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J Gen Intern Med. 2009 July; 24(7): 892.
Published online 2009 May 12. doi:  10.1007/s11606-009-1005-x
PMCID: PMC2695515

FUO and Biopsy-Negative GCA

To the Editor:—Fever of unknown origin in patients with giant cell arteritis (GCA) who have neither cephalic symptoms nor histological evidence of temporal arteritis(TA)1 has, as it corollary, potentially life-threatening aortitis in patients with isolated polymyalgia rheumatica (PMR).2,3 In one study, in spite of isolated PMR, the latter characterised by absence of cephalic stigmata as well as by non-diagnostic temporal artery (TA) biopsy, aortitis was, nevertheless, an associated feature, documented by 18-fluorodeoxyglucose positron emission tomography.2 In other cases only absence of cephalic signs sufficed to characterise isolated PMR, and, in two such instances, aortitis was documented by nuclear magnetic resonance imaging.3 Diagnostic difficulty is compounded when there is neither cephalic nor PMR symptomatology, as in a 75-year-old woman in whom the only feature of GCA was progressive enlargement of an ascending thoracic aortic aneurysm, necessitating surgical treatment. Histology of the surgical specimen showed GCA.4 Presentation of giant cell arteritis solely with aortitis-related symptoms also characterised a 76-year-old patient with ascending aortic aneurysm in whom histology of the aneurysmal wall, again, revealed giant cell arteritis.5 Diagnostic difficulties notwithstanding, it is important to identify PMR-related aortitis, because patients with isolated PMR have significantly (p < 0.05) increased risk of death from vascular disease, including ruptured aortic aneurysm.6


1. Schafer VS, Warrington KJ, Williamson EE, Kermani TA. Delayed diagnosis of biopsy-negative giant cell arteritis presenting as fever of unknown origin. J Gen Intern Med. 2009;24:532–536. doi: 10.1007/s11606-009-0925-9. [PMC free article] [PubMed] [Cross Ref]
2. Blockmans D, Ceuninck L, Vanderschueren D, et al. Repetitive 18-fluorodeoxyglucose positron emission tomography in isolated polymyalgia rheumatica: a prospective study in 35 patients. Rheumatology. 2007;46:672–7. doi: 10.1093/rheumatology/kel376. [PubMed] [Cross Ref]
3. Narvaez J, Narvaez JA, Nolla JM, et al. Giant cell arteritis and polymyalgia rheumatica: usefulness of vascular magnetic resonance imaging studies in the diagnosis of aortitis. Rheumatology. 2005;44:479–83. doi: 10.1093/rheumatology/keh513. [PubMed] [Cross Ref]
4. Nuenninghoff DM, Warrington KJ, Matteson EL. Concomitant giant cell aortitis, thoracic aortic aneurysm, and aortic arch syndrome:occurrence in a patient and significance. Arthritis Rheum. 2003;49:858–61. doi: 10.1002/art.11453. [PubMed] [Cross Ref]
5. Uchida T, Andou H, Yatsune T, et al. Thoracic aortic aneurysm due to giant cell arteritis without any specific symptom. Kyobu Geka. 2009;62:324–7. [PubMed]
6. Schaufelberger C, Bengtsson B-A, Andersson R. Epidemiology and mortality in 220 patients with polymyalgia rhumatica. Brit J Rheumatol. 1995;34:261–4. doi: 10.1093/rheumatology/34.3.261. [PubMed] [Cross Ref]

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