Radiologic Findings Are Strongly Associated With a Pathologic Diagnosis of Usual Interstitial Pneumonia

Hunninghake, Gary W.; Lynch, David A.; Galvin, Jeffrey R.; Gross, Barry H.; Müller, Néstor L.; Schwartz, David A.; King, Talmadge E.; Lynch, Joseph P.; Hegele, Richard G.; Waldron, James A.; Colby, Thomas V.; Hogg, James C.

doi:10.1378/chest.124.4.1215

Cited by 294 publications

(181 citation statements)

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“…Patients in this database were referred for enrollment in study protocols for suspected IPF based on typical symptoms and on physiologic and radiographic findings. Patients with an HRCT scan showing a definite pattern of usual interstitial pneumonia (11,17) were not required to undergo a surgical lung biopsy (n ϭ 51). Patients were treated with varied treatment regimens, including no therapy, prednisone alone, prednisone with azathioprine or cyclophosphamide, or zileuton.…”

Section: Patient Selectionmentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis

Flaherty

Andrei

Murray

et al. 2006

Am J Respir Crit Care Med

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319

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Rationale and Hypothesis: Idiopathic pulmonary fibrosis is a fatal disease with a variable rate of progression. We hypothesized that changes in distance walked and quantity of desaturation during a six-minute-walk test (6MWT) would add prognostic information to changes in FVC or diffusing capacity for carbon monoxide. Methods: One hundred ninety-seven patients with idiopathic pulmonary fibrosis were evaluated. Desaturation during the 6MWT was associated with increased mortality even if a threshold of 88% was not reached. Baseline walk distance predicted subsequent walk distance but was not a reliable predictor of subsequent mortality in multivariate survival models. The predictive ability of serial changes in physiology varied when patients were stratified by the presence/absence of desaturation р 88% during a baseline 6MWT. For patients with a baseline saturation р 88% during a 6MWT, the strongest observed predictor of mortality was serial change in diffusing capacity for carbon monoxide. For patients with saturation Ͼ 88% during their baseline walk test, serial decreases in FVC and increases in desaturation area significantly predicted subsequent mortality, whereas decreases in walk distance and in diffusing capacity for carbon monoxide displayed less consistent statistical evidence of increasing mortality in our patients. Conclusion: These data highlight the importance of stratifying patients by degree of desaturation during a 6MWT before attributing prognostic value to serial changes in other physiologic variables.Keywords: idiopathic pulmonary fibrosis; six-minute-walk test; prognosis; pulmonary function; survival Idiopathic pulmonary fibrosis (IPF) is a uniformly fatal disease with a variable rate of progression. Identification of readily available baseline and short-term serial predictors of survival is critical for physicians and patients considering options such as lung transplantation and for the investigation of novel treatments. Recent studies have suggested several demographic (age, smoking), physiologic (diffusion capacity for carbon monoxide [Dl CO ], FVC, and desaturation), radiologic (high-resolution computed tomography [HRCT] fibrosis score), and histopathologic (fibroblastic foci) features are associated with survival (1-7). Serial change in FVC adds additional prognostic information to data obtained at baseline (8-11).(Received in original form April 6, 2006; accepted in final form June 30, 2006 ) Supported by National Institutes of Health NHLBI grants P50HL-56402, NHLBI, 2 K24 HL04212, 1 K23 HL68713, and 1K23 HL077719.Correspondence and requests for reprints should be addressed to Kevin R. A central feature in the pathophysiology of IPF is impaired gas exchange, which worsens with exercise (12, 13). This exerciseinduced widening of alveolar arterial O 2 gradient and fall in Pa O 2 is believed to be secondary to multiple abnormalities, including V /Q mismatch, decreased Pv O 2 and venous admixture (12-16). The six-min-walk test (6MWT) is a safe procedure for patients with IPF. Baseline des...

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Section: Patient Selectionmentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis

Flaherty

Andrei

Murray

et al. 2006

Am J Respir Crit Care Med

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319

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“…(24) An excellent evaluation of the entire lung parenchyma is possible using HRCT, which is routine practice in the initial evaluation of interstitial lung diseases. (25) Currently, HRCT is used as a routine test in the preoperative period of any surgical lung biopsy (Figure 1). With reasonable accuracy, HRCT allows the selection of ideal sites at distinct stages of the disease in the lung parenchyma to be biopsied.…”

Section: Resultsmentioning

confidence: 99%

Análise semiquantitativa de biópsias cirúrgicas de diferentes lobos pulmonares de pacientes com pneumonia intersticial usual/fibrose pulmonar idiopática

Gonçalves¹,

Leão

Ferreira

et al. 2009

J. bras. pneumol.

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Objective: To evaluate the differences between surgical biopsies of distinct lung lobes in terms of the histopathological features of usual interstitial pneumonia, using a semiquantitative score. Methods: We selected all of the patients diagnosed with idiopathic pulmonary fibrosis and submitted to surgical biopsy in two distinct lobes between 1995 and 2005 at the Hospital São Paulo and other hospitals operated by the Federal University of São Paulo. In the histological evaluation of the specimens, we used a semiquantitative method based on previous studies, assigning a score to each of the biopsied sites. Results: In this sample of patients, we found no statistically significant differences that would alter the stage of the disease, based on the score used. This finding was independent of the biopsy site (middle lobe or lingular segment). Conclusions: No significant histological differences were found between the lung lobes studied. The definitive histological diagnosis of usual interstitial pneumonia did not alter the stage of the disease.Keywords: Lung diseases, interstitial; Pulmonary fibrosis; Thoracic surgery; Pathology; Thoracic surgery, video-assisted. ResumoObjetivo: Avaliar as diferenças histológicas da pneumonia intersticial usual entre biópsias cirúrgicas de lobos pulmonares distintos, utilizando um escore semiquantitativo. Métodos: Foram selecionados todos os pacientes com o diagnóstico de fibrose pulmonar idiopática e submetidos à biópsia cirúrgica em dois lobos distintos no Hospital São Paulo e em hospitais afiliados da Universidade Federal de São Paulo, no período entre 1995 e 2005. Foi utilizado um método semiquantitativo na avaliação histológica dos espécimes, com base em estudos prévios, aplicando-se um escore para cada local submetido à biópsia. Resultados: Nenhuma diferença estatisticamente significante foi encontrada nesta amostra de pacientes que viesse alterar o estágio da doença, com base no escore utilizado. Este achado foi independente do local da biópsia (lobo médio ou segmento lingular). Conclusões: Não foram observadas diferenças histológicas significantes entre os lobos pulmonares estudados. O diagnóstico histológico definitivo de pneumonia intersticial usual não alterou o estágio da doença.

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“…When the radiologists felt confident about their HRCT diagnosis (approximately 60% of the cases that they evaluated), the positive predictive value improved to 96%. 5 Subpleural DOI:10.1002/MSJ honeycombing and septal thickening were the HRCT features most predictive of IPF. Patients with HRCT scans that exhibited these features suffered the worst survival.…”

Section: Is High-resolution Computed Tomography Helpful In Differentimentioning

confidence: 94%

“…14 HRCT in NSIP ( Figure 4) commonly demonstrates GGO that is bilateral and symmetrical. 5,14,15 It may be associated with volume loss (especially in the lower lobes) and/or traction bronchiectasis. Honeycomb change is rare.…”

Section: Fibrotic Nonspecific Interstitial Pneumoniamentioning

confidence: 99%

“…Patients with HRCT scans that exhibited these features suffered the worst survival. 15 In contrast to IPF/UIP, it is difficult to diagnose NSIP solely on the basis of its radiologic features, 5,15,18,19 and a radiologic diagnosis of NSIP must be made with caution. Ground-glass attenuation is the only HRCT feature independently associated with NSIP.…”

Section: Is High-resolution Computed Tomography Helpful In Differentimentioning

confidence: 99%

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Spectrum of Fibrosing Diffuse Parenchymal Lung Disease

Morgenthau

Padilla

2009

Mount Sinai J Medicine

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The interstitial lung diseases are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the pulmonary interstitium. In 2002, the American Thoracic Society and the European Respiratory Society revised the classification of interstitial lung diseases and introduced the term diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are a subtype of diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are subdivided into usual interstitial pneumonia (with its clinical counterpart idiopathic interstitial pneumonia), nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis interstitial lung disease, and lymphocytic pneumonia. Sarcoidosis and hypersensitivity pneumonitis are the 2 most common granulomatous diffuse parenchymal lung diseases. Rheumatoid arthritis, systemic sclerosis, and dermatomyositis/polymyositis (causing antisynthetase syndrome) are diffuse parenchymal lung diseases of known association because these conditions are associated with connective tissue disease. Hermansky‐Pudlak syndrome is a rare genetic diffuse parenchymal lung disease characterized by the clinical triad of pulmonary disease, oculocutaneous albinism, and bleeding diathesis. This review provides an overview of the chronic fibrosing diffuse parenchymal lung diseases. Its primary objective is to illuminate the clinical challenges encountered by clinicians who manage the diffuse parenchymal lung diseases regularly and to offer potential solutions to those challenges. Treatment for the diffuse parenchymal lung diseases is limited, and for many patients with end‐stage disease, lung transplantation remains the best option. Although much has been learned about the diffuse parenchymal lung diseases during the past decade, research in these diseases is urgently needed. Mt Sinai J Med 76:2–23, © 2009 Mount Sinai School of Medicine

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Radiologic Findings Are Strongly Associated With a Pathologic Diagnosis of Usual Interstitial Pneumonia

Cited by 294 publications

References 24 publications

Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis

Análise semiquantitativa de biópsias cirúrgicas de diferentes lobos pulmonares de pacientes com pneumonia intersticial usual/fibrose pulmonar idiopática

Spectrum of Fibrosing Diffuse Parenchymal Lung Disease

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