Introduction: The simultaneous presence of Takayasu's arteritis and Crohn's disease in a patient seems to be rare. To our knowledge, no patient with the combination of Crohn's disease and Takayasu's arteritis has been reported from our region. Case presentation: Herein we present the case of a 22-year-old Iranian woman previously diagnosed as Crohn's disease and who had subsequently developed Takayasu's arteritis. Conclusion: Clinical suspicion, proper imaging, and consideration of the differential diagnosis are important for the correct diagnosis and management of patients with this coincidence. Â© 2008 Daryani et al; licensee BioMed Central Ltd

Ajdarkosh, H.

Azmi, M.

Bashashati, M.

Daryani, N.E.

Habibi, A.N.

Keramati, M.R.

Rafiee, M.J.

eprints Iran University of Medical Sciences

BioMed CentralJournal of Medical Case ReportsssOpen AcceCase reportTakayasu's arteritis associated with Crohn's disease: a case reportNasser E Daryani†1, Atoosa Nayer Habibi*†2, Mohammad Bashashati†2, Mohammad Reza Keramati2, Moezedin Javad Rafiee†3, Hossein Ajdarkosh†4 and Mehrdad Azmi†5Address: 1Department of Gastroenterology, Imam Khomeini Hospital, Tehran University of Medical Sciences, Iran, 2Researcher of Gastroenterology, Imam Khomeini Hospital, Tehran University of Medical Sciences, Iran, 3Babak Imaging Center, Tehran, Iran, 4Department of Gastroenterology, Haft-e-Tir Hospital, Iran University of Medical Sciences, Iran and 5Cancer Institute of Imam Khomeini Hospital, Tehran University of Medical Sciences, IranEmail: Nasser E Daryani - nebrahim@sina.tums.ac.ir; Atoosa Nayer Habibi* - anayery70@yahoo.com; Mohammad Bashashati - bashashati_md@yahoo.com; Mohammad Reza Keramati - dr_morezak@yahoo.com; Moezedin Javad Rafiee - ajdarkosh1345@yahoo.com; Hossein Ajdarkosh - ajdarkosh1345@yahoo.com; Mehrdad Azmi - mehrdad_azmi@hotmail.com* Corresponding author    †Equal contributorsAbstractIntroduction: The simultaneous presence of Takayasu's arteritis and Crohn's disease in a patientseems to be rare. To our knowledge, no patient with the combination of Crohn's disease andTakayasu's arteritis has been reported from our region.Case presentation: Herein we present the case of a 22-year-old Iranian woman previouslydiagnosed as Crohn's disease and who had subsequently developed Takayasu's arteritis.Conclusion: Clinical suspicion, proper imaging, and consideration of the differential diagnosis areimportant for the correct diagnosis and management of patients with this coincidence.IntroductionCoincidence of Takayasu's arteritis (TA) and Crohn's dis-ease (CD) is exceedingly rare and raises questions aboutthe possibility of similar causes or etiopathogenic mecha-nisms [1]. The chance of both diseases occurring in thesame individual has been estimated to be one in 1010 per-sons [2]. Recent studies have revealed that 3–9 percent ofpeople with TA may also have CD [2-4]. To our knowl-edge, there have no reports of simultaneous presence ofCD and TA in a patient from our region. Herein wepresent a case of an Iranian woman previously diagnosedwith CD and who subsequently developed TA.Case presentationThis patient is a 22-year-old woman with 2 and a half yearhistory of CD who has been treated with sulfasalazine andazathioprine since diagnosis. The diagnosis of CD hadbeen established by colonoscopy which showed regionalinflammation and ulceration in the sigmoid colon andthe proximal part of the ascending colon. Histologyrevealed granulomatous colitis, inflamed mucosa of thelarge intestine with inflammatory epithelial damage, andfocal mild crypt distortion. Barium meal and small bowelfollow-through were normal.Since her diagnosis of CD, she has had episodes of relapsewith symptoms including fever, weight loss, diarrhoeaPublished: 19 March 2008Journal of Medical Case Reports 2008, 2:87 doi:10.1186/1752-1947-2-87Received: 30 April 2007Accepted: 19 March 2008This article is available from: http://www.jmedicalcasereports.com/content/2/1/87© 2008 Daryani et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 4(page number not for citation purposes)Journal of Medical Case Reports 2008, 2:87 http://www.jmedicalcasereports.com/content/2/1/87and arthralgia in the form of inflammatory monoarthritisof the ankle.Recently the patient presented with symptoms which weredifferent from those seen in her previous relapses. She hadmalaise, weight loss (7 kg in 6 months), hematocheziaand arthralgia, although this time both knees wereaffected symmetrically with an inflammatory pattern. Onphysical examination, the patient was afebrile with nor-mal blood pressure. Both radial pulses were normal andsymmetrical with a bruit heard over the abdominal aorta,but no carotid or clavicular bruits were detected.Laboratory tests showed a considerable rise in ESR (100mm/h) with white blood cell count, 7800/µL; hemo-globin, 10.0 grams/dl; platelets 500000/µL.Colonoscopy revealed edematous mucosa and ulcerationin the ascending colon without any other lesions in therectosigmoid or other parts of the colon. Barium meal andsmall bowel follow-through were performed and showedno lesion in the oesophagus, stomach, or small intestineincluding the terminal ileum and cecum.Abdominal CT scan showed thickening of the abdominalaorta (4 mm) and narrowing of renal arteries which wassuggestive of TA (Figure 1).To confirm the diagnosis of TA, a CT angiography was alsodone, which showed marked circumferential mural thick-ening in branches of the thoracic aortic arch (left commoncarotid artery and subclavian arteries), right innominateartery, aortic arch and descending aorta which was con-sistent with Takayasu's arteritis (type v, diffuse involve-ment) (Figure 2).With the diagnosis of Takayasu's arteritis, in associationwith Crohn's disease, oral prednisolone (30 mg/day) wasstarted plus continuation of her previous medication, i.e.sulfasalazine (3 g/day) and azathioprine (100 mg/day).She became symptomless after 4 weeks of treatment, atwhich time the prednisolone dose was tapered and dis-continued over 8 weeks. Her treatment was continuedwith sulfasalazine and azathioprine with dosage of 3 gand 100 mg per day respectively. Five months after dis-continuing prednisolone she has remained symptomlesswith a normal ESR.DiscussionThe first reported case of concomitant occurrence ofinflammatory bowel disease (IBD) and Takayasu arteritiswas in a 35-year-old woman with ulcerative colitis (UC)in 1991 [5]. To our knowledge, 37 patients with UC andTA have been reported since then. In a literature review,25 patients were reported with concomitant CD and TA.Although, TA rarely occurs in a concomitant manner, thisshould not be perceived as an unexpected association[3,4].In our clinic, about 120 patients with CD were registeredover a six year period, and the reported case was the firstconcomitant occurrence of CD and TA.According to previous studies, Takayasu's arteritis mostlypresents simultaneously or after presentation of CD. It isunlikely to precede CD manifestations. It seems that onedisease triggers or worsens the other [2]. Unfortunately,there have been no prospective studies to prove this phe-nomenon.Most of the reported patients with CD and TA were youngwomen with early manifestations of TA [2,6]. As TAmostly presents in young women [7], it could be sug-gested that patients with inflammatory bowel diseaseswould show features of TA in the same age-sex pattern ofthe IBD.TA mainly manifests with an absent radial pulse, fever,arthralgia, night sweats and myalgia [7], but in peoplewith Crohn's Disease it may be clinically silent [2]. Due tothe involvement of the aorta and its vasculature, TA maypresent with gastrointestinal bleeding [6]. Since most ofthese features may also be present in CD, there might be adiagnostic dilemma when there is presentation of bothCD and TA.HLA haplotypes (HLA-A25, B52, DW12, and DR2), andthe involvement of immune complexes, and organ spe-Abdominal CT scan: thickening of the aortic wall (arrow)Figure 1Abdominal CT scan: thickening of the aortic wall (arrow).Page 2 of 4(page number not for citation purposes)Journal of Medical Case Reports 2008, 2:87 http://www.jmedicalcasereports.com/content/2/1/87cific antibodies have been reported [1,2,8,9]. However, nodirect genetic link has been reported and further studieson these associations are recommended [2]. Granulomasand granulomatous vasculitis are seen in both CD and TA.Granulomatous vasculitis shows a common pathophysi-ology in both diseases [6]. Location of the lesion andsometimes the age of the patient, are two factors that candifferentiate between TA and other forms of vasculitiswhich may coexist with CD, i.e. giant cell (temporal)arteritis is the most difficult vasculitis to distinct from TAbut it primarily involves external carotid artery branchesand mostly occurs after 50 years of age [10,11]. Moreover,TA can be distinguished from temporal arteritis by pre-dominant lesions at the media-adventitia junction onpathological evaluation [9], although this is mostlyimpractical.Corticosteroids are the mainstay of treatment in active TA,and cytotoxic agents are reserved for patients with steroidresistance or who experience relapse [7]. Anti-tumornecrosis factor (TNF) agents might be another treatmentpossibility [7,12]. Recently, a study which assessed theeffect of azathioprine and prednisolone in combinationon TA showed that this combination is effective in con-trolling systemic symptoms and disease activity [13].Therefore, the drugs that are utilised to treat TA and CDare often the same. Although diagnosis of the diseases incombination may not make any obvious difference in anindividual patient's treatment, early diagnosis is critical,because TA can compromise intestinal vasculature andmay present with gastrointestinal bleeding, complicatingthe diagnosis and perhaps exacerbating the clinical courseof patients with IBD [6].ConclusionTakayasu's arteritis affects the aorta and its vasculatureand may compromise intestinal vessels. Therefore, it maypresent with gastrointestinal bleeding and exacerbate theclinical course of patients with inflammatory bowel dis-ease. Clinical suspicion, proper imaging, and considera-tion of the differential diagnosis are important for thecorrect diagnosis and management of patients with bothTakayasu's arteritis and Crohn's disease.Competing interestsThe author(s) declare that they have no competing inter-ests.Authors' contributionsAll authors contributed equally to this case report exceptfor MRK who contributed on the revision of the manu-scriptConsentWritten informed consent was obtained from the patientfor publication of this Case report and any accompanyingimages. A copy of the written consent is available forreview by the Editor-in-Chief of this journal.References1. Houman MH, Doghri A, Boubaker J, Kchir MN, Mahdhaoui A, Filali A,Miled M: Takayasu disease in Crohn disease: an exceptionalassociation.  Ann Gastroenterol Hepatol (Paris) 1996, 31(6):337-40.2. Reny JL, Paul JF, Lefebvre C, Champion K, Emmerich J, Bletry O, Pie-tte JC, Fiessinger JN: Association of Takayasu's arteritis andCrohn's disease. Results of a study on 44 Takayasu patientsand review of the literature.  Ann Med Interne (Paris) 2003,154:85-90.3. Hall S, Barr W, Lie JT, Stanson AW, Kazmier FJ, Hunder GG: Taka-yasu arteritis. A study of 32 North American patients.  Medi-cine 1985, 64:89-99.4. Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rotten M,Hoffman GS: Takayasu arteritis.  Ann Intern Med 1994, 120:919-29.5. Klippel JH, Weyand CM, Wortmann RL: Takayasu's arteritis.  InPrimer on the rheumatic diseases 11th edition. Edited by: Klippel JH.Atlanta: Arthritis Foundation; 1991:294-5. 6. Levitsky J, Harrison JR, Cohen RD: Crohn's disease and Taka-yasu's arteritis.  J Clin Gastroenterol 2002, 34:454-6.7. Mubashir Ahmed M, Wolf RE: Takayasu Arteritis.   [http://www.emedicine.com/MED/topic2232.htm].8. Sato R, Sato Y, Ishikawa H, Oshima Y, Suzuki T, Watanabe S, OyanagiH, Sekine K, Kondo Y, Miyata M, Obara K, Nishimaki T, Kasukawa R:Takayasu's Disease Associated with Ulcerative Colitis .  InternMed 1994, 33(12):759-763.9. Todini AR, Heinzmann MM, Antignani PL, Paiella ML: Associationbetween Takayasu's arteritis and Crohn's disease in twoyoung women: case report.  J Mal Vasc 1999, 24:373-6.10. Hunder GG: Clinical features and diagnosis of Takayasuarteritis.   [http://www.uptodate.com/home/index.html].11. Hunder GG: Classification of and approach to the vasculitidesin adults.   [http://www.uptodate.com/home/index.html].12. Hoffman GS, Merkel PA, Brasington RD, Lenschow DJ, Liang P: Anti-tumor necrosis factor therapy in patients with difficult totreat Takayasu arteritis.  Arthritis Rheum 2004, 50:2296-304.thoracic CT angiography: thickening of the aortic wall (arrow)Figure 2thoracic CT angiography: thickening of the aortic wall (arrow).Page 3 of 4(page number not for citation purposes)Journal of Medical Case Reports 2008, 2:87 http://www.jmedicalcasereports.com/content/2/1/87Publish with BioMed Central   and  every scientist can read your work free of charge"BioMed Central will be the most significant development for disseminating the results of biomedical research in our lifetime."Sir Paul Nurse, Cancer Research UKYour research papers will be:available free of charge to the entire biomedical communitypeer reviewed and published immediately upon acceptancecited in PubMed and archived on PubMed Central yours — you keep the copyrightSubmit your manuscript here:http://www.biomedcentral.com/info/publishing_adv.aspBioMedcentral13. Valsakumar AK, Valappil UC, Jorapur V, Garg N, Nityanand S, SinhaN: Role of immunosuppressive therapy on clinical, immuno-logical, and angiographic outcome in active Takayasu'sarteritis.  J Rheumatol 2003, 30:1793-8.Page 4 of 4(page number not for citation purposes)

Takayasu's arteritis associated with Crohn's disease: A case report

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Takayasu's arteritis associated with Crohn's disease: A case report

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