{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,2]],"date-time":"2026-01-02T07:45:24Z","timestamp":1767339924029,"version":"3.44.0"},"reference-count":24,"publisher":"BMJ","issue":"10","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2019,9,25]]},"published-print":{"date-parts":[[2019,10]]},"abstract":"<jats:p>We describe a patient who was admitted to our medical centre with acute renal failure, hyponatraemia and hyperkalaemia. CT of the abdomen and pelvis showed a retroperitoneal mass with bilateral ureteral obstruction. Biopsy revealed fibrosis with inflammatory infiltrate, but rare IgG<jats:sub>4<\/jats:sub>-positive plasma cells. After placement of bilateral pigtail nephrostomy catheters, renal failure improved but metabolic derangements remained. Morning serum cortisol level was equivocal, but with blunted response on cosyntropin stimulation testing indicating adrenal insufficiency. Serology for 21-hydroxylase antibodies was strongly positive, supporting the diagnosis of Addison\u2019s disease. In addition to nephrostomy catheters for obstructive uropathy, idiopathic retroperitoneal fibrosis was treated with mycophenolate mofetil. Physiological doses of hydrocortisone and fludrocortisone for Addison\u2019s disease were also initiated. The patient continues to be monitored for regression of the mass. Based on review of the literature, this is the first reported case of IgG<jats:sub>4<\/jats:sub>-negative idiopathic retroperitoneal fibrosis presenting with autoimmune primary adrenal insufficiency.<\/jats:p>","DOI":"10.1136\/bcr-2019-230767","type":"journal-article","created":{"date-parts":[[2019,10,14]],"date-time":"2019-10-14T05:35:16Z","timestamp":1571031316000},"page":"e230767","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["Concomitant presentation of IgG<sub>4<\/sub>-negative idiopathic retroperitoneal fibrosis and Addison\u2019s disease"],"prefix":"10.1136","volume":"12","author":[{"given":"Mark","family":"Riley","sequence":"first","affiliation":[{"name":"Department of Medicine, Albany Medical Center, Albany, New York, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3740-7790","authenticated-orcid":false,"given":"Muhammad Hamza","family":"Saad Shaukat","sequence":"additional","affiliation":[{"name":"Department of Medicine, Albany Medical Center, Albany, New York, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammed","family":"Bari","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Rheumatology, Albany Medical Center, Albany, New York, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruben","family":"Peredo-Wende","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Rheumatology, Albany Medical Center, Albany, New York, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2019,10,13]]},"reference":[{"doi-asserted-by":"publisher","key":"2025082708080999000_12.10.e230767.1","DOI":"10.1097\/MD.0b013e3181afc420"},{"doi-asserted-by":"publisher","key":"2025082708080999000_12.10.e230767.2","DOI":"10.1016\/S0140-6736(06)68035-5"},{"doi-asserted-by":"publisher","key":"2025082708080999000_12.10.e230767.3","DOI":"10.3899\/jrheum.130324"},{"doi-asserted-by":"publisher","key":"2025082708080999000_12.10.e230767.4","DOI":"10.1681\/ASN.2015101110"},{"key":"2025082708080999000_12.10.e230767.5","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1111\/j.1365-2559.1990.tb01541.x","article-title":"The spectrum of chronic periaortitis","volume":"16","author":"Parums","year":"1990","journal-title":"Histopathology"},{"key":"2025082708080999000_12.10.e230767.6","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000012631","article-title":"Rituximab for idiopathic and IgG4-related retroperitoneal fibrosis","volume":"97","author":"Wallwork","year":"2018","journal-title":"Medicine"},{"doi-asserted-by":"crossref","unstructured":"Capoccia L , Riambau V , Cochrane Vascular Group . 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