QJM Advance Access published March 30, 2015 QJM: An International Journal of Medicine, 2015, 1–2 doi: 10.1093/qjmed/hcv063 Advance Access Publication Date: 14 March 2015 Case report
CASE REPORT
Takayasu arteritis and ischaemic stroke Y. Maeda1, H. Taguchi2, T. Kudo2 and Y. Okano2 From the 1Department of Internal Medicine and 2Center for Arthritis and Rheumatic Disease, Kawasaki Municipal Kawasaki Hospital, Japan Address correspondence to Y. Maeda, 12-1, Shinkawadori, Kawasaki-ku, Kawasaki, 210-0013 Japan. email: [email protected]
Learning point for clinicians
Discussion Case report A 79-year-old Japanese man visited our hospital with an 8 week history of low-grade fever and high-serum C-reactive protein (CRP) levels. He also had a history of hypertension and hyperlipidemia, although his familial history was unremarkable. His laboratory data revealed a white blood cell count of 8090/ml and CRP levels of 13.2 mg/dl. No antinuclear antibodies, antineutrophil cytoplasmic antibodies or syphilis was detected. Contrastenhanced computed tomography (CECT) revealed aortic wall thickening from the ascending section to the common iliac artery (Figure 1a), which indicated large vessel vasculitis (LVV). Two weeks later, he developed right hemiparesis and dysarthria and was transferred to our emergency room. He did not complain of headache, blurred vision, jaw claudication or myalgia during the course. On admission, different systolic blood pressures were observed in each arm (right: 135 mmHg, left: 160 mmHg), and bruit was not audible over the carotid artery. He had no induration or tenderness of his temporal arteries. Diffusion-weighted magnetic resonance imaging (MRI) revealed high-signal areas at the left corona radiata (Figure 1b). We diagnosed the patient with ischemic stroke complicated with LVV, and began oral prednisone at 0.5 mg/kg/d. Although the patient’s serum CRP levels rapidly decreased, he developed ischemic colitis with diffuse colonic ulcers 4 days
We report a patient who developed ischemic stroke as the initial manifestation of vessel stenosis related to LVV. Takayasu arteritis (TAK) and giant cell arteritis (GCA) are the two main causes of LVV, although they are difficult to differentiate, given their many similarities. In recent years, CECT, MRI and fluorodeoxy glucose-positron emission tomography have been proven to detect large vessel inflammation in both TAK and GCA.1,2 However, the histopathology of the arterial lesions in these diseases may be indistinguishable. Therefore, some physicians have argued that these two disorders represent a spectrum within the same disease.3 Nevertheless, it is important to differentiate between TAK and GCA, as their complications and managements can be different. To differentiate between these two diseases, age and sex are important considerations, as most TAK patients are young women who are 50-year-olds.4 In addition, the distribution of lesions can differentiate TAK from GCA, as almost all parts of aorta can be invaded in TKA, while the external carotid artery and its branches are more susceptible in GCA.5 Furthermore, HLA typing is helpful in distinguishing TAK from GCA. The HLA haplotyping of Japanese patients with TAK has revealed a higher frequency for HLA B39, B52 and DR2, compared with a high frequency of HLA DR3, DR4, DR5 and DRB1 in GCA.6 Our case of TAK was unusual, as the patient was an older man, although we diagnosed TAK based on the absence of
C The Author 2015. Published by Oxford University Press on behalf of the Association of Physicians. V
All rights reserved. For Permissions, please email: [email protected]
1
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Takayasu arteritis (TAK) and giant cell arteritis (GCA) have similar features, and some physicians believe that they exist as a spectrum, and are only differentiated by onset age. However, if GCA can be excluded, TAK is a possible differential diagnosis for large vessel vasculitis in men who are >50-year-olds.
later, which was confirmed by colonoscopy. Given the insufficient control of the vasculitis, we escalated the prednisone dose to 1 mg/kg/d. After prednisone escalation, no haematochezia or neurological symptoms recurred. After rehabilitation and tapering the prednisone for 6 weeks, the patient was discharged while receiving 40 mg/day of prednisone. Human leukocyte antigen (HLA) testing revealed HLA-A2, A24, B39, B52, DR2 (DR15) and DR6 (DR14).
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QJM: An International Journal of Medicine, 2015, Vol. 0, No. 0
Figure 1. (a). Inflammatory thickening of aortic wall from ascending part to common iliac artery was seen in CECT. (b). High-signal areas at left corona radiata in the diffusion-weighted MRI.
Conflict of interest: None declared.
References 1. Hellmann DB. Giant cell arteritis, polymyalgia rheumatica, and Takayasu’s arteritis. In: GS Firestein, RC Budd, SE
Gabrieleds, eds. Kelley’s Textbook of Rheumatology. 9th edn. Amsterdam, Elsevier, 2013, 1463–80. 2. Restrepo CS, Ocazionez D, Suri R, Vargas D. Aortitis: imaging spectrum of the infectious and inflammatory conditions of the aorta. Curr Rheumatol Radiographics. 2011; 31:435–51. 3. Weyand CM, Goronzy JJN. Medium- and large-vessel vasculitis. New Engl J Med 2003; 349:160–9. 4. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum 2013; 65:1–11. 5. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Takayasu arteritis and giant cell arteritis: a spectrum within the same disease? Medicine 2009; 88:221–6. 6. Takamura C, Ohhigashi H, Ebana Y, Isobe M. New HLA risk allele in Japanese patients with Takayasu arteritis. Circ J 2012; 76:1697–702.
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typical GCA symptoms (e.g. headache, induration in the temporal arteries and polymyalgia), lower systolic blood pressure in right arm (suggesting stenotic lesions in the brachiocephalic artery) and HLA B39 and B52 (associated with TAK in Japanese patients). Although TAK and GCA are occasionally considered to be the same disease, they exhibit different susceptible vessels and immunogenetic backgrounds. Therefore, if clinical presentation does not match the symptoms of GCA, TAK should be considered and further testing should be performed (e.g. HLA typing) even in elderly men.
CASE REPORT
Takayasu arteritis and ischaemic stroke Y. Maeda1, H. Taguchi2, T. Kudo2 and Y. Okano2 From the 1Department of Internal Medicine and 2Center for Arthritis and Rheumatic Disease, Kawasaki Municipal Kawasaki Hospital, Japan Address correspondence to Y. Maeda, 12-1, Shinkawadori, Kawasaki-ku, Kawasaki, 210-0013 Japan. email: [email protected]
Learning point for clinicians
Discussion Case report A 79-year-old Japanese man visited our hospital with an 8 week history of low-grade fever and high-serum C-reactive protein (CRP) levels. He also had a history of hypertension and hyperlipidemia, although his familial history was unremarkable. His laboratory data revealed a white blood cell count of 8090/ml and CRP levels of 13.2 mg/dl. No antinuclear antibodies, antineutrophil cytoplasmic antibodies or syphilis was detected. Contrastenhanced computed tomography (CECT) revealed aortic wall thickening from the ascending section to the common iliac artery (Figure 1a), which indicated large vessel vasculitis (LVV). Two weeks later, he developed right hemiparesis and dysarthria and was transferred to our emergency room. He did not complain of headache, blurred vision, jaw claudication or myalgia during the course. On admission, different systolic blood pressures were observed in each arm (right: 135 mmHg, left: 160 mmHg), and bruit was not audible over the carotid artery. He had no induration or tenderness of his temporal arteries. Diffusion-weighted magnetic resonance imaging (MRI) revealed high-signal areas at the left corona radiata (Figure 1b). We diagnosed the patient with ischemic stroke complicated with LVV, and began oral prednisone at 0.5 mg/kg/d. Although the patient’s serum CRP levels rapidly decreased, he developed ischemic colitis with diffuse colonic ulcers 4 days
We report a patient who developed ischemic stroke as the initial manifestation of vessel stenosis related to LVV. Takayasu arteritis (TAK) and giant cell arteritis (GCA) are the two main causes of LVV, although they are difficult to differentiate, given their many similarities. In recent years, CECT, MRI and fluorodeoxy glucose-positron emission tomography have been proven to detect large vessel inflammation in both TAK and GCA.1,2 However, the histopathology of the arterial lesions in these diseases may be indistinguishable. Therefore, some physicians have argued that these two disorders represent a spectrum within the same disease.3 Nevertheless, it is important to differentiate between TAK and GCA, as their complications and managements can be different. To differentiate between these two diseases, age and sex are important considerations, as most TAK patients are young women who are 50-year-olds.4 In addition, the distribution of lesions can differentiate TAK from GCA, as almost all parts of aorta can be invaded in TKA, while the external carotid artery and its branches are more susceptible in GCA.5 Furthermore, HLA typing is helpful in distinguishing TAK from GCA. The HLA haplotyping of Japanese patients with TAK has revealed a higher frequency for HLA B39, B52 and DR2, compared with a high frequency of HLA DR3, DR4, DR5 and DRB1 in GCA.6 Our case of TAK was unusual, as the patient was an older man, although we diagnosed TAK based on the absence of
C The Author 2015. Published by Oxford University Press on behalf of the Association of Physicians. V
All rights reserved. For Permissions, please email: [email protected]
1
Downloaded from by guest on November 13, 2015
Takayasu arteritis (TAK) and giant cell arteritis (GCA) have similar features, and some physicians believe that they exist as a spectrum, and are only differentiated by onset age. However, if GCA can be excluded, TAK is a possible differential diagnosis for large vessel vasculitis in men who are >50-year-olds.
later, which was confirmed by colonoscopy. Given the insufficient control of the vasculitis, we escalated the prednisone dose to 1 mg/kg/d. After prednisone escalation, no haematochezia or neurological symptoms recurred. After rehabilitation and tapering the prednisone for 6 weeks, the patient was discharged while receiving 40 mg/day of prednisone. Human leukocyte antigen (HLA) testing revealed HLA-A2, A24, B39, B52, DR2 (DR15) and DR6 (DR14).
2
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QJM: An International Journal of Medicine, 2015, Vol. 0, No. 0
Figure 1. (a). Inflammatory thickening of aortic wall from ascending part to common iliac artery was seen in CECT. (b). High-signal areas at left corona radiata in the diffusion-weighted MRI.
Conflict of interest: None declared.
References 1. Hellmann DB. Giant cell arteritis, polymyalgia rheumatica, and Takayasu’s arteritis. In: GS Firestein, RC Budd, SE
Gabrieleds, eds. Kelley’s Textbook of Rheumatology. 9th edn. Amsterdam, Elsevier, 2013, 1463–80. 2. Restrepo CS, Ocazionez D, Suri R, Vargas D. Aortitis: imaging spectrum of the infectious and inflammatory conditions of the aorta. Curr Rheumatol Radiographics. 2011; 31:435–51. 3. Weyand CM, Goronzy JJN. Medium- and large-vessel vasculitis. New Engl J Med 2003; 349:160–9. 4. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum 2013; 65:1–11. 5. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Takayasu arteritis and giant cell arteritis: a spectrum within the same disease? Medicine 2009; 88:221–6. 6. Takamura C, Ohhigashi H, Ebana Y, Isobe M. New HLA risk allele in Japanese patients with Takayasu arteritis. Circ J 2012; 76:1697–702.
Downloaded from by guest on November 13, 2015
typical GCA symptoms (e.g. headache, induration in the temporal arteries and polymyalgia), lower systolic blood pressure in right arm (suggesting stenotic lesions in the brachiocephalic artery) and HLA B39 and B52 (associated with TAK in Japanese patients). Although TAK and GCA are occasionally considered to be the same disease, they exhibit different susceptible vessels and immunogenetic backgrounds. Therefore, if clinical presentation does not match the symptoms of GCA, TAK should be considered and further testing should be performed (e.g. HLA typing) even in elderly men.
