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A renal biopsy showed a tubulointerstitial nephritis with lymphoplasmacytic infiltrates, fibrosis and IgG4-positive plasma cells on immunohistochemistry. A diagnosis of IgG4-related disease was made based on clinical presentation and pathology. Renal function improved with glucocorticoids and the patient was successfully transitioned to azathioprine as a steroid-sparing agent.<\/jats:p>","DOI":"10.1136\/bcr-2021-241942","type":"journal-article","created":{"date-parts":[[2021,11,11]],"date-time":"2021-11-11T07:16:37Z","timestamp":1636614997000},"page":"e241942","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["IgG4-related tubulointerstitial nephritis"],"prefix":"10.1136","volume":"14","author":[{"given":"Tasnim","family":"Momoniat","sequence":"first","affiliation":[{"name":"Renal Department, Hull Royal Infirmary, Hull, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepa","family":"Jacob","sequence":"additional","affiliation":[{"name":"Histopathology Department, Hull Royal Infirmary, Hull, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neelaveni","family":"Duhli","sequence":"additional","affiliation":[{"name":"Histopathology Department, Hull Royal Infirmary, Hull, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tom","family":"Jorna","sequence":"additional","affiliation":[{"name":"Renal Department, Hull Royal Infirmary, Hull, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2021,11,11]]},"reference":[{"key":"2025082622564367000_14.11.e241942.1","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2018-000787","article-title":"Igg4-Related diseases: state of the art on clinical practice guidelines","volume":"4","author":"Iaccarino","year":"2018","journal-title":"RMD Open"},{"key":"2025082622564367000_14.11.e241942.2","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1056\/NEJMra1104650","article-title":"IgG4\u2010related disease","volume":"365","author":"Stone","year":"2012","journal-title":"N Engl J Med"},{"key":"2025082622564367000_14.11.e241942.3","doi-asserted-by":"publisher","DOI":"10.1002\/art.34593"},{"key":"2025082622564367000_14.11.e241942.4","unstructured":"Haralampos MM , Fragoulis GE , Stone JH . 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