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All these entities are rare with small series published to date, mostly containing primary and secondary forms of disease. <jats:styled-content style=\"fixed-case\">LIP<\/jats:styled-content> is histologically characterized by diffuse polyclonal lymphoid cell infiltrate surrounding the airways and expanding the interstitium. Thin\u2010walled cysts and diffuse ground glass are considered the typical radiologic features. The clinical course is highly variable with corticosteroid responsiveness evident in approximately half of cases. <jats:styled-content style=\"fixed-case\">IPPFE<\/jats:styled-content> is defined histologically by coexisting upper lobe pleural and intra\u2010alveolar fibrosis with elastosis. Dense subpleural irregular fibrosis and consolidation are the cardinal radiologic features. A history of recurrent lower respiratory tract infection is frequent. Responses to immunomodulation have not been reported and the rate of progression appears to be highly variable. <jats:styled-content style=\"fixed-case\">AFOP<\/jats:styled-content> is a rare histologic pattern lying within the spectrum of acute\/subacute lung injury, characterized by organizing pneumonia and intra\u2010alveolar fibrin deposition without hyaline membranes. <jats:styled-content style=\"fixed-case\">BPIP<\/jats:styled-content> is characterized histologically by fibrosis and\/or inflammation confined to the alveolar interstitium around bronchovascular bundles, overlapping with peribronchial metaplasia and fibrosis in some series. Currently, <jats:styled-content style=\"fixed-case\">AFOP<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">BPIP<\/jats:styled-content> are both best viewed as histological entities rather than true clinical disorders, in the absence of characteristic associated imaging patterns and clinical features.<\/jats:p>","DOI":"10.1111\/resp.12693","type":"journal-article","created":{"date-parts":[[2015,12,2]],"date-time":"2015-12-02T11:01:51Z","timestamp":1449054111000},"page":"600-614","source":"Crossref","is-referenced-by-count":50,"title":["Rare idiopathic interstitial pneumonias: <scp>LIP<\/scp> and <scp>PPFE<\/scp> and rare histologic patterns of interstitial pneumonias: <scp>AFOP<\/scp> and <scp>BPIP<\/scp>"],"prefix":"10.1111","volume":"21","author":[{"given":"Maria A.","family":"Kokosi","sequence":"first","affiliation":[{"name":"Interstitial Lung Disease Unit Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew G.","family":"Nicholson","sequence":"additional","affiliation":[{"name":"Department of Histopathology Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust  London UK"},{"name":"National Heart and Lung Institute Imperial College  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David M.","family":"Hansell","sequence":"additional","affiliation":[{"name":"Department of Radiology Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust  London UK"},{"name":"National Heart and Lung Institute Imperial College  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Athol U.","family":"Wells","sequence":"additional","affiliation":[{"name":"Interstitial Lung Disease Unit Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust  London UK"},{"name":"National Heart and Lung Institute Imperial College  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,12,2]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201308-1483ST"},{"key":"e_1_2_8_3_1","first-page":"102","volume-title":"Frontiers of Pulmonary Radiology","author":"Leibow AA","year":"1969"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.165.2.ats01"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2001-17384"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.sult.2013.05.002"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2559.1995.tb00247.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.165.2.ats01"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-88-5-616"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.06.00076705"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.3109\/00313028709077131"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.212.2.r99au05567"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/00004728-199409000-00012"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.173.1.10397102"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.149.2.8306056"},{"key":"e_1_2_8_16_1","first-page":"27","article-title":"Lymphoreticular tumors and infiltrates of the lungs","volume":"18","author":"Colby TV","year":"1983","journal-title":"Pathol. 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