{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T11:00:46Z","timestamp":1790074846091,"version":"4.0.1"},"reference-count":11,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,5,26]],"date-time":"2021-05-26T00:00:00Z","timestamp":1621987200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,5,26]],"date-time":"2021-05-26T00:00:00Z","timestamp":1621987200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Rheumatol"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Polyarteritis nodosa is a type of vasculitis affecting medium- and small-sized arteries that has been associated with hepatitis B but does not have an established relationship with autoimmune hepatitis. Here we report the case of an adult patient with autoimmune hepatitis who, shortly after diagnosis, developed life-threatening polyarteritis nodosa.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>A 45-year-old woman was diagnosed with autoimmune hepatitis after initially presenting with a two-month history of fatigue, nausea, and anorexia and a three-week history of scleral icterus. Her liver biopsy showed mild portal fibrosis and her liver chemistries improved with prednisone and azathioprine. Three months later, she presented to the emergency department with fever, bilateral ankle pain, rash, oral ulcers, and poor vision. Physical examination was notable for erythema nodosum, anterior uveitis, retinal vasculitis, and frosted branch angiitis (frosted branch angiitis (a widespread florid translucent perivascular exudate). She subsequently developed repeated episodes of ischemic acute bowel necrosis that required multiple surgeries and extensive small bowel resections. Surgical pathology of the small bowel resection revealed ischemic necrosis, medium and small vessel vasculitis with microvascular thrombi consistent with polyarteritis nodosa. Azathioprine was discontinued and she was treated with pulse steroids followed by a prednisone taper, cyclophosphamide, and intravenous immune globulin with overall improvement in her symptomatology. Since her hospitalization, she has been maintained on low-dose prednisone and mycophenolate mofetil.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>In patients with recent diagnosis of autoimmune hepatitis, there should be a modest suspicion for concomitant polyarteritis nodosa if symptoms and signs of multisystem vasculitis develop.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s41927-021-00188-1","type":"journal-article","created":{"date-parts":[[2021,5,25]],"date-time":"2021-05-25T23:06:25Z","timestamp":1621983985000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["A rare case of Polyarteritis Nodosa associated with autoimmune hepatitis: a case report"],"prefix":"10.1186","volume":"5","author":[{"given":"Freda","family":"Kennedy","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel","family":"Kapelow","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bilge D.","family":"Kalyon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nitzan C.","family":"Roth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arvind","family":"Rishi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7702-5826","authenticated-orcid":false,"given":"Maria-Louise","family":"Barilla-LaBarca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,5,26]]},"reference":[{"issue":"6","key":"188_CR1","doi-asserted-by":"publisher","first-page":"564","DOI":"10.1016\/j.autrev.2016.02.015","volume":"15","author":"A De Virgilio","year":"2016","unstructured":"De Virgilio A, Greco A, Magliulo G, Gallo A, Ruoppolo G, Conte M, et al. Polyarteritis nodosa: a contemporary overview. Autoimmun Rev. 2016;15(6):564\u201370. https:\/\/doi.org\/10.1016\/j.autrev.2016.02.015.","journal-title":"Autoimmun Rev"},{"issue":"2","key":"188_CR2","first-page":"52","volume":"37 Suppl 117","author":"HE S\u00f6nmez","year":"2019","unstructured":"S\u00f6nmez HE, Arma\u011fan B, Ayan G, Barut K, Batu ED, Erden A, et al. Polyarteritis nodosa: lessons from 25 years of experience. Clin Exp Rheumatol. 2019;37 Suppl 117(2):52\u20136.","journal-title":"Clin Exp Rheumatol"},{"issue":"2","key":"188_CR3","doi-asserted-by":"publisher","first-page":"omx100","DOI":"10.1093\/omcr\/omx100","volume":"2018","author":"M Roberto","year":"2018","unstructured":"Roberto M, Meytes V, Liu S. Ruptured hepatic aneurysm as first presenting symptom of polyarteritis nodosa. Oxf Med Case Rep. 2018;2018(2):omx100.","journal-title":"Oxf Med Case Rep"},{"issue":"1A","key":"188_CR4","doi-asserted-by":"publisher","first-page":"23S","DOI":"10.1016\/0002-9343(94)90186-4","volume":"96","author":"EL Krawitt","year":"1994","unstructured":"Krawitt EL. Autoimmune hepatitis: classification, heterogeneity, and treatment. Am J Med. 1994;96(1A):23S\u20136S. https:\/\/doi.org\/10.1016\/0002-9343(94)90186-4.","journal-title":"Am J Med"},{"issue":"1","key":"188_CR5","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1097\/SMJ.0b013e3181fd0be6","volume":"104","author":"O Giouleme","year":"2011","unstructured":"Giouleme O, Mpoumponaris A, Aslanidis S, Anagnostis P, Giamalis P, Nikolaidis N, Vasiliadis T, Evgenidis N. Polyarteritis nodosa in a patient with type 1 autoimmune hepatitis. South Med J. 2011;104(1):49\u201352. https:\/\/doi.org\/10.1097\/SMJ.0b013e3181fd0be6.","journal-title":"South Med J"},{"issue":"4","key":"188_CR6","doi-asserted-by":"publisher","first-page":"442","DOI":"10.2340\/00015555-0659","volume":"89","author":"WJ Lee","year":"2009","unstructured":"Lee WJ, Kim CH, Chang SE, Lee MW, Choi JH, Moon KC, Koh JK. A case of cutaneous polyarteritis nodosa in autoimmune hepatitis. Acta Derm Venereol. 2009;89(4):442\u20133. https:\/\/doi.org\/10.2340\/00015555-0659.","journal-title":"Acta Derm Venereol"},{"issue":"12","key":"188_CR7","doi-asserted-by":"publisher","first-page":"1775","DOI":"10.1016\/S0161-6420(93)31405-3","volume":"100","author":"YA Akova","year":"1993","unstructured":"Akova YA, Jabbur NS, Foster CS. Ocular presentation of polyarteritis nodosa. Clinical course and management with steroid and cytotoxic therapy. Ophthalmology. 1993;100(12):1775\u201381. https:\/\/doi.org\/10.1016\/S0161-6420(93)31405-3.","journal-title":"Ophthalmology."},{"issue":"3","key":"188_CR8","doi-asserted-by":"publisher","first-page":"452","DOI":"10.1007\/s12016-017-8612-9","volume":"53","author":"F Chasset","year":"2017","unstructured":"Chasset F, Franc\u00e8s C. Cutaneous manifestations of medium- and large-vessel Vasculitis. Clin Rev Allergy Immunol. 2017;53(3):452\u201368. https:\/\/doi.org\/10.1007\/s12016-017-8612-9.","journal-title":"Clin Rev Allergy Immunol"},{"issue":"2","key":"188_CR9","doi-asserted-by":"publisher","first-page":"616","DOI":"10.1002\/art.27240","volume":"62","author":"C Pagnoux","year":"2010","unstructured":"Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, le Guern V, Bienvenu B, Mouthon L, Guillevin L, French Vasculitis Study Group. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis study group database. Arthritis Rheum. 2010;62(2):616\u201326. https:\/\/doi.org\/10.1002\/art.27240.","journal-title":"Arthritis Rheum"},{"issue":"11","key":"188_CR10","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1007\/s11926-017-0699-8","volume":"19","author":"H Hashem","year":"2017","unstructured":"Hashem H, Kelly SJ, Ganson NJ, Hershfield MS. Deficiency of adenosine Deaminase 2 (DADA2), an inherited cause of Polyarteritis Nodosa and a mimic of other systemic rheumatologic disorders. Curr Rheumatol Rep. 2017;19(11):70. https:\/\/doi.org\/10.1007\/s11926-017-0699-8.","journal-title":"Curr Rheumatol Rep"},{"issue":"1","key":"188_CR11","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1007\/s00296-017-3740-3","volume":"38","author":"S Sahin","year":"2018","unstructured":"Sahin S, Adrovic A, Barut K, Ugurlu S, Turanli ET, Ozdogan H, Kasapcopur O. Clinical, imaging and genotypical features of three deceased and five surviving cases with ADA2 deficiency. Rheumatol Int. 2018;38(1):129\u201336. https:\/\/doi.org\/10.1007\/s00296-017-3740-3.","journal-title":"Rheumatol Int"}],"container-title":["BMC Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s41927-021-00188-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s41927-021-00188-1\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s41927-021-00188-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,5,25]],"date-time":"2021-05-25T23:06:32Z","timestamp":1621983992000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcrheumatol.biomedcentral.com\/articles\/10.1186\/s41927-021-00188-1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5,26]]},"references-count":11,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["188"],"URL":"https:\/\/doi.org\/10.1186\/s41927-021-00188-1","relation":{},"ISSN":["2520-1026"],"issn-type":[{"value":"2520-1026","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,5,26]]},"assertion":[{"value":"23 October 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 March 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"26 May 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The need for approval was waived from the institutional review board of Feinstein Institutes for Medical Research Northwell Health.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"17"}}