On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung parenchyma and a well-defined interface with the normal adjacent lung. The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. In a retrospective analysis of the HRCT findings in 50 patients diagnosed with chronic paracoccidioidomycosis, we found lung cysts in 5 cases (10%), indicating that patients with paracoccidioidomycosis can present with lung cysts on HRCT scans. Therefore, paracoccidioidomycosis should be included in the differential diagnosis of cystic lung diseases.

Keywords: Paracoccidioidomycosis; Cysts; Multidetector computed tomography.

Objective: To evaluate the treatment compliance of patients with paracoccidioidomycosis. Methods: We studied 188 patients with paracoccidioidomycosis admitted to a tertiary referral hospital in the Central-West Region of Brazil from 2000 to 2010, to assess their compliance to treatment. In order to be considered compliant, patients needed to present two established criteria: (1) receive medicines from the pharmacy, and (2) achieve a self-reported utilization of at least 80% of the dispensed antifungal compounds prescribed since their previous appointment. Results: Most patients were male (95.7%), had the chronic form of the disease (94.2%), and were treated with cotrimoxazole (86.2%). Only 44.6% of patients were treatment compliant. The highest loss to follow-up was observed in the first 4 months of treatment (p < 0.02). Treatment compliance was higher for patients with than for those without pulmonary involvement (OR: 2.986; 95%CI 1.351-6.599), and higher for patients with than without tuberculosis as co-morbidity (OR: 2.763; 95%CI 1.004-7.604). Conclusions: Compliance to paracoccidioidomycosis treatment was low, and the period with the highest loss to follow-up corresponds to the first four months. Pulmonary paracoccidioidal involvement or tuberculosis comorbidity predicts a higher compliance to paracoccidioidomycosis therapy.

Keywords: Paracoccidioidomycosis; Treatment adherence and compliance; loss to follow-up; mycoses

Paracoccidioidomycosis is a systemic mycosis caused by the dimorphic fungus Paracoccidioides brasiliensis. The disease is restricted to Latin America. It is the principal systemic mycosis in Brazil, with higher incidences in the southern, southeastern and central regions. The disease is acquired by inhaling fungal propagules. In endemic areas, the primary infection occurs during childhood and involves the immune system. The most common chronic form of paracoccidioidomycosis in adults is the multifocal form, in which there is dissemination to the lungs, lymph nodes, skin and mucosae. This form of the disease has a chronic progression, and the diagnosis is typically delayed. Cough, dyspnea and weight loss due to cutaneous and mucosal lesions are evident and are the principal complaints reported by paracoccidioidomycosis patients. Chest X-rays reveal diffuse reticulonodular infiltrates, which are more evident in the upper lobes. The etiologic diagnosis is based on the identification of P. brasiliensis in clinical specimens, such as lymph node aspirates or BAL fluid, by direct microscopy and culture. Histopathological testing of tissue samples reveals the thick birefringent cell wall of the fungus and the typical pattern of multiple budding around the mother cell. Double agar gel immunodiffusion is useful for the diagnosis when the fungus cannot be detected through mycological tests. Although paracoccidioidomycosis is most often treated with the sulfamethoxazole-trimethoprim combination, itraconazole is preferable. Amphotericin B is used in severe cases.

Keywords: Paracoccidioidomycosis; Mycosis; Lung diseases, fungal.

Objective: To analyze the association between paracoccidioidomycosis (Pcm) and cancer in a series of 25 cases and to review the literature on this topic. Methods: A retrospective review of 25 cases diagnosed with Pcm and cancer, retrieved from a series of 808 consecutive adult patients diagnosed with Pcm based on tests conducted in the Mycology Laboratory of the Santa Casa Complexo Hospitalar, in the city of Porto Alegre, Brazil, between 1972 and 2007. The diagnosis of Pcm was confirmed by means of direct microscopic examination, histopathological examination or immunodiffusion test. All cancer cases were confirmed by histopathological or cytopathological examination. Results: Respiratory symptoms were the principal complaints of the patients evaluated. Pulmonary involvement predominated, followed by skin and lymph node involvement. The most prevalent tumor was bronchial carcinoma, in 15 patients, followed by other types of carcinoma, and 1 patient had Hodgkin's lymphoma. In 16 patients (64%), the site of the Pcm was the same as that of the tumor. In most cases, Pcm treatment consisted of the isolated administration of sulfanilamide, sulfamethoxazole-trimethoprim, ketoconazole, itraconazole or amphotericin B. The most common treatment for cancer was surgery, followed by radiotherapy and chemotherapy. Of the 25 patients, 12 were cured of Pcm, and 4 died. In 9 patients, the final outcome was unknown. In the general population of the area under study, the prevalence of lung cancer was significantly higher in smokers with Pcm than in smokers without Pcm (p < 0.001). Conclusions: A diagnosis of Pcm appears to increase the risk of lung cancer.

Keywords: Paracoccidioides; Paracoccidioidomycosis; Neoplasms.

Primary infection with Paracoccidioides brasiliensis has rarely been observed. A 28-year-old male patient presented with a three-month history of fever, respiratory symptoms and malaise. Chest X-rays revealed bilateral apical infiltrates, right pleuritis and hilar lymphadenomegaly. The patient presented with leukocytosis, severe eosinophilia and increasing titers of anti-P. brasiliensis antibodies in serum. To our knowledge, this is the first report of the primary pulmonary lymph node complex of paracoccidioidomycosis accompanied by hypereosinophilia and affecting a previously healthy adult.

Keywords: Paracoccidioidomycosis; Eosinophilia; Lung diseases, fungal.

Objective: To evaluate the frequency of the real association between paracoccidioidomycosis (PCM) and tuberculosis (TB) as well as the rate of previous TB misdiagnosis in individuals with PCM among the patients treated in the Pulmonology Division of the State University of Campinas Hospital das Clínicas, Campinas, Brazil. Methods: A retrospective study of 227 adult patients with PCM (chronic form) treated between 1980 and 2005. Results: Of the 227 patients studied, 36 (15.8%) had been previously treated for TB. However, only 18 (7.9%) presented positive sputum smear microscopy results. The remaining 18 (7.9%) neither presented positive sputum smear microscopy nor showed improvement after receiving specific anti-TB treatment. Conclusion: Although the existence of an association between PCM and TB has been documented in the literature, misdiagnosis is common due to the superimposition of and the similarity between their clinical and radiographic presentations, thereby warranting the need for bacteriological diagnosis before initiating specific treatment.

Keywords: Paracoccidioidomycosis; Tuberculosis, pulmonary; Diagnosis, differential.

The incidence of pulmonary fungal infections is very low in Uruguay, and such infections typically affect immunocompromised patients. We report the case of an immunocompetent patient presenting with a two-month history of cough, dyspnea, and fever. The patient resided in a rural area. Imaging tests revealed extensive pneumonitis and pulmonary fibrosis. On the basis of direct mycological examination, culture, and serological testing, we made a diagnosis of concomitant histoplasmosis and paracoccidioidomycosis. The patient presented arterial hypotension that was diagnostic of adrenocortical insufficiency. Although the pulmonary fibrosis and pneumonia were irreversible, the clinical condition of the patient improved after antifungal treatment. This was an exceptional case of two pulmonary fungal infections occurring simultaneously in the same patient.

Keywords: Paracoccidioidomycosis; Histoplasmosis; Lung diseases, fungal.

Interstitial lung diseases (ILDs) in children constitute a heterogeneous group of rare diseases that have been described and classified according to experiences and research in adults. However, pediatric pulmonologists have observed that the clinical spectrum is broader in children than in adults, and that many of these disorders have different courses and treatment responses. In addition, probably due to the various stages of lung development and maturation, new clinical forms have been described, particularly in infants. This has broadened the classification of ILDs in this age bracket. The understanding that neither the usual definition nor the standard classification of these disorders entirely apply to children has prompted multicenter studies designed to increase knowledge of these disorders, as well as to standardize diagnostic and therapeutic strategies. We have reviewed the conceptualization of ILDs in children, taking into consideration the particularities of this group of patients when using the criteria for the classification of these diseases in adults. We have also made a historical review of several multicenter studies in order to further understanding of the problem. We have emphasized the differences in the clinical presentation, in an attempt to highlight knowledge of newly described entities in young children. We underscore the need to standardize management of laboratory and radiological routines, as well as of lung biopsy processing, taking such knowledge into account. It is important to bear in mind that, among the recently described disorders, genetic surfactant dysfunction, which is often classified as an idiopathic disease in adults, should be included in the differential diagnosis of ILDs.

Keywords: Lung diseases, interstitial; Lung diseases, interstitial/diagnosis; Lung diseases, interstitial/therapy; Child.

Objective: Respiratory muscle weakness is a functional repercussion of chronic lung disease (CLD). The objective of this study was to assess the effects of home-based respiratory muscle training (RMT) in children and adolescents with CLD or neuromuscular disease (NMD). Methods: This was a quasi-experimental study involving children and adolescents with CLD or NMD. Before and after 6 months of home-based RMT, we measured respiratory muscle strength (MIP and MEP), PEF, and peak cough flow (PCF). We made statistical comparisons between the pre-RMT and post-RMT values, as well as evaluating the correlation between the duration and effect of RMT. Results: The study included 29 patients, with a mean age of 12 years (range, 5-17 years), of whom 18 (62.1%) were male. The CLD group comprised 11 patients (37.9%), and the NMD group comprised 18 (62.1%). The mean duration of the RMT was 60 weeks (range, 46-90 weeks) in the CLD group and 39 weeks (range, 24-89 weeks) in the NMD group. In comparison with the pre-RMT values, the post-RMT values for MIP and MEP were significantly higher in both groups, whereas those for PEF and PCF were significantly higher only in the NMD group. We found no correlation between the duration and the effect of RMT. Conclusions: Home-based RMT appears to be an effective strategy for increasing respiratory muscle strength in children and adolescents with CLD or NMD, although it increased the ability to cough effectively only in those with NMD.

Keywords: Breathing exercises; Cystic fibrosis; Bronchiolitis obliterans; Neuromuscular diseases; Cough.

Objective: Whether blood eosinophils (bEOS) in chronic obstructive pulmonary disease (COPD) are associated with disease progression is a topic of debate. We aimed to evaluate whether the differential white blood cell (WBC) count, symptoms and treatment may predict lung function decline and exacerbations in COPD patients. Methods: We retrospectively examined stable COPD patients with a minimum follow-up of 3 years at our outpatients' clinic. We collected information about lung volumes (FEV1, FVC), the total and differential WBC count, acute exacerbations of COPD (number in the 12 months before the beginning of the study=AE-COPD-B, and during the follow-up=AE-COPD-F), smoking status and treatment. FEV1 decline and AE-COPD-F were described by using a generalized linear model and a 2-level random intercept negative binomial regression, respectively. The models included eosinophil and neutrophil counts as potential predictors and were adjusted by sex, age, smoking status, AE-COPD-B, treatment with bronchodilators and inhaled corticosteroids (ICS). Results: Sixty-eight patients were considered, 36 bEOS- (<170 cells/µL, the median value) and 32 bEOS+ (=170 cells/µL). ?FEV1 was higher in bEOS+ than bEOS- (34.86 mL/yr vs 4.49 mL/yr, p=0.029). After adjusting for potential confounders, the eosinophil count was positively (ß=19.4; CI 95% 2.8, 36.1; p=0.022) and ICS negatively (ß=-57.7; CI 95% -91.5,-23.9; p=0.001) associated with lung function decline. bEOS were not found to be associated with the number of AE-COPD-F. Conclusion: In stable COPD patients, a higher level of blood eosinophils (albeit in the normal range) predicts a greater FEV1 decline, while ICS are associated with a slower progression of airflow obstruction.

Keywords: COPD; Blood eosinophils; FEV1 decline; Exacerbations; Biomarkers. Correspondence to:

Step tests are typically used to assess exercise capacity. Given the diversity of step tests, the aim of this review was to describe the protocols that have been used in healthy subjects and in patients with chronic lung disease. Step tests for use in healthy subjects have undergone a number of modifications over the years. In most step tests, the duration is variable (90 s-10 min), but the step height (23.0-50.8 cm) and stepping rate (22.5-35.0 steps/min) remain constant throughout the test. However, the use of a fixed step height and constant stepping rate might not provide adequate work intensity for subjects with different levels of fitness, the workload therefore being above or below individual capacity. Consequently, step test protocols have been modified by introducing changes in step heights and stepping rates during the test. Step tests have been used in patients with chronic lung diseases since the late 1970s. The protocols are quite varied, with adjustments in step height (15-30 cm), pacing (self-paced or externally paced), and test duration (90 s-10 min). However, the diversity of step test protocols and the variety of outcomes studied preclude the determination of the best protocol for use in individuals with chronic lung disease. Shorter protocols with a high stepping rate would seem to be more appropriate for assessing exercise-related oxygen desaturation in chronic lung disease. Symptom-limited testing would be more appropriate for evaluating exercise tolerance. There is a need for studies comparing different step test protocols, in terms of their reliability, validity, and ability to quantify responses to interventions, especially in individuals with lung disease.

Keywords: Pulmonary disease, chronic obstructive; Asthma; Cystic fibrosis; Idiopathic pulmonary fibrosis; Exercise tolerance; Exercise test.