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      Kryptogen organisierende Pneumonie versus sekundäre organisierende Pneumonie Translated title: Cryptogenic organizing pneumonia versus secondary organizing pneumonia

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          Abstract

          Die organisierende Pneumonie (OP) beschreibt ein histologisches Muster des akuten bzw. subakuten Lungenschadens. Klinisch fallen die Patienten mit Husten, Fieber und Dyspnoe auf. Es wird zwischen der idiopathischen bzw. kryptogen organisierenden (COP) und der sekundären organisierenden Pneumonie (OP) unterschieden. Bei der COP kann weder klinisch/radiologisch noch histologisch eine Ursache bestimmt werden. Sie wird zu den interstitiellen idiopathischen Pneumonien (IIP) gezählt nach Kriterien der American Thoracic Society (ATS) und der European Respiratory Society (ERS). Bei der sekundären organisierenden Pneumonie liegt ein bekannter auslösender Mechanismus vor, wie z. B. infektiöse Erreger, bestimmte Medikamente oder auch Begleiterscheinungen anderer primär pulmonaler Erkrankungen und Erkrankungen anderer Organsysteme. Beiden Formen gemeinsam ist das histologische Bild der intraalveolären Mesenchymknospen. Diese sind Myofibroblastenproliferate, welche sich zopfartig entlang der Alveolarräume verzweigen. Sie werden in der Regel von einem bis mäßigen interstitiellen und alveolären, chronischen und makrophagenreichen Entzündungszellinfiltrat begleitet. Wichtigste Differenzialdiagnose ist die gewöhnliche interstitielle Pneumonie (UIP). Diese zeigt ebenfalls Fibroblastenproliferate, welche allerdings interstitiell liegen. Die korrekte Zuordnung einer IIP als COP mittels klinischer, radiologischer und histologischer Befunderhebung ist essenziell, da die COP im Gegensatz zur UIP sehr gut auf Kortikosteroide anspricht und somit gegenüber der UIP eine exzellente Prognose hat. Der Verlauf sekundärer organisierender Pneumonien hängt von der jeweiligen Grunderkrankung ab. Hier ist es wichtig, dass der Pathologe histologische Begleitcharakteristika einer OP korrekt identifiziert, um Hinweise auf eine mögliche Ursache geben zu können.

          Translated abstract

          Organizing pneumonia (OP) describes a histological pattern of acute or subacute lung damage. Clinically, patients present with cough, fever, and dyspnea. A distinction is made between idiopathic or cryptogenic organizing pneumonia (COP) and secondary organizing pneumonia (OP). In COP, neither clinical/radiological nor histological causes can be determined. It is classified as an interstitial idiopathic pneumonia (IIP) according to the criteria of the American Thoracic Society (ATS) and the European Respiratory Society (ERS). Secondary organizing pneumonia has a known triggering mechanism, such as infectious agents, certain medications, or concomitant symptoms of other primary pulmonary diseases and diseases of other organ systems. Common to both forms is the histological picture of intra-alveolar mesenchymal buds. These are myofibroblast proliferates that branch out along the alveolar spaces. They are usually accompanied by a moderate interstitial and alveolar, chronic, and macrophage-rich inflammatory cell infiltrate. The most important differential diagnosis is common interstitial pneumonia (UIP). It also shows fibroblast proliferates, which are, however, located in the interstitium. The correct classification of an IIP as a COP by means of clinical, radiological, and histological findings is essential, since the COP, in contrast to the UIP, responds very well to corticosteroids and therefore has an excellent prognosis compared to the UIP. The course of secondary organizing pneumonia depends on the respective underlying disease. Here it is important for the pathologist to correctly identify potential accompanying histological characteristics in order to be able to provide clues to a possible cause of OP.

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          An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias.

          In 2002 the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of idiopathic interstitial pneumonias (IIPs) defined seven specific entities, and provided standardized terminology and diagnostic criteria. In addition, the historical "gold standard" of histologic diagnosis was replaced by a multidisciplinary approach. Since 2002 many publications have provided new information about IIPs. The objective of this statement is to update the 2002 ATS/ERS classification of IIPs. An international multidisciplinary panel was formed and developed key questions that were addressed through a review of the literature published between 2000 and 2011. Substantial progress has been made in IIPs since the previous classification. Nonspecific interstitial pneumonia is now better defined. Respiratory bronchiolitis-interstitial lung disease is now commonly diagnosed without surgical biopsy. The clinical course of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia is recognized to be heterogeneous. Acute exacerbation of IIPs is now well defined. A substantial percentage of patients with IIP are difficult to classify, often due to mixed patterns of lung injury. A classification based on observed disease behavior is proposed for patients who are difficult to classify or for entities with heterogeneity in clinical course. A group of rare entities, including pleuroparenchymal fibroelastosis and rare histologic patterns, is introduced. The rapidly evolving field of molecular markers is reviewed with the intent of promoting additional investigations that may help in determining diagnosis, and potentially prognosis and treatment. This update is a supplement to the previous 2002 IIP classification document. It outlines advances in the past decade and potential areas for future investigation.
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            American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001.

            , (2002)
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              An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features.

              Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). Researchers have proposed differing criteria and terms to describe these patients, and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort.The "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity.The task force proposes the term "interstitial pneumonia with autoimmune features" (IPAF) and offers classification criteria organised around the presence of a combination of features from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantibodies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features.A designation of IPAF should be used to identify individuals with IIP and features suggestive of, but not definitive for, a CTD. With IPAF, a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort.
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                Author and article information

                Contributors
                rkrupar@fz-borstel.de
                Journal
                Pathologe
                Pathologe
                Der Pathologe
                Springer Medizin (Heidelberg )
                0172-8113
                1432-1963
                18 January 2021
                : 1-9
                Affiliations
                [1 ]GRID grid.418187.3, ISNI 0000 0004 0493 9170, Pathologie, , Forschungszentrum Borstel, Leibniz Lungenzentrum, ; Parkallee 3A, 23845 Borstel, Deutschland
                [2 ]Institut für Pathologie, Universität zu Lübeck und Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Lübeck, Deutschland
                [3 ]GRID grid.412468.d, ISNI 0000 0004 0646 2097, Klinik für Radiologie und Nuklearmedizin, , Universitätsklinikum Schleswig-Holstein, Campus Lübeck, ; Lübeck, Deutschland
                [4 ]GRID grid.452624.3, Airway Research Center North, , Deutsches Zentrum für Lungenforschung, ; Großhansdorf, Deutschland
                Author notes
                [Schwerpunktherausgeber]

                S. Perner, Lübeck

                F. Stellmacher, Borstel

                Article
                903
                10.1007/s00292-020-00903-8
                7812985
                33462627
                aec736a6-60f4-4442-b618-a2415234ce10
                © Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2021

                This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.

                History
                : 17 December 2020
                Categories
                Schwerpunkt: Nicht-neoplastische Lungenerkrankungen

                idiopathische organisierende pneumonie,masson-körper,mesenchymknospen,idiopathische interstitielle pneumonie,bronchiolitis obliterans mit organisierender pneumonie (boop),idiopathic organizing pneumonia,masson bodies,fibroblast proliferation,idiopathic interstitial pneumonia,bronchiolitis obliterans organizing pneumonia (boop)

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