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To determine if the pattern of macrophage activation reflects differences in the pathogenesis and clinical presentation of giant cell arteritis and primary angiitis of the central nervous system, specimens of 10 patients with giant cell arteritis and five with primary angiitis of the central nervous system were immunohistochemically studied and the expression of the macrophage activation markers 27E10, MRP14, MRP8 and 25F9 was determined in the vasculitic infiltrates. Thus, a partly different expression pattern of macrophage activation markers in giant cell arteritis and primary angiitis of the central nervous system was observed. The group comparison revealed that giant cell arteritis cases had significantly higher numbers of acute activated MRP14-positive macrophages, whereas primary angiitis of the central nervous system is characterized by a tendency toward more MRP8-positive intermediate/late activated macrophages. Furthermore, in giant cell arteritis comparably fewer CD8-positive lymphocytes were observed. These observations suggest, that despite their histopathological similarities, giant cell arteritis and primary angiitis of the central nervous system appear to represent either distinct entities within the spectrum of granulomatous vasculitides or different stages of similar disease processes. Their discrete clinical presentation is reflected by different activation patterns of macrophages, which may characterize giant cell arteritis as a more acute process and primary angiitis of the central nervous system as a more advanced inflammatory process.

To determine the frequency of aneurysm and dissection of the aorta in patients with giant cell arteritis and to assess the effects of these events on these patients. Population-based cohort study. A multispecialty and a primary care clinic in southern Minnesota. 96 residents of Olmsted County, Minnesota, who developed giant cell arteritis between 1950 and 1985. The presence of aortic aneurysm, dissection, or both was confirmed using computed tomography, ultrasonography, angiography, or autopsy. 11 of the 96 patients were found to have thoracic aortic aneurysms. In 2 of these patients, the aneurysms were detected when giant cell arteritis was diagnosed. In the remaining 9 patients, the aneurysms occurred a median of 5.8 years after giant cell arteritis was diagnosed. Six of the 11 died suddenly of acute thoracic aortic dissection. Five patients who did not have thoracic aortic aneurysms developed isolated abdominal aortic aneurysms a median of 2.5 years after giant cell arteritis was diagnosed. The incidence of thoracic aortic aneurysm in patients with giant cell arteritis was 999 per 100,000 person-years; the incidence of abdominal aortic aneurysm in these patients was 555 per 100,000 person-years. Compared with all persons of the same age and sex living in Olmsted County, patients with giant cell arteritis were 17.3 times (95% Cl, 7.9 to 33.0) more likely to develop thoracic aortic aneurysm and 2.4 times (Cl, 0.8 to 5.5) more likely to develop isolated abdominal aortic aneurysm. Giant cell arteritis is associated with a markedly increased risk for the development of aortic aneurysm, which is often a late complication and may cause death.

British Journal of Hospital MedicineEarly view Case ReportAortic dissection secondary to giant cell arteritisConnor Cotton, Sarah Chatharoo, Pankaj ChaturvediConnor CottonCorrespondence to: Connor Cotton; E-mail Address: [email protected]Department of Diabetes and Endocrinology, Doncaster and Bassetlaw Teaching Hospitals NHS Trust, Worksop, UKSearch for more papers by this author, Sarah ChatharooDepartment of Diabetes and Endocrinology, Doncaster and Bassetlaw Teaching Hospitals NHS Trust, Worksop, UKSearch for more papers by this author, Pankaj ChaturvediDepartment of Diabetes and Endocrinology, Doncaster and Bassetlaw Teaching Hospitals NHS Trust, Worksop, UKSearch for more papers by this authorConnor Cotton; Sarah Chatharoo; Pankaj ChaturvediPublished Online:17 Apr 2023https://doi.org/10.12968/hmed.2022.0529AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail View article References Agard C, Ponge T, Fradet G et al.. Giant cell arteritis presenting with aortic dissection: two cases and review of the literature. Scand J Rheumatol. 2006;35(3):233–236. https://doi.org/10.1080/03009740500395252 Crossref, Medline, Google Scholarde Boysson H, Espitia O, Samson M et al.. Giant cell arteritis-related aortic dissection: a multicenter retrospective study. Semin Arthritis Rheum. 2021;51(2):430–435. https://doi.org/10.1016/j.semarthrit.2021.03.001 Crossref, Medline, Google ScholarEvans JM, O'Fallon WM, Hunder GG. Increased incidence of aortic aneurysm and dissection in giant cell (temporal) arteritis: a population-based study. Ann Intern Med. 1995;122(7):502. https://doi.org/10.7326/0003-4819-122-7-199504010-00004 Crossref, Medline, Google ScholarHiratzka LF, Bakris GL, Beckman JA et al.. Faculty opinions recommendation of 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/sir/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 2010;121(13): e266–369. https://doi.org/10.1161/CIR.0b013e3181d4739e Medline, Google ScholarKermani TA, Warrington KJ, Crowson CS et al.. Large-vessel involvement in giant cell arteritis: a population-based cohort study of the incidence-trends and prognosis. Ann Rheum Dis. 2013;72(12):1989–1994. https://doi.org/10.1136/annrheumdis-2012-202408 Crossref, Medline, Google ScholarMackie SL, Dejaco C, Appenzeller C et al.. British society for rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology. 2020;59(3):e01–e23. https://doi.org/10.1093/rheumatology/kez672 Crossref, Medline, Google ScholarNayar AK, Casciello M, Slim JN, Slim AM. Fatal aortic dissection in a patient with giant cell arteritis: a case report and review of the literature. Case Rep Vasc Med. 2013;2013:1–4. https://doi.org/10.1155/2013/590721 Google Scholar FiguresReferencesRelatedDetails Ahead of PrintISSN (print): 1750-8460ISSN (online): 1759-7390 Metrics History Published online 17 April 2023 Information© MA Healthcare LimitedAcknowledgementsThe authors would like to thank Sarah Gardner, Library and Information Service, Doncaster and Bassetlaw Teaching Hospitals NHS Foundation Trust Knowledge, Doncaster, for her help with the literature search.PDF download

To elucidate epidemiologic and clinical manifestations of Japanese patients with giant cell arteritis (GCA), the first nationwide survey for GCA was conducted in 1998 in Japan. The first questionnaire on GCA for patients treated in 1997 was sent to 10,717 medical departments in Japan. A total of 177 patients were reported. Among the 177 patients, 66 GCA patients with detailed clinical and epidemiologic features on second survey were analyzed. Prevalence in patients 50 years of age and older in 1997 was 1.47 per 100,000 population in Japan. The average age at onset was 71.5 years old. The male:female ratio was 1:1.7. The association with permanent and complete visual loss (6.5%), jaw claudication (15.2%), and polymyalgia rheumatica (PMR) (30.3%) were low in frequency compared with those reported from other countries. All patients were treated with corticosteroids. Only 3 (4.5%) patients were reported as deceased due to other causes. The prevalence of GCA in Japan was revealed to be extremely low compared with other countries. Clinical findings of permanent and complete visual loss, jaw claudication, and PMR were infrequent among Japanese patients with GCA.

Visual Morbidity in Giant Cell Arteritis: Clinical Characteristics and Prognosis for Vision

AB0449 Isolated aortitis as an atypical presentation of giant cell arteritis

Comment on: Deaths caused by bedrails.Parker K, Miles SH. J Am Geriatr Soc. 1997; 45(7):797-802. Language: en

To evaluate the positivity rate of temporal artery biopsies (TAB) performed in suspects of giant cell arteritis (GCA) and to study the epidemiological and clinical factors associated to the biopsy result. A retrospective, multicenter, case-control study was performed, including three hundred and thirty-five patients who underwent TAB for a suspicion of GCA from 2001 to 2010. Clinical, epidemiological and pathology data were recovered from the patients' clinical records. Histologic diagnosis of GCA was made when active inflammation or giant cells were found in the arterial wall. Eighty-one biopsies (24.2%) were considered positive for GCA. Clinical factors independently associated to TAB result in a logistic regression analysis were temporal cutaneous hyperalgesia (OR = 10.8; p < 0.001), jaw claudication (OR = 4.6; p = 0.001), recent-onset headache (OR = 4.4; p = 0.001), decreased temporal pulse (OR = 2.8; p = 0.02), pain and stiffness in neck and shoulders (OR = 2.3; p = 0.05), unintentional weight loss (OR = 1.33; p = 0.003) and age (OR = 1.085; p = 0.004). Other factors such as length of the surgical specimen (OR = 1.079; p = 0.028) and erythrocyte sedimentation rate (OR = 1.042; p < 0.001) were also statistically significant. The model was accurate (C-index = 0.921), reliable (pHosmer-Lemeshow = 0.733) and consistent in the bootstrap sensitivity analysis. No significant association was detected between TAB result and number of days of previous systemic corticosteroid treatment (p = 0.146). However, an association was observed between TAB result and the total accumulated dose of previous systemic corticotherapy (p = 0.043). Exhaustive anamnesis and clinical examination remain of paramount importance in the diagnosis of GCA. To improve the yield of TAB, it should be performed specially in older patients with GCA-compatible clinic. TAB could be avoided in patients with an isolated elevation of acute phase reactants, without GCA-compatible clinic.

Dépistage de l'atteinte aortique abdominale par échographie-doppler au diagnostic de maladie de Horton. Étude prospective de 30 patients