A Stab in the Dark: A Case Report of an Atypical Presentation of Giant Cell Arteritis (GCA)

Abstract

Background: Giant cell arteritis/temporal arteritis (GCA) is an inflammatory condition that affects large to medium vessels such as the aorta and its primary branches. Patients classically present with fatigue, fever, headache, jaw claudication and in severe cases, may suffer either transient (amaurosis fugax) or permanent visual loss. The reference standard for diagnosis is the temporal artery biopsy (TAB) and the mainstay of treatment is with immunosuppression. Our patient JG, presented with a range of non-specific symptoms that mimicked generalised sepsis, but was ultimately diagnosed with GCA through effective, methodical multi-disciplinary team (MDT) work. Clinical case: JG, an 81 year old gentleman, presented acutely with a 3-4 weeks history of fatigue, lethargy, pyrexia and a marked inflammatory response suggestive of a sepsis but without a clear primary source or clinical features of vasculitis. His inflammatory markers were markedly raised although his erythrocyte sedimentation rate (ESR) was not elevated. He was initially treated for sepsis of unknown origin however, body imaging after admission suggested a possible infection around a previous aortic graft site. This was refuted in subsequent 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET/CT) scanning. Microbiological, parasitic, as well as autoimmune assays were unremarkable. He underwent a TAB which was diagnostic for GCA and as a result, was started on oral corticosteroids with immediate symptom relief. He was discharged and followed up on an outpatient basis. Conclusions: This case highlights how a vasculitis can present with a range of non-specific symptoms that may resemble a fever of unknown origin (FUO)/sepsis that can lead to a delay in making the correct diagnosis. It also highlights the importance of considering a diagnosis of vasculitis in patients who present with a FUO where there is no clear focus of infection. Delays in diagnosis and management of these conditions can potentially lead to significant irreversible morbidity.

Giant Cell Arteritis With Sepsis-Like Symptoms in an Elderly Man: A Case Report

“…Next, considering hospital-acquired infections, such as Pseudomonas aeruginosa , the patient was changed to tazobactam/piperacillin, but no response was observed. Blood culture results were also negative, and the possibility of infection was ruled out [ 7 ]. The condition was progressive, and contrast-enhanced CT findings showed thickening of the wall of the aortic arch, so treatment with prednisolone was started, considering the possibility of giant cell arteritis.…”

Atypical giant cell arteritis presentations diagnosed with FDG-18 whole body PET imaging

“…PMR patients with atypical features, which include inflammatory-type lower back pain, predominant pelvic girdle discomfort or lower limb pain, severe systemic symptoms and poor response to moderately dosed steroids, were more likely to have concurrent LVV and therefore should be investigated as such 39. One study recommended screening for LVV in all elderly patients with non-specific symptoms and biochemical evidence of systemic inflammation (elevated CRP, ESR or platelets) 40. An extension of this concept was also depicted by Chan et al in a retrospective audit, which suggested that the combination of high CRP and thrombocytosis demonstrated high sensitivity and specificity for GCA and possibly a better diagnostic yield for the condition than isolated elevations in CRP or ESR (figure 3).…”