{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T23:33:14Z","timestamp":1787009594083,"version":"build-2736575974"},"reference-count":22,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2020,12,30]],"date-time":"2020-12-30T00:00:00Z","timestamp":1609286400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"National Institute for Health Research Academic Clinical Fellowship"},{"name":"Ken and Mary Minton Chair of Renal Medicine"},{"name":"National Institute for Health Research (NIHR) Biomedical Research Centre"},{"name":"Imperial College Healthcare NHS Trust and Imperial College London"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,3,22]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>The impact that rare chronic disorders, such as retroperitoneal fibrosis (RPF), can have on the physical and psychological aspects of a patient\u2019s health is poorly understood. Patient-related outcome measures and experiences provide a unique opportunity to understand the impact rare chronic disorders have on a patient\u2019s life as well as allowing healthcare providers to compare and improve performance.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>To understand the physical and psychosocial impact that RPF has upon peoples\u2019 lives.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Design<\/jats:title>\n                    <jats:p>An international online questionnaire was therefore created to gain insights into how patients with RPF, a rare fibro-inflammatory condition, viewed their health and experiences.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>An international online questionnaire comprising 62 questions\/free text options, was designed in collaboration with two patient advocates and the multi-disciplinary Renal Association Rare Disease Registry (RaDaR) RPF Group the questionnaire was anonymous and freely accessible on a GOOGLE Form online platform for 6\u2009months.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 229 patients from 30 countries across 5 continents responded. Four key issues were identified; (i) pain; (ii) therapy-related side effects; (iii) lack of informed doctors\/information about their condition and its management; and (iv) psychological burden. Variations in diagnosis and management are highlighted with 55% undergoing a biopsy to reach a diagnosis of RPF; 75% of patients underwent a further interventional procedure with 60% concurrently treated medically.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This study will guide further development of clinical and academic multi-disciplinary activity and shows the importance of trying to understand the impact of rare chronic disorders on the physical and psychological aspects of a patient\u2019s health.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/qjmed\/hcaa327","type":"journal-article","created":{"date-parts":[[2020,12,7]],"date-time":"2020-12-07T21:19:37Z","timestamp":1607375977000},"page":"148-154","source":"Crossref","is-referenced-by-count":5,"title":["An international patient-centred study of retroperitoneal fibrosis"],"prefix":"10.1093","volume":"115","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1257-1017","authenticated-orcid":false,"given":"R","family":"Dattani","sequence":"first","affiliation":[{"name":"West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T D","family":"Barwick","sequence":"additional","affiliation":[{"name":"Department of Surgery and Cancer, Imperial College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G","family":"El Wardany","sequence":"additional","affiliation":[{"name":"Retroperitoneal Fibrosis (RPF) Rare Disease Group, Patient Representative London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N","family":"Gibbons","sequence":"additional","affiliation":[{"name":"Department of Urology, Imperial College Healthcare NHS Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J C","family":"Mason","sequence":"additional","affiliation":[{"name":"Imperial College London, National Heart and Lung Institute London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P","family":"Morgan","sequence":"additional","affiliation":[{"name":"Retroperitoneal Fibrosis (RPF) Rare Disease Group, Patient Representative London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C D","family":"Pusey","sequence":"additional","affiliation":[{"name":"West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK"},{"name":"Imperial College London, Centre for inflammatory Disease London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F W K","family":"Tam","sequence":"additional","affiliation":[{"name":"West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK"},{"name":"Imperial College London, Centre for inflammatory Disease London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J A P","family":"Tomlinson","sequence":"additional","affiliation":[{"name":"West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the RaDaR Rare Disease Group (RDG)","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,12,30]]},"reference":[{"key":"2022032516082477200_hcaa327-B1"},{"key":"2022032516082477200_hcaa327-B2","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1177\/1077558712465774","article-title":"Patient-centered care and outcomes: a systematic review of the literature","volume":"70","author":"Rathert","year":"2013","journal-title":"Med Care Res Rev"},{"key":"2022032516082477200_hcaa327-B3","doi-asserted-by":"crossref","first-page":"1095","DOI":"10.1016\/S0140-6736(19)30034-0","article-title":"Practical guidance for patient-centred health research","volume":"393","author":"De Wit","year":"2019","journal-title":"Lancet"},{"key":"2022032516082477200_hcaa327-B4","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1093\/bjaed\/mkw060","article-title":"Patient-reported outcome measures and patient-reported experience measures","volume":"17","author":"Kingsley","year":"2017","journal-title":"BJA Educ"},{"key":"2022032516082477200_hcaa327-B5","doi-asserted-by":"crossref","first-page":"f167","DOI":"10.1136\/bmj.f167","article-title":"Patient reported outcome measures could help transform healthcare","volume":"346","author":"Black","year":"2013","journal-title":"BMJ"},{"key":"2022032516082477200_hcaa327-B6","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41533-017-0047-5","article-title":"Stage 1 Development of a Patient-Reported Experience Measure (PREM) for Chronic Obstructive Pulmonary Disease (COPD)","volume":"27","author":"Walker","year":"2017","journal-title":". NPJ Prim Care Respir Med"},{"key":"2022032516082477200_hcaa327-B7","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1093\/intqhc\/mzv060","article-title":"Development and validation of patient-reported outcomes scale for hypertension","volume":"27","author":"Zhi","year":"2015","journal-title":"Int J Qual Health Care"},{"key":"2022032516082477200_hcaa327-B8","doi-asserted-by":"crossref","first-page":"1880","DOI":"10.1681\/ASN.2015101110","article-title":"Idiopathic retroperitoneal fibrosis","volume":"27","author":"Vaglio","year":"2016","journal-title":"J Am Soc Nephrol"},{"key":"2022032516082477200_hcaa327-B9","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1038\/sj.ki.5002427","article-title":"Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis","volume":"72","author":"Corradi","year":"2007","journal-title":"Kidney Int"},{"key":"2022032516082477200_hcaa327-B10","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1093\/qjmed\/hcz149","article-title":"IgG4-related disease in a multi-ethnic community: clinical characteristics and association with malignancy","volume":"112","author":"Poo","year":"2019","journal-title":"QJM"},{"key":"2022032516082477200_hcaa327-B11","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1186\/s13023-018-0810-x","article-title":"Patient reported outcome measures in rare diseases: a narrative review","volume":"13","author":"Slade","year":"2018","journal-title":"Orphanet J Rare Dis"},{"key":"2022032516082477200_hcaa327-B12"},{"key":"2022032516082477200_hcaa327-B13","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/S0140-6736(06)68035-5","article-title":"Retroperitoneal fibrosis","volume":"367","author":"Vaglio","year":"2006","journal-title":"Lancet"},{"key":"2022032516082477200_hcaa327-B14","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1016\/S0140-6736(11)60934-3","article-title":"Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial","volume":"378","author":"Vaglio","year":"2011","journal-title":"Lancet"},{"key":"2022032516082477200_hcaa327-B15","doi-asserted-by":"crossref","first-page":"297","DOI":"10.4065\/mcp.2010.0663","article-title":"Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes","volume":"86","author":"Kermani","year":"2011","journal-title":"Mayo Clin Proc"},{"key":"2022032516082477200_hcaa327-B16","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1136\/annrheumdis-2019-216258","article-title":"Rituximab for chronic periaortitis without evidence of IgG4-related disease: a long-term follow-up study of 20 patients","volume":"79","author":"Urban","year":"2020","journal-title":"Ann Rheum Dis"},{"key":"2022032516082477200_hcaa327-B17","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1016\/j.healthpol.2012.02.018","article-title":"How do patients with rare diseases experience the medical encounter? Exploring role behavior and its impact on patient-physician interaction","volume":"105","author":"Budych","year":"2012","journal-title":"Health Policy"},{"key":"2022032516082477200_hcaa327-B18","volume-title":"Retroperitoneal Fibrosis","author":"Engelsgjerd","year":"2020"},{"key":"2022032516082477200_hcaa327-B19"},{"key":"2022032516082477200_hcaa327-B20","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1016\/S2213-2600(18)30075-4","article-title":"Regressing or progressing: what next for the doctor\u2013patient relationship?","volume":"6","author":"Harrison","year":"2018","journal-title":"Lancet Respir Med"},{"key":"2022032516082477200_hcaa327-B21","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.ijsu.2006.01.005","article-title":"The evolution of the doctor-patient relationship","volume":"5","author":"Kaba","year":"2007","journal-title":"Int J Surg"},{"key":"2022032516082477200_hcaa327-B22"}],"container-title":["QJM: An International Journal of Medicine"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/qjmed\/advance-article-pdf\/doi\/10.1093\/qjmed\/hcaa327\/36152555\/hcaa327.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/qjmed\/article-pdf\/115\/3\/148\/42980753\/hcaa327.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/qjmed\/article-pdf\/115\/3\/148\/42980753\/hcaa327.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,3,25]],"date-time":"2022-03-25T16:15:51Z","timestamp":1648224951000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/qjmed\/article\/115\/3\/148\/6055561"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,12,30]]},"references-count":22,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2020,12,30]]},"published-print":{"date-parts":[[2022,3,22]]}},"URL":"https:\/\/doi.org\/10.1093\/qjmed\/hcaa327","relation":{},"ISSN":["1460-2725","1460-2393"],"issn-type":[{"value":"1460-2725","type":"print"},{"value":"1460-2393","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2022,3,1]]},"published":{"date-parts":[[2020,12,30]]}}}