Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) is a progressive interstitia pulmonary disease of unknown etiology. Since Hamman's and Rich's (1944) reports, many studies have tried to find a histological marker for the correlation between prognosis and response to therapy. However, there are many doubts regarding pathogenesis. In addition, it is generally accepted that response to therapy is related to the relative degree of cellularity and fibrosis. The purpose of this study is to describe the results of inflammatory/exudative changes, fibrotic/reparative changes, and airway alterations, using a semi-quantitative method by independent evaluation of two pathologists, in 24 open lung biopsies with the diagnosis of idiopathic pulmonary fibrosis. Fourteen histological features were analyzed using the 0 to 5 scale for interstitial alterations and the 0 to 2 scale for the airway changes. There was significant interobserver agreement for all histological features with Kw (Kappa) variations between 0.47 and 0.92. There was significant disagreement only for septal inflammatory intensity analysis, suggesting that these features must be discussed by the pathologists. The semi-quantitative method assessment was effective.

Keywords: Idiopathic pulmonary fibrosis (IPF). Usual interstitial pneumonia (UIP). Semi-quantitative method assessment.

Objective: To determine the frequency of and the factors related to delayed diagnosis of sarcoidosis in Brazil. Methods: We evaluated patients with a biopsy-proven diagnosis of sarcoidosis, using a questionnaire that addressed the following: time since symptom onset and since the first medical visit; and the number and specialty of the physicians visited. We divided the patients by the timeliness of the diagnosis-timely (< 6 months) and delayed ( 6 months)-comparing the two groups in terms of systemic and pulmonary symptoms; extrathoracic involvement; spirometric data; radiological staging; level of education; income; and tuberculosis (diagnosis and treatment). Results: We evaluated 100 patients. The median number of physicians consulted was 3 (range, 1-14). In 11 cases, sarcoidosis was diagnosed at the first visit. In 54, the first physician seen was a general practitioner. The diagnosis of sarcoidosis was timely in 41 patients and delayed in 59. The groups did not differ in terms of gender; race; type of health insurance; level of education; income; respiratory/systemic symptoms; extrathoracic involvement; and radiological staging. In the delayed diagnosis group, FVC was lower (80.3 ± 20.4% vs. 90.5 ± 17.1%; p = 0.010), as was FEV1 (77.3 ± 19.9% vs. 86.4 ± 19.5%; p = 0.024), misdiagnosis with and treatment for tuberculosis ( 3 months) also being more common (24% vs. 7%, p = 0.032, and 20% vs. 0%; p = 0.002, respectively). Conclusions: The diagnosis of sarcoidosis is often delayed, even when the imaging is suggestive of sarcoidosis. Delayed diagnosis is associated with impaired lung function at the time of diagnosis. Many sarcoidosis patients are misdiagnosed with and treated for tuberculosis.

Keywords: Sarcoidosis; Sarcoidosis, pulmonary/diagnosis; Tuberculosis.

The incidence of lung neoplasms is increasing in Brazil and in the world, probably as a result of the increase in smoking. Due to the greater number of cases, atypical presentations appear. We report the case of a 66-year-old hypertensive male smoker who presented progressive proximal muscular weakness and, in two months, evolved to dysphagia, dysphonia, and V-shaped skin lesions on the chest. A chest X-ray showed a spiculated pulmonary nodule in the right upper lobe. The biochemical analysis revealed elevated creatine kinase levels. After complementary tests and biopsies, the patient underwent right upper lobectomy. Histopathology showed a moderately differentiated adenocarcinoma. The overall analysis of the case and a review of the literature allow us to suggest that the clinical profile of the patient was a result of an overlap of two paraneoplastic syndromes (dermatomyositis and Lambert-Eaton myasthenic syndrome) secondary to lung adenocarcinoma.

Keywords: Lung neoplasms; Paraneoplastic syndromes; Dermatomyositis; Lambert-Eaton myasthenic syndrome; Adenocarcinoma.

Objective: To report the cases of four patients with bronchiolitis caused by exposure to artificial butter flavoring at a cookie factory in Brazil. Methods: We described the clinical, tomographic, and spirometric findings in the four patients, as well as the lung biopsy findings in one of the patients. Results: All four patients were young male nonsmokers and developed persistent airflow obstruction (reduced FEV1/FVC ratio and FEV1 at 25-44% of predicted) after 1-3 years of exposure to diacetyl, without the use of personal protective equipment, at a cookie factory. The HRCT findings were indicative of bronchiolitis. In one patient, the surgical lung biopsy revealed bronchiolitis obliterans accompanied by giant cells. Conclusions: Bronchiolitis resulting from exposure to artificial flavoring agents should be included in the differential diagnosis of airflow obstruction in workers in Brazil.

Keywords: Diacetyl; Flavoring agents; Bronchiolitis.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with airflow obstruction is a rare form of lung injury. All of the reported cases have been diagnosed by surgical lung biopsy. Only three of the reported cases presented with diffuse interstitial lung opacities on HRCT scans. We report three additional cases of this entity. All of the patients were female and presented with mild-to-moderate airflow obstruction. In the first case, transbronchial biopsy and imaging data were sufficient to make the diagnosis. Although the HRCT scans of all three cases revealed a mosaic pattern, that of the third patient also revealed diffuse interstitial infiltrate. In extremely rare cases, HRCT findings can simulate those seen in other interstitial lung diseases.

Keywords: Neuroendocrine cells; Carcinoid tumor; Bronchiolitis obliterans.

Background: Idiopathic pulmonary fibrosis was recently redefined as usual interstitial pneumonia of unknown etiology. Consequently, the prognostic value of histological findings needs to be reassessed. Objective: To correlate clinical, functional and histological findings with survival in patients with idiopathic pulmonary fibrosis. Method: Patients (n = 51; mean age: 66 ± 8 years; gender: 21 females/30 males) were evaluated. Of the 51, 26 were smokers or ex-smokers. Duration of symptoms, forced vital capacity and smoking habits were recorded. All patients presented usual interstitial pneumonia verified through histology. Degree of honeycombing, established fibrosis, desquamation, cellularity, myointimal thickening of blood vessels and number of fibroblastic foci were graded according to the semiquantitative method. Results: Median duration of symptoms was 12 months and initial forced vital capacity was 72 ± 21%. Cox multivariate analysis revealed that survival correlated inversely and significantly (p < 0.05) with duration of symptoms and fibroblastic foci score, as well as with myointimal thickening of blood vessels. Limited numbers of fibroblastic foci, as well as myointimal thickening involving less than 50% of blood vessels, were predictive of greater survival. No correlation with survival was found for gender, age, forced vital capacity, inflammation or degree of cellularity. Conclusion: Semiquantitative analysis of lung biopsies yields relevant prognostic information regarding patients with usual interstitial pneumonia.

Keywords: Pulmonary fibrosis. Lung diseases, interstitial. Survival analysis.

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.