PMID- 26778729 OWN - NLM STAT- MEDLINE DCOM- 20180117 LR - 20180117 IS - 1440-1827 (Electronic) IS - 1320-5463 (Linking) VI - 66 IP - 3 DP - 2016 Mar TI - A case of 37 year long Behcet disease resembling Takayasu arteritis: An autopsy report. PG - 164-168 LID - 10.1111/pin.12380 [doi] AB - A 19-year-old woman with a history of recurrent aphthous stomatitis and genital ulceration was diagnosed with Behcet disease. She was treated with steroids and immunosuppressive agents for more than 30 years, but multiple complications manifested including ileocecal ulcer, aortic valve regurgitation, renal failure, ischemic enterocolitis, and arteriosclerotic obliterans until her death at the age of 56 from pneumonia. An autopsy examination demonstrated an entirely calcified aorta and major aortic branches. The ascending aorta was dilatated 55 mm in diameter and branches were all stenosed. Microscopically, the aortic arch and its branches showed collagenous fibrosis of the outer media and adventitia, whereas coronary and abdominal aortic branches showed conventional atherosclerosis. Although the ante-mortem diagnosis was angio-Behcet disease, its pathophysiology along with her clinical history, morphology of the lead pipe-like aorta, predominant destruction of the outer arteries, and a human leukocyte antigen (HLA) haplotype of B39 were all suggestive of Takayasu arteritis. Thus, this case implies that HLA-B39 may be associated with the pathogenesis of arteritis like Takayasu arteritis, even if the primary disease is Behcet disease. CI - (c) 2016 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd. FAU - Sato, Naoko AU - Sato N AD - Department of Diagnostic Pathology, Kitakyushu General Hospital, Kitakyushu, Japan. AD - Department of Pathology, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Irie, Koji AU - Irie K AD - Department of Diagnostic Pathology, Kitakyushu General Hospital, Kitakyushu, Japan. FAU - Kouzuma, Ryoji AU - Kouzuma R AD - Department of Cardiology, Kitakyushu General Hospital, Kitakyushu, Japan. FAU - Inoue, Katsumi AU - Inoue K AD - Department of Laboratory Medicine, Kokura Memorial Hospital, Kitakyushu, Japan. FAU - Nakayama, Toshiyuki AU - Nakayama T AD - Department of Pathology, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. LA - eng PT - Case Reports DEP - 20160118 PL - Australia TA - Pathol Int JT - Pathology international JID - 9431380 RN - 0 (HLA-B39 Antigen) SB - IM MH - Aorta/pathology MH - Aorta, Thoracic/pathology MH - Aortic Valve Insufficiency MH - Autopsy MH - Behcet Syndrome/complications/*diagnosis/drug therapy/pathology MH - Fatal Outcome MH - Female MH - Fibrosis/etiology/pathology MH - HLA-B39 Antigen/*metabolism MH - Humans MH - Pneumonia/complications MH - Takayasu Arteritis/diagnosis/*etiology/pathology MH - Vascular Calcification MH - Young Adult OTO - NOTNLM OT - Angio-Behcet disease OT - HLA-B39 OT - Takayasu arteritis EDAT- 2016/01/19 06:00 MHDA- 2018/01/18 06:00 CRDT- 2016/01/19 06:00 PHST- 2015/10/13 00:00 [received] PHST- 2015/12/16 00:00 [accepted] PHST- 2016/01/19 06:00 [pubmed] PHST- 2018/01/18 06:00 [medline] PHST- 2016/01/19 06:00 [entrez] AID - 10.1111/pin.12380 [doi] PST - ppublish SO - Pathol Int. 2016 Mar;66(3):164-168. doi: 10.1111/pin.12380. Epub 2016 Jan 18.