RESUMO
The 2013 American Thoracic Society/European Respiratory Society consensus classification update of the idiopathic interstitial pneumonias (IIP) included several important modifications to the organization and spectrum of the diseases that were proposed in an earlier multidisciplinary consensus document in 2002. The histopathology of the now 'major' and 'rare' IIP is presented here with exposition of the newly included entity of a distinctive upper lobe fibrotic lung disease referred to as idiopathic pleuroparenchymal fibroelastosis. The 'rare histological patterns' of acute fibrinous and organizing pneumonia and bronchiolocentric patterns of interstitial pneumonia are illustrated and discussed.
Assuntos
Pneumonias Intersticiais Idiopáticas/patologia , Diagnóstico Diferencial , HumanosRESUMO
BACKGROUND: Airway-centered Interstitial Fibrosis (ACIF) is a common pathologic pattern observed in our practice. OBJECTIVES: The objectives of this study are to describe the causes associated with ACIF in a large sample of patients and its effect on survival. METHODS: A 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. RESULTS: There 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. CONCLUSIONS: ACIF 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.
Assuntos
Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/etiologia , Idoso , Estudos de Coortes , Feminino , Humanos , Doenças Pulmonares Intersticiais/mortalidade , Masculino , Pessoa de Meia-Idade , Prognóstico , Fibrose Pulmonar/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida/tendênciasRESUMO
Malignant tumors in the lung (both primary and metastatic) rarely may be associated with markedly discohesive tumor cells, resulting in airspace filling reminiscent of "desquamative interstitial pneumonia" (DIP) on histopathology evaluation. A peculiar aspect of this growth pattern is the relatively bland appearance of the tumor cells, in many cases simulating benign alveolar macrophages at scanning magnification. We searched the Charles Carrington Memorial consultation files in the Department of Laboratory Medicine and Pathology at Mayo Clinic Arizona for instances of malignant tumors in lung simulating DIP, from 1992 to 2011. We identified 7 cases involving transbronchial biopsies, needle core samples, or resected lung specimens. Clinical, histopathologic, and immunohistochemical analyses of these 7 patients were performed, including detailed morphometric analysis of the individual tumor cells using calibrated measurement tools on digital images. We compared the results with those of a control group of 4 patients with benign DIP-macrophage reactions in smoking-related lung disease. The study group comprised 5 male and 2 female patients, 48 to 86 years in age (median: 67 y). The radiologic findings included lobar consolidation, localized ground-glass opacities, and 1 or more nodules. None of the patients had typical bilateral infiltrates of DIP. Microscopically, the lung parenchyma was dominated by the presence of prominent tumor cells filling alveolar spaces. Four patients had primary lung carcinoma (adenocarcinoma), whereas 3 had metastases from other sites, including a melanoma. Immunohistochemical staining studies were performed on 6 of 7 cases to establish the diagnosis. Nuclear diameter, cytoplasmic diameter, and nuclear/cytoplasmic (N/C) ratios in patient and control groups were compared using the Wilcoxon rank sum test. No significant difference in the diameters of nucleus and cytoplasm between cases and control groups (P=0.3447 and 0.7055, respectively) was seen, and only a marginally significant difference in N/C ratios (P=0.0890) was seen. A more complex analysis, generalized estimating equation analysis, showed a significant difference in N/C ratio between the 2 groups (P=0.0278). A "DIP-growth pattern" of malignant tumors in the lung is presented. Although the N/C ratio differences approached statistical significance when compared with controls, the key to diagnosis is the recognition of the malignant cytology of the tumor nuclei. Immunohistochemical studies (keratin or other markers) are helpful in establishing an accurate diagnosis in this setting.
Assuntos
Adenocarcinoma/patologia , Pneumonias Intersticiais Idiopáticas/patologia , Neoplasias Pulmonares/patologia , Pulmão/patologia , Adenocarcinoma/química , Adenocarcinoma de Pulmão , Idoso , Idoso de 80 Anos ou mais , Arizona , Biomarcadores Tumorais/análise , Biópsia , Estudos de Casos e Controles , Diferenciação Celular , Tamanho do Núcleo Celular , Diagnóstico Diferencial , Feminino , Humanos , Imuno-Histoquímica , Pulmão/química , Neoplasias Pulmonares/química , Neoplasias Pulmonares/secundário , Macrófagos Alveolares/patologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos TestesRESUMO
High-resolution computed tomography (HRCT) is the radiological imaging technique best suited to revealing changes in lung structure. Various HRCT findings, taken together, can represent typical patterns. These patterns, in conjunction with the anatomical distribution of findings and with clinical data, can narrow the differential diagnosis of diffuse interstitial lung disease and, in many cases, indicate the correct diagnosis with a high degree of accuracy. The most common HRCT patterns seen in cases of diffuse interstitial lung diseases are the nodular pattern, linear/reticular opacities, cystic lesions, ground-glass opacities and consolidations. This article reviews the correlations between HRCT patterns and pathologic findings, summarizing the most common causes, as well as detailing the methods of investigation employed in order to diagnose the most common types of chronic diffuse lung disease.
Assuntos
Doenças Pulmonares Intersticiais/patologia , Pulmão/patologia , Interpretação de Imagem Radiográfica Assistida por Computador/métodos , Tomografia Computadorizada por Raios X/métodos , Biópsia , Diagnóstico Diferencial , Humanos , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/patologiaRESUMO
A tomografia de alta resolução (TCAR) é a técnica de imagem radiológica que reflete mais de perto as alterações da estrutura pulmonar. Os vários achados tomográficos podem ser combinados para formar padrões típicos. Estes, conjuntamente com a distribuição anatômica dos achados, e com os dados clínicos, podem estreitar o diagnóstico das doenças intersticiais pulmonares difusas, e em vários casos sugerir o diagnóstico correto com alto grau de acurácia. Os padrões mais comuns das doenças intersticiais pulmonares difusas na TCAR são o nodular, linear e reticular, lesões císticas, opacidades em vidro fosco e consolidações. Este artigo revisa as correlações entre os padrões tomográficos na TCAR e os achados patológicos e resume as causas mais comuns e os métodos de investigação para se atingir um diagnóstico nas doenças pulmonares crônicas difusas mais comuns.
High-resolution computed tomography (HRCT) is the radiological imaging technique best suited to revealing changes in lung structure. Various HRCT findings, taken together, can represent typical patterns. These patterns, in conjunction with the anatomical distribution of findings and with clinical data, can narrow the differential diagnosis of diffuse interstitial lung disease and, in many cases, indicate the correct diagnosis with a high degree of accuracy. The most common HRCT patterns seen in cases of diffuse interstitial lung diseases are the nodular pattern, linear/reticular opacities, cystic lesions, ground-glass opacities and consolidations. This article reviews the correlations between HRCT patterns and pathologic findings, summarizing the most common causes, as well as detailing the methods of investigation employed in order to diagnose the most common types of chronic diffuse lung disease.
Assuntos
Humanos , Doenças Pulmonares Intersticiais/patologia , Pulmão/patologia , Interpretação de Imagem Radiográfica Assistida por Computador/métodos , Tomografia Computadorizada por Raios X/métodos , Biópsia , Diagnóstico Diferencial , Doenças Pulmonares Intersticiais , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares , PulmãoRESUMO
Acute fibrinous and organizing pneumonia, a recently described form of diffuse acute lung injury, sometimes affects adults with inflammatory myopathy. We describe a child with juvenile dermatomyositis who had development of acute fibrinous and organizing pneumonia. There does not appear to be a successful method of treatment, particularly in severe cases with respiratory failure.