Clinical Features and Differential Diagnosis of Idiopathic Pulmonary Fibrosis

2003 ◽  
pp. 84-109
2018 ◽  
Vol 36 (2) ◽  
pp. 298-317 ◽  
Author(s):  
Johan van Cleemput ◽  
Andrea Sonaglioni ◽  
Wim A. Wuyts ◽  
Monica Bengus ◽  
John L. Stauffer ◽  
...  

2010 ◽  
Vol 134 (3) ◽  
pp. 462-480 ◽  
Author(s):  
Victor L. Roggli ◽  
Allen R. Gibbs ◽  
Richard Attanoos ◽  
Andrew Churg ◽  
Helmut Popper ◽  
...  

Abstract Asbestosis is defined as diffuse pulmonary fibrosis caused by the inhalation of excessive amounts of asbestos fibers. Pathologically, both pulmonary fibrosis of a particular pattern and evidence of excess asbestos in the lungs must be present. Clinically, the disease usually progresses slowly, with a typical latent period of more than 20 years from first exposure to onset of symptoms. Differential Diagnosis: Idiopathic Pulmonary Fibrosis The pulmonary fibrosis of asbestosis is interstitial and has a basal subpleural distribution, similar to that seen in idiopathic pulmonary fibrosis, which is the principal differential diagnosis. However, there are differences between the 2 diseases apart from the presence or absence of asbestos. First, the interstitial fibrosis of asbestosis is accompanied by very little inflammation, which, although not marked, is better developed in idiopathic pulmonary fibrosis. Second, in keeping with the slow tempo of the disease, the fibroblastic foci that characterize idiopathic pulmonary fibrosis are infrequent in asbestosis. Third, asbestosis is almost always accompanied by mild fibrosis of the visceral pleura, a feature that is rare in idiopathic pulmonary fibrosis. Differential Diagnosis: Respiratory Bronchiolitis Asbestosis is believed to start in the region of the respiratory bronchiole and gradually extends outward to involve more and more of the lung acinus, until the separate foci of fibrosis link, resulting in the characteristically diffuse pattern of the disease. These early stages of the disease are diagnostically problematic because similar centriacinar fibrosis is often seen in cigarette smokers and is characteristic of mixed-dust pneumoconiosis. Fibrosis limited to the walls of the bronchioles does not represent asbestosis. Role of Asbestos Bodies Histologic evidence of asbestos inhalation is provided by the identification of asbestos bodies either lying freely in the air spaces or embedded in the interstitial fibrosis. Asbestos bodies are distinguished from other ferruginous bodies by their thin, transparent core. Two or more asbestos bodies per square centimeter of a 5-μm-thick lung section, in combination with interstitial fibrosis of the appropriate pattern, are indicative of asbestosis. Fewer asbestos bodies do not necessarily exclude a diagnosis of asbestosis, but evidence of excess asbestos would then require quantitative studies performed on lung digests. Role of Fiber Analysis Quantification of asbestos load may be performed on lung digests or bronchoalveolar lavage material, employing either light microscopy, scanning electron microscopy, or transmission electron microscopy. Whichever technique is employed, the results are only dependable if the laboratory is well practiced in the method chosen, frequently performs such analyses, and the results are compared with those obtained by the same laboratory applying the same technique to a control population.


PLoS ONE ◽  
2018 ◽  
Vol 13 (9) ◽  
pp. e0203779 ◽  
Author(s):  
Mariel Maldonado ◽  
Ivette Buendía-Roldán ◽  
Vanesa Vicens-Zygmunt ◽  
Lurdes Planas ◽  
Maria Molina-Molina ◽  
...  

Respiration ◽  
2009 ◽  
Vol 77 (4) ◽  
pp. 407-415 ◽  
Author(s):  
Tomoko Nozu ◽  
Mitsuko Kondo ◽  
Kazuo Suzuki ◽  
Jun Tamaoki ◽  
Atsushi Nagai

2020 ◽  
Vol 92 (3) ◽  
pp. 102-108
Author(s):  
E. I. Shmelev ◽  
A. E. Ergeshov ◽  
V. Ya. Gergert

The review is devoted to the urgent problem of modern pulmonology: the differential diagnosis of idiopathic pulmonary fibrosis (ILF). ILF occupies a special place among many interstitial lung diseases for a number of reasons: 1) it is a deadly disease; 2) early diagnosis and adequate antifibrotic therapy significantly extend the life expectancy of patients; 3) anti-inflammatory drugs (corticosteroids) and cytostatics with ILF that are widely used in other forms of interstitial lung diseases are ineffective and accelerate the progression of the process; 4) the commonality of the main clinical signs (increasing respiratory failure) of various interstitial lung diseases. The list of respiratory diseases with which ILF should be differentiated is huge, and if with diffuse lung lesions of a known nature (disseminated pulmonary tuberculosis, pneumoconiosis, etc.) with a certain experience/qualification, the diagnosis is relatively simple, then the isolation of ILF from the group of idiopathic interstitial pneumonias always represents certain difficulties. The main methods used in the diagnosis of ILF are summarized taking into account current international and national recommendations.


2019 ◽  
Vol 92 (1099) ◽  
pp. 20181003 ◽  
Author(s):  
Michael P. Mohning ◽  
John Caleb Richards ◽  
Tristan J. Huie

Radiologists have a critical role in the evaluation and diagnosis of suspected idiopathic pulmonary fibrosis (IPF). Accurate pattern identification on imaging is key in the multidisciplinary diagnostic process and frequently obviates the need for a surgical lung biopsy. In this review, we describe the clinical and imaging features of IPF in the context of recently revised international guidelines; contrast findings in other diseases that may inform differential diagnosis of fibrotic lung disease; and highlight common complications associated with pulmonary fibrosis.


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