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=0.007). On multivariate analysis remained associated with increasing mortality: decline in FVC by ≥10% (HR 4.13, 95% CI 1.96 to 8.70, p=0.005), lower FVC% (HR 1.03, 95% CI 1.01 to 1.05, p=0.003) and with decreasing mortality improvement with antigen avoidance (HR 0.18, 95% CI 0.04 to 0.77, p=0.021).

Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.Keywords: Lung diseases, interstitial; Guidelines as topic; Brazil. ResumoAs doenças pulmonares intersticiais (DPIs) são afecções heterogêneas, envolvendo um elevado número de condições, cuja abordagem ainda é um grande desafio para o pneumologista. As Diretrizes de DPIs da Sociedade Brasileira de Pneumologia e Tisiologia, publicadas em 2012, foram estabelecidas com o intuito de fornecer aos pneumologistas brasileiros um instrumento que possa facilitar a abordagem dos pacientes com DPIs, padronizando-se os critérios utilizados para a definição diagnóstica das diferentes condições, além de orientar sobre o melhor tratamento nas diferentes situações. Esse artigo teve como objetivo descrever resumidamente os principais destaques dessas diretrizes.Descritores: Doenças pulmonares intersticiais; Guias como assunto; Brasil.

The oculoauriculovertebral spectrum (OAVS) is characterized by anomalies involving the development of the first and second pharyngeal arches during the embryonic period. The phenotype is highly heterogeneous, involving ears, eyes, face, neck, and other systems and organs. There is no agreement in the literature for the minimum phenotypic inclusion criteria, but the primary phenotype involves hemifacial microsomia with facial asymmetry and microtia. Most cases are sporadic and the etiology of this syndrome is not well known. Environmental factors, family cases that demonstrate Mendelian inheritance, such as preauricular appendages, microtia, mandibular hypoplasia, and facial asymmetry; chromosomal abnormalities and some candidate genes suggest a multifactorial inheritance model. We evaluated clinical, cytogenomic and molecularly 72 patients with OAVS, and compared our findings with patients from the literature. We found 15 CNVs (copy number variations) considered pathogenic or possibly pathogenic in 13 out of 72 patients. Our results did not indicated a single candidate genomic region, but recurrent chromosomal imbalances were observed in chromosome 4 and 22, in regions containing genes relevant to the OAVS phenotype or related to known OMIM diseases suggesting different pathogenic mechanisms involved in this genetically and phenotypic heterogeneous spectrum.

BackgroundAirway-centered Interstitial Fibrosis (ACIF) is a common pathologic pattern observed in our practice.ObjectivesThe objectives of this study are to describe the causes associated with ACIF in a large sample of patients and its effect on survival.MethodsA retrospective study in three centers of interstitial lung disease in São Paulo, between January of 1995 and December of 2012. The surgical lung biopsy specimens were reviewed by three pathologists. The clinical, functional and tomographic findings were analyzed by a standardized protocol.ResultsThere were 68 cases of ACIF, most of them women. The mean age was 57 ± 12 yr. Dyspnea, cough, restrictive pattern at spirometry and oxygen desaturation at exercise were common. A reticular pattern with peribronchovascular infiltrates was found in 79% of the cases. The etiologies of ACIF were hypersensitivity pneumonitis in 29 (42.6%), gastroesophageal reflux disease in 17 (25.0%), collagen vascular disease in 4 (5.9%), a combination of them in 15 cases and idiopathic in 3 (4.4%). The median survival was 116 months (95% CI = 58.5 – 173.5). Lower values of oxygen saturation at rest, presence of cough and some histological findings - organizing tissue in the airways, fibroblastic foci and microscopic honeycombing - were predictors of worse survival.ConclusionsACIF is an interstitial lung disease with a better survival when compared with IPF. The main etiologies are HP and GERD. The oxygen saturation at rest, the presence of cough and some histological findings are predictors of survival.

OBJECTIVE: To evaluate the spirometry values predicted by the 2012 Global Lung Function Initiative (GLI) equations, which are recommended for international use, in comparison with those obtained for a sample of White adults used for the establishment of reference equations for spirometry in Brazil. METHODS: The sample comprised 270 and 373 healthy males and females, respectively. The mean differences between the values found in this sample and the predicted values calculated from the GLI equations for FVC, FEV1, and VEF1/FVC, as well as their lower limits, were compared by paired t-test. The predicted values by each pair of equations were compared in various combinations of age and height. RESULTS: For the males in our study sample, the values obtained for all of the variables studied were significantly higher than those predicted by the GLI equations (p < 0.01 for all). These differences become more evident in subjects who were shorter in stature and older. For the females in our study sample, only the lower limit of the FEV1/FVC ratio was significantly higher than that predicted by the GLI equation. CONCLUSIONS: The predicted values suggested by the GLI equations for White adults were significantly lower than those used as reference values for males in Brazil. For both genders, the lower limit of the FEV1/FVC ratio is significantly lower than that predicted by the GLI equations.

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The use of 15 mg of midazolam made no difference compared with the placebo. The use of 15 mg of midazolam did not show an advantage in the incidence of arrhythmias The anxiolytic premedication does not prevent myocardial arrhythmias in endosseous implant placement. The clinical significance of the arrhythmias may not represent serious risks.

Objective:To determine the cut-off point for the six-minute walk distance (6MWD) that indicates lower survival time in patients with idiopathic pulmonary fibrosis (IPF) in Brazil. Methods:This was retrospective study carried out in two referral centers for IPF. The 6MWT was performed twice, considering the highest value of the 6MWD. Various cut-off points were estimated, in absolute values and in percentage of predicted values, using ROC curves, the Kaplan-Meier method, and data from other studies. Results:The sample comprised 70 patients with IPF. The mean age was 71.9 ± 6.4 years, and 50 patients (71.4%) were male. The mean FVC was 76.6 ± 18.2% of predicted value. The mean SpO2 at rest before and after 6MWT were 93.8 ± 2.5% and 85.3 ± 6.5%, respectively. The median survival time was 44 months (95% CI: 37-51 months). The mean 6MWD was 381 ± 115 m (79.2 ± 24.0% of predicted). After the analyses, the best cut-off points for estimating survival were 6MWD < 330 m and < 70% of predicted. The median survival time of patients with a 6MWD < 330 m was 24 months (95% CI: 3-45 months), whereas that of those with a 6MWD ≥ 330 m was 59 months (95% CI: 41-77 months; p = 0.009). Similarly, the median survival times of those with a 6MWD < 70% and ≥ 70% of predicted, respectively, were 24 months (95% CI: 13-35 months) and 59 months (95% CI: 38-80 months; p = 0.013). Cox multivariate regression models including age, sex, smoking status, SpO2 at the end of the 6MWT, and FVC% showed that 6MWD remained significantly associated with survival (p = 0.003). Conclusions:Values of 6MWD < 330 m and < 70% of predicted value were associated with lower survival time in IPF patients in Brazil.