{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,29]],"date-time":"2025-08-29T00:03:28Z","timestamp":1756425808433,"version":"3.44.0"},"reference-count":26,"publisher":"BMJ","issue":"8","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2022,7,29]]},"published-print":{"date-parts":[[2022,8]]},"abstract":"<jats:p>A woman in her 50s was referred to nephrology clinic due to progressive chronic kidney disease. She exhibited features of proximal renal tubulopathy, namely Fanconi syndrome, including normoglycaemic glycosuria, normal anion gap metabolic acidosis, and intermittent hypouricaemia and hypophosphataemia. Kidney biopsy showed tubulointerstitial inflammation and focal chronic damage. In addition, antimitochondrial antibodies were present and she had abnormal liver blood tests. A unifying diagnosis of primary biliary cholangitis with an associated renal tubulopathy and interstitial nephritis was made. She was commenced on sodium bicarbonate, ursodeoxycholic acid and oral prednisolone, leading to an improvement in liver biochemistry. Kidney function was stabilised, but a sustained improvement was not seen. This case acts as a reminder of the rare association of tubulointerstitial nephritis and Fanconi syndrome with primary biliary cholangitis, which may be an under-recognised phenotype.<\/jats:p>","DOI":"10.1136\/bcr-2021-248461","type":"journal-article","created":{"date-parts":[[2022,8,16]],"date-time":"2022-08-16T12:10:14Z","timestamp":1660651814000},"page":"e248461","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Primary biliary cholangitis presenting with Fanconi syndrome: an important phenotype"],"prefix":"10.1136","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5075-102X","authenticated-orcid":false,"given":"Chaoxui","family":"Er","sequence":"first","affiliation":[{"name":"Renal Medicine, Freeman Hospital, Newcastle-upon-Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessica","family":"Dyson","sequence":"additional","affiliation":[{"name":"Hepatology, Freeman Hospital, Newcastle-upon-Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Jones","sequence":"additional","affiliation":[{"name":"Hepatology, Freeman Hospital, Newcastle-upon-Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Sayer","sequence":"additional","affiliation":[{"name":"Renal Medicine, Freeman Hospital, Newcastle-upon-Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2022,8,16]]},"reference":[{"key":"2025082801471984000_15.8.e248461.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra043898"},{"key":"2025082801471984000_15.8.e248461.2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.22906"},{"key":"2025082801471984000_15.8.e248461.3","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840100102"},{"key":"2025082801471984000_15.8.e248461.4","doi-asserted-by":"publisher","DOI":"10.1136\/gutjnl-2017-315259"},{"key":"2025082801471984000_15.8.e248461.5","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2017.03.022"},{"key":"2025082801471984000_15.8.e248461.6","doi-asserted-by":"publisher","DOI":"10.1053\/he.2000.5984"},{"key":"2025082801471984000_15.8.e248461.7","doi-asserted-by":"crossref","first-page":"e57","DOI":"10.1016\/j.clinre.2015.08.001","article-title":"Changing nomenclature for PBC: From 'cirrhosis' to 'cholangitis'","volume":"39","author":"Beuers","year":"2015","journal-title":"Clin Res Hepatol Gastroenterol"},{"key":"2025082801471984000_15.8.e248461.8","doi-asserted-by":"crossref","first-page":"771","DOI":"10.5009\/gnl16365","article-title":"Extrahepatic manifestations of primary biliary cholangitis","volume":"11","author":"Chalifoux","year":"2017","journal-title":"Gut Liver"},{"key":"2025082801471984000_15.8.e248461.9","doi-asserted-by":"publisher","DOI":"10.1053\/j.ajkd.2005.05.021"},{"key":"2025082801471984000_15.8.e248461.10","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfq232"},{"key":"2025082801471984000_15.8.e248461.11","doi-asserted-by":"crossref","first-page":"1004","DOI":"10.1016\/j.ekir.2018.04.001","article-title":"IgM-Positive tubulointerstitial nephritis associated with asymptomatic primary biliary cirrhosis","volume":"3","author":"Mizoguchi","year":"2018","journal-title":"Kidney Int Rep"},{"key":"2025082801471984000_15.8.e248461.12","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1007\/s00467-016-3394-5","article-title":"Tubulointerstitial nephritis: diagnosis, treatment, and monitoring","volume":"32","author":"Joyce","year":"2017","journal-title":"Pediatr Nephrol"},{"key":"2025082801471984000_15.8.e248461.13","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/16.4.867"},{"key":"2025082801471984000_15.8.e248461.14","doi-asserted-by":"publisher","DOI":"10.1016\/S0272-6386(12)70327-X"},{"key":"2025082801471984000_15.8.e248461.15","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfm189"},{"key":"2025082801471984000_15.8.e248461.16","first-page":"36","article-title":"Interstitial nephritis and primary biliary cirrhosis: a new association?","volume":"27","author":"Macdougall","year":"1987","journal-title":"Clin Nephrol"},{"key":"2025082801471984000_15.8.e248461.17","first-page":"401","article-title":"Tubulointerstitial nephritis with renal tubular acidosis and asymptomatic primary biliary cirrhosis accompanied by antibody to a 52-kDa mitochondrial protein alone","volume":"45","author":"Kodama","year":"1996","journal-title":"Clin Nephrol"},{"key":"2025082801471984000_15.8.e248461.18","doi-asserted-by":"publisher","DOI":"10.1093\/ckj\/sfs085"},{"key":"2025082801471984000_15.8.e248461.19","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.52.9003"},{"key":"2025082801471984000_15.8.e248461.20","first-page":"29","article-title":"Tubulointerstitial nephritis associated with primary biliary cirrhosis","volume":"3","author":"Rasolzadegan","year":"2014","journal-title":"J Nephropharmacol"},{"key":"2025082801471984000_15.8.e248461.21","doi-asserted-by":"publisher","DOI":"10.1186\/s12882-015-0184-4"},{"key":"2025082801471984000_15.8.e248461.22","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2016101074"},{"key":"2025082801471984000_15.8.e248461.23","doi-asserted-by":"crossref","first-page":"3269","DOI":"10.1002\/hep.32011","article-title":"The serum proteome and ursodeoxycholic acid response in primary biliary cholangitis","volume":"74","author":"Barron-Millar","year":"2021","journal-title":"Hepatology"},{"key":"2025082801471984000_15.8.e248461.24","doi-asserted-by":"publisher","DOI":"10.1093\/qjmed\/hct258"},{"key":"2025082801471984000_15.8.e248461.25","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0098261","article-title":"Assessment of proteinuria in patients with chronic kidney disease stage 3: albuminuria and non-albumin proteinuria","volume":"9","author":"Fraser","year":"2014","journal-title":"PLoS One"},{"key":"2025082801471984000_15.8.e248461.26","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1007\/s12325-021-01994-2","article-title":"Chronic kidney disease and SGLT2 inhibitors: a review of the evolving treatment landscape","volume":"39","author":"Mende","year":"2022","journal-title":"Adv Ther"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2021-248461","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T08:47:30Z","timestamp":1756370850000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2021-248461"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8]]},"references-count":26,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2022,8,16]]},"published-print":{"date-parts":[[2022,8]]}},"alternative-id":["10.1136\/bcr-2021-248461"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2021-248461","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2022,8]]}}}