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Pathology Outlines. Radiology. It is a histomorphologic pattern and has a DDx (see below). Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. Acute interstitial pneumonia (AIP) is an idiopathic interstitial lung disease that is clinically characterized by sudden onset of dyspnea and rapid development of respiratory failure. Respiratory medicine. (2019) The British Journal of Radiology. Res. The positive predictive value of CT in the diagnosis of UIP is high and ranges from 70-100% 1. Idiopathic pulmonary fibrosis: the radiologist’s role in making the diagnosis. Usually, due to the more extensive involvement of the lower lobes, the major fissure is shifted inferiorly which is best seen on the lateral chest radiograph. Pneumothorax is a common complication and can occur at presentation or at other times during the course of the disease. 261 0 obj <>stream 2009;251 (1): 271-9. Challenges in pulmonary fibrosis x 5: the NSIP/UIP debate. Proc Am Thorac Soc. Kim EA, Lee KS, Johkoh T et-al. 13. Usual Interstitial Pneumonia (UIP) is the pattern of fibrosis classically described as having geographic and temporal variability. European Respiratory Journal. h�b```�b�# cb�w�1h30R���_pǜ[�맥� � �P�a`���$@jE��,7�%024;�4p,abf���Ͱ��0cC �C6cՍ ��A��!�q��5�>@�]˼��F)���`Q�������@� � )#� Idiopathic pulmonary fibrosis (IPF), the most common and lethal of the idiopathic interstitial pneumonias, is defined by a radiological and/or pathological pattern of usual interstitial pneumonia (UIP). Jeong YJ, Lee KS, Müller NL et-al. What every radiologist should know about idiopathic interstitial pneumonias. Usual Interstitial Pneumonia Definition. Lukas Ebner, Stergios Christodoulidis, Thomai Stathopoulou, Thomas Geiser, Odile Stalder, Andreas Limacher, Johannes T. Heverhagen, Stavroula G. Mougiakakou, Andreas Christe. Riha, E.E. Check for errors and try again. The term usual interstitial pneumonitis (UIP) has also often been used, but again, the -itis part of that name may overemphasize inflammation. 1. 2011;140 (4 Meeting Abstracts): . About this page. Lynch DA, Newell JD, Logan PM, King TE, Müller NL. In the past, the term usual interstitial pneumonia was used synonymously with idiopathic pulmonary fibrosis. 2005;236 (1): 10-21. It is sometimes used incorrectly as a synonym for idiopathic pulmonary fibrosis. This article will focus solely on the usual interstitial pneumonia pattern as a radiological or histopathological descriptor, for further discussion in the clinical aspects, please refer to the parental article on the specific underlying clinical diagnosis (e.g. AJR. 7. 0 Radiographics. As described in the 2002 statement, the pathology of IPF is that of usual interstitial pneumonia (UIP). On imaging, usual interstitial pneumonia usually presents with a lung volume loss and a craniocaudal gradient of peripheral septal thickening, bronchiectasis, and honeycombing. NSIP is less common than UIP, but occurs more frequently than the remaining idiopathic interstitial pneumonias. 28 Affected infants are generally full-term and develop symptoms and signs of lung disease within hours of birth, and radiographically have diffuse lung disease that resembles RDS in prematurely born infants. However, in certain conditions such as leflunomide-induced acute interstitial pneumonia, patients have pre-existing lung disease. R.L. Wells AU. Idiopathic Interstitial Pneumonias. Download as PDF. Otaola M, Quadrelli S, Tabaj G et-al. 12. However more recently the term idiopathic pulmonary fibrosis has been applied solely to the clinical syndrome associated with the morphologic pattern of UIP, with the specific exclusion of entities such as non-specific interstitial pneumonia (NSIP) and desquamative interstitial pneumonia (DIP) 1. Hartman TE, Primack SL, Kang EY et-al. {"url":"/signup-modal-props.json?lang=us\u0026email="}, {"containerId":"expandableQuestionsContainer","displayRelatedArticles":true,"displayNextQuestion":true,"displaySkipQuestion":true,"articleId":16895,"mcqUrl":"https://radiopaedia.org/articles/usual-interstitial-pneumonia/questions/1605?lang=us"}. atlas of interstitial lung disease pathology pathology with high resolution ct correlations Nov 19, 2020 Posted By Danielle Steel Ltd TEXT ID c91ae8ad Online PDF Ebook Epub Library 978 0 7020 3369 8 3 hasleton flieder spencers pathology of the lung 6th edition cambridge isbn 978 0 521 atlas of interstitial lung disease pathology pathology with high Korean J Radiol. Akira M, Inoue Y, Kitaichi M et-al. Eur Respir Rev. Thorax. Similar to the pathology specimen, cross-sectional imaging also reveals heterogeneity, with patchy areas of fibrosis alternating with areas of normal lung 5. 17. Usual Interstitial Pneumonia; Silo-Filler's Disease; Organizing Pneumonia; View all Topics. Types of interstitial pneumonias One prominent example is the specific pattern of a usual interstitial pneumonia (UIP), which is further classified in a definite, probable or possible UIP pattern according to the current guidelines. Akira M, Sakatani M, Ueda E. Idiopathic pulmonary fibrosis: progression of honeycombing at thin-section CT. Radiology. %%EOF Idiopathic interstitial lung injury demonstrating temporal and geographic heterogeneity; Alternate/Historical Names. The revised ATS/ERS/JRS/ALAT diagnostic criteria for idiopathic pulmonary fibrosis (IPF)-practical implications. Jonathan H. Chung, Christian W. Cox, Steven M. Montner, et al. Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fibrotic interstitial lung disease of unknown origin with a characteristic imaging and histologic pattern called usual interstitial pneumonia (UIP). ��3���� ��"`�,�5��[ ܨ~H�������6[4�DqA/��U�-�+���O;\�I�,k"�����>?��`���8��s���*���$�w���e��6I�=�y�~��1ꭣb����_��&� �����>Cn��7M������:uq0�*[7eE�n�����ϱ���Gs~tN�vO�J)�8�Dl��?��͖ƺ=h�VX�H����S�p��Y1*�l��$����1������5R�1i�p����ӛ���}V=�� �*PW����A�D���As9�����!��^����p�w��=�ߕzϵ�X��������ŀ����ܬ #&��Vs�|�NF��kT��\z�l�������5�ηr)_�R�R���Ϫ�\*�r��(`�._J�. Respir. Chest. Set alert. However more recently the term idiopathic pulmonary fibrosis has been applied solely to the clinical syndrome associated with the morphologic pattern of UIP, with the specific exclusion of entities such as non-specific interstitial pneumonia (NSIP) and desquamative interstitial pneumonia (DIP) 1. These would include: In practice, the diagnosis is usually made in a multidisciplinary approach involving chest physicians, radiologists and pathologists with expertise in interstitial lung disease (ILD) 12. American journal of roentgenology. 2. disease acute interstitial pneumonia bronchiolitis obliterans anizing pneumonia usual' 'pathology outlines usual interstitial pneumonia uip june 3rd, 2020 - the first or second most mon 17 86 interstitial lung disease clin epidemiol 2013 5 483 rarely younger than 50 years old these patients may have a genetic factor or underlying disease male 2014;23 (133): 308-19. (2017) American Journal of Roentgenology. We evaluate these diagnostic categories as prognostic markers among patients with IPF. The average rate of progression of honeycombing in patients with idiopathic usual interstitial pneumonia according to one study was 0.4% of lung volume per month 7. Wells AU. INTRODUCTION Interstitiallungdisease(ILD)includesalargenumber of conditions that are characterized by inflammation or fibrosis of the pulmonary parenchyma. 19 (6): 1114. Idiopathic pulmonary fibrosis/usual pneumonia pattern. ILD can occur due to a number of specific causes or may be classified as an idiopathic interstitial pneumonia 224 0 obj <> endobj Idiopathic interstitial pneumonia is a term that encompasses the following entities: usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), acute interstitial pneumonia (AIP), and nonspecific interstitial pneumonia (NSIP) 1.Several recent studies have demonstrated variable treatment responses and survival based on this classification 2–8. Res. Idiopathic interstitial pneumonias: CT features. 19. %PDF-1.6 %���� 10. Inter-society consensus guidelines on IPF diagnosis and management outline radiologic patterns including definite usual interstitial pneumonia (UIP), possible UIP, and inconsistent with UIP. Andras Khoor, in Pulmonary Pathology, 2008. Wuyts WA, Cavazza A, Rossi G et-al. Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. 3. Mod Pathol 25 Suppl 1 : S68-78. h�bbd``b`z${A�& ��H0�q�@,%�X,�e"$@�v���@�@Hl(c`bd���B�gd~ ` � 16. Am J Respir Crit Care Med 1999; 160:899. monia; UIP, usual interstitial pneumonia. UIP pattern of ILD can be seen in idiopathic pulmonary fibrosis or secondary to underlying systemic diseases. Mueller-mang C, Grosse C, Schmid K et-al. The term “bronchiolitis obliterans organizing pneumonia (BOOP)” is a relatively recent term for a clinicopathologic entity that has been recognized for some time.1 The name derives from a series of cases collected in the 1970s and 1980s by Charles Carrington. 6 (2): 138-153. J Thorac Imaging. differential diagnoses of airspace opacification, presence of non-lepidic patterns such as acinar, papillary, solid, or micropapillary, myofibroblastic stroma associated with invasive tumor cells. 15 (1): e0226084. 165 (4): 807-11. }F�N�N��l���cxɨ}��qGF��ݯT8�h�9�Pd�. The diagnosis of IPF is a complex procedure that requires the support of various specialists, who must integrate clinical, radiological, and histological data. Meta-analysis of the radiological and clinical features of Usual Interstitial Pneumonia (UIP) and Nonspecific Interstitial Pneumonia (NSIP). In patients with UIP, areas of ground-glass attenuation tend to increase in extent or progress to fibrosis despite treatment 8,13. Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia. Radiology. 2016;206 (3): . In recent times some authors have suggested certain signs within a UIP pattern more suggestive of it being due to connective tissue disorder interstitial lung disease over IPF 22. Chung JH, Lynch DA. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. asbestos-related interstitial lung disease: reticular opacity-to-ground glass opacity ratio: one or greater, HP usually involves the mid and upper zones of the lung, and also the presence of centrilobular nodules and areas of air trapping are very useful hints to differentiate it from UIP, UIP cases are also thought to have honeycombing and peripheral or lower lung zone predominance of disease, and less likely to have. The histopathologic features of an idiopathic, nonspecific interstitial pneumonia (NSIP) pattern observed on surgical lung biopsy (SLB) have also been thought to be the pulmonary manifestation of this process. 11. The histological diagnosis of UIP is based on temporal and spatial heterogeneity, which is the identification of fibrotic lesions at different stages (fibroblastic infiltrates, mature fibrosis, and honeycombing) within the same biopsy specimen and architectural distortion. 18. This condition is characteristically most prominent subpleurally in the lower lobes of the lung. Usual interstitial pneumonia and nonspecific interstitial pneumonia with and without concurrent emphysema: thin-section CT findings. The revised ATS/ERS/JRS/ALAT diagnostic criteria for idiopathic pulmonary fibrosis (IPF)-practical implications. Nonspecific interstitial pneumonia (NSIP) Cryptogenic organizing pneumonia (COP) Desquamative interstitial pneumonia Respiratory bronchiolitis-interstitial lung disease (RB-ILD) Acute interstitial pneumonia (AIP) Lymphoid interstitial pneumonia Idiopathic pleuroparenchymal fibroelastosis (PPFE) References and Links. Clarke, et al. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. "Smoking-related interstitial fibrosis (SRIF), pathogenesis and treatment of usual interstitial pneumonia (UIP), and transbronchial biopsy in UIP.". Chest. (2020) PLOS ONE. 14. 2014;14 Suppl 1: S2. American journal of roentgenology. 2 The term “UIP” was originally introduced by Liebow and Car-rington, 3 2006;3 (4): 285-92. Assessment with serial CT. 6 (3): 143-52. Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of respiratory diseases affecting the interstitium (the tissue and space around the alveoli (air sacs) of the lungs. Classification and natural history of the idiopathic interstitial pneumonias. On a typical biopsy, there are areas of normal lung alternating with interstitial fibrosis and honeycombing. Survival appears to be favorable. Usual interstitial pneumonia: relationship between disease activity and the progression of honeycombing at thin-section computed tomography. 2007;62 (11): 1008-12. 4. 1993;189 (3): 687-91. Usual interstitial pneumonia (UIP) is a histopathologic and radiologic pattern of interstitial lung disease, which is the hallmark pattern for idiopathic pulmonary fibrosis (IPF). endstream endobj startxref 27 (3): 595-615. The Value of a Multidisciplinary Approach to the Diagnosis of Usual Interstitial Pneumonitis and Idiopathic Pulmonary Fibrosis: Radiology, Pathology, and Clinical Correlation. Kim DS, Collard HR, King TE. Gruden J. American Journal of Roentgenology. 1996;110 (2): 378-82. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. Plain film features are non-specific. While chest radiographs can be even normal in patients with very early disease, in advanced disease, it may show decreased lung volumes and basal fine to coarse reticulation. Truly idiopathic AIP tends to occur in those without pre-existing lung disease and typically affects middle-aged adults (mean ~ 50 years 5). UIP has distinctive morphologic features that allow precise diagnosis in classical cases. Usual interstitial pneumonia (UIP) is a histologic pattern characterized by nonuniform, lower zone, subpleural, and paraseptal predominant lung injury defined by geographic heterogeneity and architectural distortion. (2016) AJR. 2014;14 Suppl 1: S2. ��I�e|EE '�m7$�2��%V�dHJ[�ݑ�M%��&�`Hb������#!-aD(F����D���DV�S�Xsx*b �.44,b�u�C���C��A�X���Y�LD�H7E0�aE���]18��J���,��|�@ǣz��_�[̾�mIN����"+�0�c�����]���IY4�,�*�c4�Pڸ�,[�����u�J Katzenstein AL, Zisman DA, Litzky LA, et al. [1, 2] AIP is histologically characterized by diffuse alveolar damage with subsequent fibrosis. This granulation tissue polyp is a very non-specific histologic finding as it is seen in most infectious pneumonias, diffuse alveolar damage, aspiration, usual interstitial pneumonia, cryptogenic organizing pneumonia, among other conditions. 20. Respir. When describing imaging features, the term UIP pattern is often used, which has specific diagnostic criteria on HRCT 16. (2018) The Lancet. confidence of a histologic usual interstitial pneumonia (UIP) pattern. Duhig, B.E. BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing lung disease of unknown etiology. In the past, the term usual interstitial pneumonia was used synonymously with IPF. Geographic variability means there are some areas of advanced scarring while other areas are completely normal. Usual interstitial pneumonia and non-specific interstitial pneumonia: serial thin-section CT findings correlated with pulmonary function. 22. It is rarely the sole major lung biopsy finding in patients presenting with interstitial lung disease (PBM-ILD). Lee JS, Gong G, Song KS et-al. doi : 10.1038/modpathol.2011.154 . ... (CFA), a term that has fallen out of favor since the basic underlying pathology is now thought to be fibrosis, not inflammation. CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease–Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. Usual interstitial pneumonia (UIP) defines idiopathic pulmonary fibrosis and is the most common of the idiopathic interstitial pneumonias. IPF is the most important and common form of chronic intersti-tial lung disease (ILD). 9. A key imaging differential on cross-sectional imaging would be: ADVERTISEMENT: Supporters see fewer/no ads, Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Cryptogenic fibrosing alveolitis ; Idiopathic pulmonary fibrosis; UIP Diagnostic Criteria. h޼�mo�6ǿ Unable to process the form. 206 (3): 463-71. idiopathic pulmonary fibrosis). Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis?. Inflammation is absent or mild and mostly limited to the areas of honeycombing 1-12. non-specific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), connective tissue disorder interstitial lung disease, diagnostic HRCT criteria for UIP pattern - ATS/ERS/JRS/ALAT (2018), diagnostic HRCT criteria for UIP pattern - Fleischner society guideline (2018), fibrotic non-specific interstitial pneumonia, chronic hypersensitivity pneumonitis (HP), combined pulmonary fibrosis and emphysema (CPFE), acute unilateral airspace opacification (differential), acute bilateral airspace opacification (differential), acute airspace opacification with lymphadenopathy (differential), chronic unilateral airspace opacification (differential), chronic bilateral airspace opacification (differential), osteophyte induced adjacent pulmonary atelectasis and fibrosis, pediatric chest x-ray in the exam setting, normal chest x-ray appearance of the diaphragm, posterior tracheal stripe/tracheo-esophageal stripe, obliteration of the retrosternal airspace, leflunomide-induced acute interstitial pneumonia, cellular non-specific interstitial pneumonia, respiratory bronchiolitis–associated interstitial lung disease, diagnostic HRCT criteria for UIP pattern - ATS/ERS/JRS/ALAT (2011), diagnostic HRCT criteria for UIP pattern - Fleischner society guideline (2018)​, domestically acquired particulate lung disease, lepidic predominant adenocarcinoma (formerly non-mucinous BAC), micropapillary predominant adenocarcinoma, invasive mucinous adenocarcinoma (formerly mucinous BAC), lung cancer associated with cystic airspaces, primary sarcomatoid carcinoma of the lung, large cell neuroendocrine cell carcinoma of the lung, squamous cell carcinoma in situ (CIS) of lung, minimally invasive adenocarcinoma of the lung, diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH), calcifying fibrous pseudotumor of the lung, IASLC (International Association for the Study of Lung Cancer) 8th edition (current), IASLC (International Association for the Study of Lung Cancer) 7th edition (superseeded), 1996 AJCC-UICC Regional Lymph Node Classification for Lung Cancer Staging, connective tissue disorders (CTD associated UIP): falls under the borader spectrum of. Videos 2002;22 Spec No : S151-65. Du bois R, King TE. In summary, PBM is a common histologic finding in various interstitial lung disorders. Patients are mostly older women, with mild symptoms and CT findings. 21. endstream endobj 225 0 obj <>/Metadata 28 0 R/Pages 222 0 R/StructTreeRoot 73 0 R/Type/Catalog>> endobj 226 0 obj <>/MediaBox[0 0 595.32 841.92]/Parent 222 0 R/Resources<>/ProcSet[/PDF/Text/ImageB/ImageC/ImageI]>>/Rotate 0/StructParents 0/Tabs/S/Type/Page>> endobj 227 0 obj <>stream Acute interstitial pneumonia (AIP) is a rapidly progressive condition of unknown cause that occurs in a previously healthy individual and produces the histologic findings of diffuse alveolar damage ().Because of its acute presentation and histologic features similar to those of acute respiratory distress syndrome (ARDS), AIP has been considered an idiopathic form of ARDS (1, 2). Pathologically, interstitial fibrosis takes the form of a dense consolidation with some preservation of alveolar septal outlines and demonstrates a distinctly … In those with more active inflammation involving the pulmonary interstitium, there is a faster progression of honeycombing in long-term follow-up 10. CLINICAL FEATURES. 5 A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. The distribution of UIP characteristically is with an apicobasal gradient with basal and peripheral (subpleural) predominance, although it is often patchy. Lynch DA, Sverzellati N, Travis WD, et al. 15. 1998;13 (3): 199-203. Pathology of Usual Interstitial Pneumonia (UIP) Dr Sampurna Roy MD Usual interstitial pneumonia is the most common form of idiopathic interstitial fibrosis (70% of all cases), and unfortunately carries a poor prognosis. Other forms of idiopathic interstitial pneumonia include desquamative interstitial pneumonia, respiratory bronchiolitis–associated interstitial lung disease, … Foci of fibroblastic activity and honeycomb change are hallmarks of the UIP pattern. Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?. Human lung disease due to an inability to produce SP-B was the first recognized genetic cause of surfactant dysfunction. Lynch DA, Travis WD, Müller NL et-al. Over time, as the understanding of the clinical behavior and histologic features of the IIPs has evolved, the categories of IIP have changed: usual interstitial pneumonia (UIP) and desquamative interstitial pneumonia (DIP) have persisted as important histologic groups, nonspecific interstitial pneumonia (NSIP) has been added, and bronchiolitis obliterans with classical interstitial pneumonia (BIP) and giant cell … Michael P. Mohning, John Caleb Richards, Tristan J. Huie. 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