Usual Interstitial Pneumonia Definition. 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. 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. Videos 206 (3): 463-71. 3. UIP has distinctive morphologic features that allow precise diagnosis in classical cases. UIP pattern of ILD can be seen in idiopathic pulmonary fibrosis or secondary to underlying systemic diseases. Radiology. 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. 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. 11. In the past, the term usual interstitial pneumonia was used synonymously with idiopathic pulmonary fibrosis. 20. Meta-analysis of the radiological and clinical features of Usual Interstitial Pneumonia (UIP) and Nonspecific Interstitial Pneumonia (NSIP). Duhig, B.E. The term usual interstitial pneumonitis (UIP) has also often been used, but again, the -itis part of that name may overemphasize inflammation. Challenges in pulmonary fibrosis x 5: the NSIP/UIP debate. ��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 2016;206 (3): . Gruden J. American Journal of Roentgenology. {"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"}. 2014;14 Suppl 1: S2. (2016) AJR. Pathology Outlines. Download as PDF. Plain film features are non-specific. 16. Other forms of idiopathic interstitial pneumonia include desquamative interstitial pneumonia, respiratory bronchiolitis–associated interstitial lung disease, … 17. 2014;14 Suppl 1: S2. The positive predictive value of CT in the diagnosis of UIP is high and ranges from 70-100% 1. Human lung disease due to an inability to produce SP-B was the first recognized genetic cause of surfactant dysfunction. 2 The term “UIP” was originally introduced by Liebow and Car-rington, 3 Lee JS, Gong G, Song KS et-al. 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. In the past, the term usual interstitial pneumonia was used synonymously with IPF. The Value of a Multidisciplinary Approach to the Diagnosis of Usual Interstitial Pneumonitis and Idiopathic Pulmonary Fibrosis: Radiology, Pathology, and Clinical Correlation. Inflammation is absent or mild and mostly limited to the areas of honeycombing 1-12. Kim DS, Collard HR, King TE. It is a histomorphologic pattern and has a DDx (see below). 18. 21. In summary, PBM is a common histologic finding in various interstitial lung disorders. In those with more active inflammation involving the pulmonary interstitium, there is a faster progression of honeycombing in long-term follow-up 10. As described in the 2002 statement, the pathology of IPF is that of usual interstitial pneumonia (UIP). Inter-society consensus guidelines on IPF diagnosis and management outline radiologic patterns including definite usual interstitial pneumonia (UIP), possible UIP, and inconsistent with UIP. Radiology. It is rarely the sole major lung biopsy finding in patients presenting with interstitial lung disease (PBM-ILD). Akira M, Inoue Y, Kitaichi M et-al. 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. 5. 4. 1993;189 (3): 687-91. Du bois R, King TE. Idiopathic interstitial pneumonias: CT features. The revised ATS/ERS/JRS/ALAT diagnostic criteria for idiopathic pulmonary fibrosis (IPF)-practical implications. 27 (3): 595-615. When describing imaging features, the term UIP pattern is often used, which has specific diagnostic criteria on HRCT 16. Chest. Proc Am Thorac Soc. Survival appears to be favorable. 0 NSIP is less common than UIP, but occurs more frequently than the remaining idiopathic interstitial pneumonias. Chung JH, Lynch DA. Mueller-mang C, Grosse C, Schmid K et-al. 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. 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. Patients are mostly older women, with mild symptoms and CT findings. Check for errors and try again. differential diagnoses of airspace opacification, presence of non-lepidic patterns such as acinar, papillary, solid, or micropapillary, myofibroblastic stroma associated with invasive tumor cells. Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?. Usual Interstitial Pneumonia; Silo-Filler's Disease; Organizing Pneumonia; View all Topics. AJR. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. 2. 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). 7. Respir. confidence of a histologic usual interstitial pneumonia (UIP) pattern. Pneumothorax is a common complication and can occur at presentation or at other times during the course of the disease. Clarke, et al. CLINICAL FEATURES. 6. Akira M, Sakatani M, Ueda E. Idiopathic pulmonary fibrosis: progression of honeycombing at thin-section CT. Radiology. European Respiratory Journal. 224 0 obj <> endobj Usual Interstitial Pneumonia (UIP) is the pattern of fibrosis classically described as having geographic and temporal variability. 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. On imaging, usual interstitial pneumonia usually presents with a lung volume loss and a craniocaudal gradient of peripheral septal thickening, bronchiectasis, and honeycombing. 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. 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. Riha, E.E. What every radiologist should know about idiopathic interstitial pneumonias. Thorax. 2005;236 (1): 10-21. Usual interstitial pneumonia and non-specific interstitial pneumonia: serial thin-section CT findings correlated with pulmonary function. 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. Mod Pathol 25 Suppl 1 : S68-78. Foci of fibroblastic activity and honeycomb change are hallmarks of the UIP pattern. About this page. The diagnosis of IPF is a complex procedure that requires the support of various specialists, who must integrate clinical, radiological, and histological data. Respiratory medicine. 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. [1, 2] AIP is histologically characterized by diffuse alveolar damage with subsequent fibrosis. 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. (2017) American Journal of Roentgenology. 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