Progressive fibrotic interstitial lung disease.

Many interstitial lung diseases (ILDs) share mechanisms that result in a progressive fibrosing phenotype. In Brazil, the most common progressive fibrosing interstitial lung diseases (PF-ILDs) are chronic hypersensitivity pneumonitis, idiopathic pulmonary fibrosis, unclassified ILD, and connective tissue diseases. PF-ILD is seen in approximately 30% of patients with ILD.

Transbronchial biopsy in chronic hypersensitivity pneumonitis.

Introduction: The diagnosis of chronic hypersensitivity pneumonitis (CHP) is based on relevant exposure, tomographic findings and, in some cases, pathological data. The role of bronchoscopy is uncertain, especially in the fibrotic form of CHP.

Aim: To analyze the yield of transbronchial biopsy (TBBx) in patients with CHP according to tomographic findings and to evaluate the importance of bronchoalveolar lavage (BAL) in the diagnostic approach.

Executive Summary: Diagnosis and Evaluation of Hypersensitivity Pneumonitis: CHEST Guideline and Expert Panel Report.

Background: The purpose of this summary is to provide a synopsis of evidence-based and consensus-derived guidance for clinicians to improve individual diagnostic decision-making for hypersensitivity pneumonitis (HP) and decrease diagnostic practice variability.

Study Design And Methods: Approved panelists developed key questions regarding the diagnosis of HP using the PICO (Population, Intervention, Comparator, and Outcome) format. MEDLINE (via PubMed) and the Cochrane Library were systematically searched for relevant literature, which was supplemented by manual searches.

Diagnosis and Evaluation of Hypersensitivity Pneumonitis: CHEST Guideline and Expert Panel Report.

Background: The purpose of this analysis is to provide evidence-based and consensus-derived guidance for clinicians to improve individual diagnostic decision-making for hypersensitivity pneumonitis (HP) and decrease diagnostic practice variability.

Study Design And Methods: Approved panelists developed key questions regarding the diagnosis of HP using the PICO (Population, Intervention, Comparator, Outcome) format. MEDLINE (via PubMed) and the Cochrane Library were systematically searched for relevant literature, which was supplemented by manual searches.

Uncovering the mechanisms of exertional dyspnoea in combined pulmonary fibrosis and emphysema.

The prevailing view is that exertional dyspnoea in patients with combined idiopathic pulmonary fibrosis (IPF) and emphysema (CPFE) can be largely explained by severe hypoxaemia. However, there is little evidence to support these assumptions.We prospectively contrasted the sensory and physiological responses to exercise in 42 CPFE and 16 IPF patients matched by the severity of exertional hypoxaemia.

Squawks in interstitial lung disease prevalence and causes in a cohort of one thousand patients.

Squawks are lung adventitious sounds with a mix of both musical and nonmusical components heard during the inspiratory phase. Small series have described squawks in interstitial lung diseases. Hypersensitivity pneumonitis and other diseases involving small airways can result in squawks, but new interstitial lung diseases (ILDs) involving peripheral airways are being described.

Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis.

The predictive value of the decline in FVC by ≥10% on survival in patients with fibrotic hypersensitivity pneumonitis is unknown. Of 112 patients included, 66 (59%) had surgical lung biopsies. Patients with ≥10% decline in predicted FVC after 6-12 months had a significantly increased risk of all-cause mortality (median survival 53 months, 95% CI 37 to 69 vs 139 months, 95% CI 66 to 212 months, p=0.

Diagnostic Approach to Advanced Fibrotic Interstitial Lung Disease: Bringing Together Clinical, Radiologic, and Histologic Clues.

Context: - Idiopathic pulmonary fibrosis (IPF) is a distinctive clinicopathologic entity and the most common form of progressive diffuse lung scarring in older adults. Idiopathic pulmonary fibrosis manifests histopathologically as the usual interstitial pneumonia pattern. The usual interstitial pneumonia pattern is distinguished by geographically and temporally heterogeneous fibrosis that is peripherally accentuated, often with honeycombing and traction bronchiectasis.

Cytological analysis of bronchoalveolar lavage in patients with interstitial lung diseases and the relation of cytological analysis to fibrosis in high-resolution computed tomography.

Objective: To evaluate the value of differential cytology of bronchoalveolar lavage (BAL) in common interstitial lung diseases (ILDs) and its relation with high-resolution computed tomography (HRCT) fibrosis.

Study Design: A retrospective analysis of BAL from 91 patients. The total and percentage of cells were collected and analyzed according to published guidelines.

Clinical phenotypes in sarcoidosis.

Purpose Of Review: To describe the methods for derivation of clinical phenotypes in general and how they should be applied in sarcoidosis, taking into account the dimensions of the disease. The results from the small number of studies in this area are summarized.

Recent Findings: Clinical phenotypes are determined by diverse ways.

A haemodynamic study of pulmonary hypertension in chronic hypersensitivity pneumonitis.

Chronic hypersensitivity pneumonitis is a common fibrotic interstitial lung disease. The prevalence of pulmonary hypertension diagnosed by right heart catheterisation and its cardiopulmonary function findings in patients with chronic hypersensitivity pneumonitis are unknown. Consecutive symptomatic patients with chronic hypersensitivity pneumonitis were prospectively evaluated.

Reference values for spirometry in preschool children.

Objectives: Reference values for lung function tests differ in samples from different countries, including values for preschoolers. The main objective of this study was to derive reference values in this population.

Methods: A prospective study was conducted through a questionnaire applied to 425 preschool children aged 3 to 6 years, from schools and day-care centers in a metropolitan city in Brazil.

Sarcoidosis Masquerading as Atrial Fibrillation: Interesting Case Discussion as Well as Recent Advances in Diagnosis and Management of Cardiac Sarcoidosis.

This report presents a case of cardiac sarcoidosis initially manifested with atrial fibrillation. This behavior is very uncommon in spite of the fact that the disease is multisystemic, affecting predominantly the lungs. It is emphasized that the diagnosis of the cardiac involvement is difficult, and when this occurs, can lead to conducting system disturbances, heart failure or sudden death (SD).

Subacute and chronic hypersensitivity pneumonitis: histopathological patterns and survival.

Background: In hypersensitivity pneumonitis (HP), survival can be predicted on the basis of the severity of fibrosis in surgical lung biopsy, but few data are available on the influence of clinical, functional, tomographic and histologic findings on prognosis.

Objectives: To describe the impact on survival of clinical data, histological patterns, and HRCT findings in subacute/chronic HP.

Methods: A retrospective analysis of 103 patients diagnosed with HP submitted to surgical lung biopsy.

Survival in idiopathic pulmonary fibrosis-cytotoxic agents compared to corticosteroids.

Study Objective: To compare the survival of patients with IPF treated retrospectively with corticosteroids alone, to survival of patients treated with immunosuppressive and corticosteroids combined.

Design: Non-randomized retrospective cohort study.

Setting: Three tertiary centers in Brazil.

[Bronchial provocation tests using methacholine, cycle ergometer exercise and free running in children with intermittent asthma].

Objective: To compare airway responsiveness to methacholine, cycle ergometer exercise and free running in children with intermittent asthma.

Methods: A randomized study was conducted with 30 children of both genders with intermittent asthma. Each child was submitted to challenge testing on three separate days, in random order: a) Methacholine challenge using a dosimeter; b) Exercise challenge testing -- free running along a 50-meter-long corridor; c) Dry-air exercise challenge on a cycle ergometer.

Clinical, radiographic and functional predictors of pulmonary gas exchange impairment at moderate exercise in patients with sarcoidosis.

Background: Pulmonary gas exchange impairment (GEI) is a common consequence of intrathoracic sarcoidosis presenting with important therapeutic and prognostic implications.

Objective: To determine the role of clinical, radiographic and functional variables in predicting GEI during moderate exercise at the estimated lactate threshold (theta(L)) in patients with sarcoidosis.

Methods: Fifty-four outpatients (29 females) with biopsy-proven sarcoidosis had clinical evaluation (baseline dyspnea index), lung function tests and an incremental cardiopulmonary exercise test with theta(L) estimation.