136 Giant cell arteritis—Raja et al

Giant cell arteritis presenting with aortic aneurysm,normal erythrocyte sedimentation rate, and normal C-reactive proteinMohan K. Raja,*MSc; Alain A. Proulx,*† MD, MSc; Larry H. Allen,*† MD

ABSTRACT • RÉSUMÉ

Case report: We report on an extremely rare case of giant cell arteritis (GCA) presenting without elevatederythrocyte sedimentation rate or C-reactive protein level, with aortic root involvement, and in the absenceof typical clinical findings.

Comments: The clinical signs and symptoms as well as the laboratory investigations involved in the diagnosisof GCA are discussed.

Observation : Nous signalons un cas extrêmement rare d’artérite giganto-cellulaire (AGC) se présentantsans vitesse élevée de sédimentation globulaire ni de niveau élevé de protéine C-réactive, mais avecimplication de la racine de l’aorte et en l’absence de résultats cliniques typiques.

Commentaires : Les signes et symptômes cliniques de même que les investigations de laboratoireconcernés dans le diagnostic de l’AGC font l’objet de la discussion.

Giant cell arteritis (GCA) is a systemic granuloma-tous vasculitis that affects large- and medium-sized

arteries of the head and neck. Clinical features mayinclude headache, jaw claudication, fever, symptoms ofpolymyalgia rheumatica (PMR), visual disturbance, andraised serologic inflammatory markers such as the ery-throcyte sedimentation rate (ESR) or C-reactive protein(CRP). Temporal artery biopsy is widely accepted as thegold standard for diagnosis. In the absence of typicalsigns and symptoms, diagnosis becomes more difficult,thus increasing the risk of severe complications. Wereport an atypical presentation of GCA with aortic rootinvolvement, but in the absence of typical symptoms ofGCA or a diagnosis of PMR, and without raised ESRand CRP.

CASE REPORT

A 73-year-old man presented to hospital with recentepisodes of dizziness and syncope. Echocardiography

revealed dilatation of the proximal aortic root and anascending aortic aneurysm. Surgery was performed inwhich the aortic aneurysm was resected and repairedwith a synthetic graft. The surgical pathology reportrevealed pathological changes highly suggestive of giantcell aortitis (Fig. 1).

Upon questioning, the patient denied any symptomssuggestive of GCA. Since his surgery, he had com-plained of intermittent numbness of the left foot;however, that numbness had been decreasing over time.He also described a vague episode of blurred vision onthe right side that resolved within 24 hours. The ESRand CRP were normal at 14 mm/h and less than 4mg/L, respectively. Results of tests for antinuclear anti-body, antiphospholipid antibody, and lupus anticoagu-lant were all negative.

Results of a temporal artery biopsy confirmed thediagnosis of active GCA on the right side (Fig. 2). Visualacuity was 20/30 in the right eye and 20/40 in the left.Neuro-ophthalmic examination showed no abnormali-ties. Results of examination of the anterior and posteriorsegments of the eyes were unremarkable as well.

COMMENTS

In the absence of characteristic signs and symptoms,diagnosing GCA can pose a challenge. The present casedemonstrates that the condition can manifest in manyways, even with a normal ESR, CRP, and with aorticinvolvement. GCA affects the aorta and major branchesin only 15–17% of cases, making it an unusual presen-tation for the condition.1 Traditionally, a commonscreening test for GCA has been the ESR. Although an

From *the Schulich School of Medicine, and †the Ivey Eye Institute,Department of Ophthalmology, University of Western Ontario, London, Ont.

Originally received Feb. 23, 2006. Revised May 28, 2006Accepted for publication June 2, 2006

Correspondence to: Larry H. Allen, Department of Ophthalmology,University of Western Ontario, Ivey Eye Institute, St. Joseph’s HealthCare, 268 Grosvenor St., London ON N6A 4V2; fax 519-646-6248;larry.allen@lhsc.on.ca

This article has been peer-reviewed. Cet article a été évalué par les pairs.

Can J Ophthalmol 2007;42:136–7doi:10.3129/can j ophthalmol.06-102

Giant cell arteritis—Raja et al

CAN J OPHTHALMOL—VOL. 42, NO. 1, 2007 137

elevated ESR is widely regarded as a hallmark of activeGCA, a significant number of cases have been reportedwith an ESR within normal limits. The incidence of lowESR with biopsy-proven GCA has been described in8%–22.5% of patients.2,3 Alternatively, an elevated CRPmay be more sensitive in detecting GCA, with one studyfinding the test 100% sensitive for the population exam-ined.4 When used in conjunction with one another, ESRand CRP usually provide a powerful diagnostic tool toaid in the diagnosis of GCA. Nonetheless, the literature

does cite rare exceptions to this general rule. Poole et alreported a case of GCA with ocular involvement in theabsence of an elevated ESR and CRP.5 Similarly, Hayrehet al reported a high incidence of ischemic optic neu-ropathy with only a slightly raised ESR and CRP inoccult GCA.6

In the present case, the patient exhibited none of theocular or neurologic signs typical of GCA. The lack ofelevated serological markers not only makes the initialdiagnosis difficult, but also creates a dilemma in themedical management of the condition. Treatment ofGCA typically involves high-dose corticosteroids overan extended period of time, with therapy being guidedboth by the clinical response as well as by a stabilizationof the ESR. Steroid therapy can be associated with sig-nificant morbidity, however. In the context of both anormal initial ESR and CRP, maintenance and taperingof steroids must be done empirically with very closefollow-up to monitor for the appearance of signs of thedisease.

Since first being diagnosed, the patient has been mon-itored closely in conjunction with the rheumatologyservice. Steroid and methotrexate therapy were initiatedat standard doses and have both been gradually taperedover the course of 36 months without serious sequelae.Repeat Doppler imaging of temporal arteries have beennegative. Given the high risk of ocular or neurologicinvolvement with GCA, long-term follow-up of uniquecases such as this is essential.2

REFERENCES

1. Evans J, Hunder GG. The implications of recognizing large-vessel involvement in elderly patients with giant cell arteritis.Curr Opin Rheumatol 1997;9:37–40.

2. Ghanchi FD, Dutton GN. Current concepts in giant cell(temporal) arteritis. Surv Ophthalmol 1997;42:99–123.

3. Salvarani C, Hunder GG. Giant cell arteritis with low ery-throcyte sedimentation rate: frequency of occurrence in a pop-ulation-based study. Arthritis Rheum 2001;45:140–5.

4. Hayreh SS, Podhajsky PA, Raman R, et al. Giant cell arteritis:validity and reliability of various diagnostic criteria. Am JOphthalmol 1997;123:285–96.

5. Poole TR, Graham EM, Lucas SB. Giant cell arteritis with anormal ESR and CRP. Eye 2003;17:92–3.

6. Hayreh SS, Podhajsky PA, Zimmerman B. Occult giant cellarteritis: ocular manifestations. Am J Ophthalmol 1998;125:521–6.

Key words: giant cell arteritis, ESR, C-reactive protein, temporalartery, aortic root

Fig.1—Representative section through the segmentof excised aorta. Sample, stained with Massonelastic trichrome, reveals the extensive disruptionof elastic fibres (original magnification ×40).

Fig. 2—Representative cross-section through theexcised segment of temporal artery, showingcharacteristic changes of giant cell arteritis. Giantcells are seen within the tunica media and there isextensive disruption of the internal elastic lamina.Intimal hyperplasia and fibrosis are also seen,compromising the vessel’s lumen size (Movat’spentachrome stain; original magnification ×100).