A síndrome de Mounier-Kühn, ou traqueobroncomegalia congênita, é uma entidade clínica rara caracterizada pela dilatação anormal de traquéia e brônquios principais. O diagnóstico geralmente pode ser realizado através da mensuração do diâmetro traqueal. Os autores apresentam o caso de um homem de 40 anos com pneumonia refratária ao tratamento, no qual a traqueobroncomegalia foi confirmada através de tomografia computadorizada.

Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical entity characterized by abnormal dilation of the trachea and main bronchi. The diagnosis can usually be made by measuring the tracheal diameter. We report the case of a 40-year-old black man with refractory lower respiratory tract infection. Tracheobronchomegaly was confirmed through computed tomography.

Epidemiologic data on idiopathic pulmonary fibrosis are relatively scarce, and its real incidence and prevalence are unknown. Recent studies suggest that mortality due to idiopathic pulmonary fibrosis is rising in developed countries. OBJECTIVE: To describe mortality caused by idiopathic pulmonary fibrosis in the State of Rio Grande do Sul (RS), Brazil, from 1970 to 2000, analyzing its trend and comparing it with that from other countries. METHOD: Prevalence study, using data from the Brazilian Institute of Geography and Statistics (IBGE), analyzing death certificates in which idiopathic pulmonary fibrosis was stated as the ultimate cause of death. RESULTS: Annual mortality rate adjusted to the population was 0.22/100,000 people in the 1970’s, 0.3/100,000 people in the 1980’s, and 0.48/100,000 people in the 1990’s. Total mortality raised 36% from 1970 to 1980, and 73% from 1980 to 1990. The mortality rate adjusted to the population raised 36% and 60% during the same periods. The rise in both total and adjusted mortality from IPF was statistically significant (p < 0.05). The mean mortality rate per 100,000 inhabitants between 1996 and 1998, however, was 0.683, corresponding to a 70% increase when compared to the previous three-year period, which was 0.4 (p = 0.0002), probably reflecting coding practices. CONCLUSION: There was a significant increase in IPF mortality in RS from 1970 to 2000, partly due to changes in coding practices. This increase is in conformity with observations in other countries, although mortality rates in RS are considerably lower.

A pneumonia em organização, acompanhada ou não de bronquiolite obliterante, é uma condição anatomopatológica pulmonar específica com uma grande variedade de apresentações clínicas e radiológicas. A pneumonia em organização pode ser idiopática ou secundária a várias doenças, incluindo infecções e drogas. Objetivos: Descrever as manifestações clínicas, dados radiológicos e espirométricos de pacientes com pneumonia em organização. Métodos: Análise retrospectiva de pacientes com o diagnóstico de pneumonia em organização. Resultados: 38 pacientes foram incluídos na análise. Quatorze apresentavam também condições clínicas relacionadas à pneumonia em organização, e estes mais freqüentemente apresentaram infiltração pulmonar difusa (três de quatro casos) e bronquiolite obliterante associada (57% vs. 20%, p = 0,05). Dos 13 pacientes com bronquiolite obliterante associada apenas um era assintomático e dois apresentavam lesões localizadas (15%). De oito pacientes assintomáticos com lesões localizadas, nenhum possuía bronquiolite obliterante associada, e a apresentação radiológica freqüentemente mimetizou carcinoma brônquico. A espirometria de modo geral não contribuiu sobremaneira para o diagnóstico, provavelmente devido à alta prevalência de tabagismo na amostra. Conclusões: A pneumonia em organização possui apresentação clínica e radiológica variada. Fatores que sugerem a ausência de bronquiolite obliterante são a ausência de sintomas e lesões radiológicas localizadas, e esta forma da doença mais freqüentemente deve ser diferenciada de carcinoma brônquico na prática clínica.

Organizing pneumonia, whether or not accompanied by bronchiolitis obliterans, is a specific anatomicopathological condition of the lungs that can present in a variety of clinical and radiographic ways. It can be either idiopathic or secondary to a number of diseases, including infection and drugs. Objectives: To describe the clinical manifestations, radiographic and spirometric data seen in patients with organizing pneumonia. Methods: Retrospective analysis of patients with a diagnosis of organizing pneumonia. Results: 38 patients were included in the analysis. Fourteen also had clinical conditions related to organizing pneumonia, and they presented more frequently with diffuse pulmonary infiltrates (three of four cases) and associated bronchiolitis obliterans (57% vs. 20%, p = 0,05). Of the 13 patients with bronchiolitis obliterans, only one was asymptomatic and two had localized lesions (15%). Of the eight asymptomatic patients with localized lesions, no one had associated bronchiolitis obliterans, and the radiographic appearance often resembled bronchial carcinoma. Spirometry was generally of little value to diagnosis, probably because of the high prevalence of smoking in the sample. Conclusion: The clinical and radiographic presentation of organizing pneumonia is variable. Factors that suggest the absence of coexisting bronchiolitis obliterans are the absence of symptoms and localized radiographic lesions, and this form of the disease has to be more often differentiated from bronchial carcinoma in clinical practice.

Relata-se um caso típico de sarcoidose pediátrica com apresentação de doença granulomatosa pulmonar e acometimento sistêmico, revisando aspectos clínicos, diagnósticos e particularidades da apresentação da doença nesta faixa etária, com ênfase na freqüentemente difícil diferenciação com a tuberculose.

The authors report a typical case of pediatric sarcoidosis presenting a granulomatous pulmonary disease with systemic impairment, reviewing clinical, diagnostic, and particular aspects of the disease in this age group, with emphasis on the difficulty to differentiate its diagnosis from that of tuberculosis.