0.05). CONCLUSIONS: Segmentation-based MRI analysis is more reproducible than a qualitative evaluation with visual assessment and measurement of few regions of interest.

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.

Erasmus Syndrome: An Underrecognized Entity.

We present a case of a 33-year-old male who worked as a plumber and a locksmith. The patient presented with diffuse myalgia and asthenia, skin sclerosis and puffy fingers, Raynaud's phenomenon, exertional dyspnea and erectile dysfunction. The presence of specific autoantibodies enabled the diagnosis of systemic sclerosis. Chest-computed tomography revealed upper lobe consolidation. After extensive evaluation, the multidisciplinary interstitial lung disease team concluded that the patient also had advanced silicosis. After a year, there was significant clinical, radiologic, and functional deterioration of the lung disease. The patient was referred for lung transplant. Silica inhalation is the cause of silicosis but is also implicated in the development of systemic sclerosis (Erasmus syndrome). Although they share a common risk factor, it is rare to find both diseases co-existing. We present this case of a young patient where both diseases presented aggressively in order to raise awareness to this association.

Apresentamos o caso de um homem de 33 anos que trabalhava como canalizador e serralheiro. Apresentava mialgias e astenia, esclerose cutânea e puffy fingers, fenómeno de Raynaud, dispneia de esforço e disfunção erétil. A presença de autoanticorpos específicos permitiu o diagnóstico de esclerose sistémica. A tomografia computadorizada de tórax revelou consolidações dos lobos superiores. Após extensa avaliação, o grupo multidisciplinar de doenças do interstício concluiu que o doente tinha também silicose avançada. Após um ano, houve agravamento clínico, radiológico e funcional significativo da doença pulmonar. O doente foi encaminhado para transplante pulmonar. A inalação de sílica é a causa da silicose, mas também está implicada no desenvolvimento da esclerose sistémica (síndrome de Erasmus). Embora tenham um fator de risco comum, é raro encontrar as duas doenças simultaneamente. Apresentamos o caso de um doente jovem em que ambas as doenças se apresentaram de forma agressiva para alertar sobre esta associação.

A disease with many faces.

Can you diagnose this man with progressively worsening shortness of breath, mucous productive cough, weight loss, fatigue and a history of suspected pulmonary tuberculosis? http://bit.ly/2VUdnTr.

Prevalence and clinical characterisation of patients with severe partial respiratory failure admitted to an intensive care unit.

OBJECTIVE: The authors analysed patients with severe partial respiratory failure (SPRF) admitted to a general Intensive Care Unit (ICU). The prevalence and clinical characteristics of these patients were evaluated. This work aims to study the rate of and to clinically characterise the patient population admitted to an Intensive Care Unit with acute severe partial respiratory failure. MATERIAL AND METHODS: In 16-bed ICU of a central Hospital in Lisbon, patients admitted in the year 2004 were analysed. Patients with SPRF were recruited form patients with an ICU stay> 24 hours. They were selected according to PaO2 and FiO2 and clinically characterized. RESULTS: During the study period 472 patients were admitted, and 378 presented an ICU stay> 24 hours and were enrolled. From those, 142 (37.6%) met criteria for SPRF. Of these, 45 (31.7%) a pulmonary aetiology of SPRF was identified. Patients with SRPF were older, had longer ICU stay, and presented higher severity indexes and mortality. The prevalence of adult respiratory distress syndrome was possible to evaluate in the deceased patients with SPRF (n=52). In these we could find 12 (23%) patients that met criteria for that entity. By multivariate analysis the mortality of patients with SRPF correlated with older age and the presence of circulatory failure (p<0.001). CONCLUSIONS: SPRF is a situation highly prevalent in the ICU studied. To better understand the prevalence of this entity, properly designed studies are needed in order to establish its epidemiology and clinical characteristics.

Non invasive evaluation of central venous pressure using echocardiography in the intensive care--specific features of patients with right ventricular enlargement and chronic exacerbated pulmonary disease.

OBJECTIVES: To determine the possibility of non-invasive estimation of central venous pressure (CVP) through inferior vena cava (IVC) analysis, using transthoracic echocardiography (TTE). DESIGN: A prospective 3-year study. SETTING: A 16-bed medical/surgical Intensive Care Unit (ICU). METHODS: Patients admitted to the ICU were enrolled. CVP measurement and TTE (determining cardiac chambers dimension and left ventricular shortening fraction) with IVC analysis (maximum dimension and IVC index) were performed simultaneously. Parametric and non-parametric statistical analysis was performed to establish correlations between variables. RESULTS: 560 patients were admitted to the study, including 477 in whom IVC was analysed, aging 62.2 +/- 17.3 years, a mean ICU stay 11.9 +/- 18.7 days, a APA- CHE II score 23.9 +/- 8.9 and a SAPS II score 55.7 +/- 20.4. Through linear regression analysis CVP was influenced by IVC index (p=0.001), IVC maximum dimension (p=0.013) and presence of mechanical ventilation (p=0.002). A statistically significant correlation was found between the following parameters: an IVC index < 25% and a CVP > 13 mmHg; an IVC index and a CVP 26%-50%; an IVC index > 51% and CVP < 7 mmHg; an IVC maximum dimension > 20mm and a CVP > 13 mmHg; an IVC maximum dimension < 10 mmHg and CVP < 7 mmHg. Patients with right ventricle enlargement presented a lack of agreement between IVC maximum dimension and CVP > 7 mmHg was observed, and in patients with chronic respiratory failure (who presented a high prevalence of right ventricular enlargement) a lack of agreement between IVC index > 50% and CVP < 7 mmHg was also observed. CONCLUSIONS: IVC analysis is a possible way to non-invasively estimate CVP in a medical /surgical ICU. However, patients with right ventricular enlargement and admitted with chronic respiratory failure present a lack of agreement between IVC parameters and low values of CVP. IVC dimension is a marker of chronic disease and IVC index correlated better with CVP.

[Oral steroids effects on the respiratory muscles function in severe asthmatic patients]. / Efeito da corticoterapia oral na função dos músculos respiratórios dos doentes com asma brônquica grave.

INTRODUCTION: Several studies suggest that severe asthmatic patients under oral steroids show higher risk of myopathy induced by steroids. Nevertheless, no conclusive data exist about the prolonged oral steroid therapy effects on the res- piratory muscles function. We didn't found correlation between the severity of obs- truction (evaluated by FEV1), hyperinflation (residual volu- me- RV) and the muscle pressures in both groups. Also, there were no significant correlation between the G1 PImax and oral steroid accumulated dose. CONCLUSION: We found a significant compromise of the res- piratory muscle function in the both studied groups with severe asthma. The oral steroid therapy caused no additional adverse effect in muscle respiratory function even after a prolon- ged treatment. The compromise of the muscle respiratory function foun- ded can be justified by the prolonged exposition to inhaled steroids. Key-words: Asthma, respiratory muscles, oral steroids.