PO:37:268 | Antineutrophil cytoplasmic antibodies-associated vasculitis mimicking giant cell arteritis: a diagnostic dilemma

Web of Confusion

Is Temporal Artery Biopsy Prudent?

Giant Cell Arteritis: Read the Fine Print!

The Early History of Giant Cell Arteritis and Polymyalgia Rheumatica: First Descriptions to 1970

A retrospective chart review garnered patient demographics, symptoms, comorbidities, and steroid treatment duration in patients undergoing TAB at a single center. Steroid treatment was compared between TAB+ and TAB - patients. One hundred seven patients undergoing TAB were included. Patients were predominantly women (70.1%) with a median age of 74 years (46 -91). Of 107 TAB results, 74 (69.2%) were negative, 23 (21.5%) were positive, and 10 (9.3%) were found to be indeterminate. In TAB+ patients, the mean erythrocyte sedimentation rate was not significantly different than TAB - patients (60.2 versus 43.7, P = 0.45), nor was the median C-reactive protein (38.8 versus 18.1, P = 0.17). Regarding steroid use, both TAB+ and TAB - patients had a similarly high rate of prebiopsy steroid initiation (82.6% versus 70.3%, P = 0.32). More TAB+ patients remained on steroids at 6 weeks (95.0% versus 57.4%, P = 0.004), 6 months (95% versus 37.7%, P < 0.001), 1 year (65.0% versus 31.1%, P = 0.024), and 18 months (50.0% versus 19.7%, P = 0.045). By 2 years, the difference no longer met significance (35.0% versus 14.8%, P = 0.12). P = 0.12). TAB positivity does seem to influence maintenance of steroids up to 18 months after biopsy.

Unexpected Visual Loss in Appropriately Treated Giant Cell Arteritis

76-Year-Old Man With New Bilateral Shoulder Pain

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.

Background:Polymyalgia Rheumatica (PMR) is an inflammatory condition affecting individuals aged 50 years and over, predominantly women. PMR sometimes coexists with Giant Cell Arteritis (GCA), the most commonly occurring vasculitis in...

Treatment of Acute Visual Loss in Giant Cell Arteritis

AB0439 Contribution of anti-ferritin antibodies to the diagnosis of giant cell arteritis

Giant cell arteritis and its mimics: A comparison of three patient cohorts

OP0066 METABOLIC PROFILE AND COMORBIDITIES IN GIANT CELL ARTERITIS AND POLYMYALGIA RHEUMATICA PATIENTS BEFORE AND AFTER TREATMENT

Editor, G iant cell arteritis (GCA) is a neuro-ophthalmological emergency given the risk of permanent, severe visual loss if left untreated (Hayreh et al. 1998a). We present a rare case of biopsy-proven GCA with normal inflammatory markers at first presentation. A 74-year-old woman was referred to us with a 2 month history of rightsided temporal headaches. She had no relevant past medical history but her sister suffered from polymyalgia rheumatica. The headaches occurred almost daily and often lasted several hours. She reported no visual symptoms, jaw claudication or other classical symptoms of polymyalgia rheumatica or GCA. Her Snellen visual acuity was 6 ⁄ 6 in both eyes and ocular examination was entirely normal. Her temporal arteries on both sides were pulsatile, non-tender and normal in appearance. Inflammatory markers were requested to rule out GCA; these were normal, with an erythrocyte sedimentation rate (ESR) of 30 mm ⁄hr and a C-reactive protein (CRP) <5 mg ⁄L. The patient was therefore discharged from clinic. She re-presented 6 months later, still complaining of right-sided temporal headaches, but over the past 3 months she had also become aware of scalp tenderness, transient episodes of jaw ache and feeling tired. She again denied any visual disturbance, and ocular examination was unremarkable. However, her right temporal artery was now thickened, although non-tender and still pulsatile. Inflammatory markers were raised with an ESR of 96 mm ⁄hr and a CRP of 18 mg ⁄L. Other routine bloods, including a full blood count, were normal. She was started on 60 mg of oral prednisolone, and a right temporal artery biopsy (TAB) showed unequivocal histological features of GCA. Her symptoms resolved completely on steroids and, a year later, there has been no disease recurrence as her treatment has been tapered down. This case illustrates an atypical, insidious presentation of GCA. The recent onset of unilateral temporal headaches, its persistence and the subsequent development of other clinical features indicate an evolution of the underlying pathological process. The normal inflammatory markers and absence of other systemic and ocular symptoms could be explained by localized involvement of her temporal arteries. Temporal headache is the most common clinical symptom of GCA and is reported by up to 90% of patients (Rahman & Rahman 2005). In Jonasson’s series of 124 biopsy-proven cases, a higher ESR and more generalized systemic features were also associated with longer disease duration (Jonasson et al. 1979). Although an ESR is frequently requested by clinicians in order to rule out this condition, a normal level has been reported in 5–30% of patients with GCA (Rahman & Rahman 2005). In Hayreh’s series of 363 patients who had TAB for suspected GCA, CRP was more sensitive (100%) than ESR (92%) in detecting GCA, and a combination of ESR and CRP gave the best specificity (97%) (Hayreh et al. 1997). It is also well established that patients with occult GCA can have significantly lower inflammatory markers compared to those with the more classical systemic features (Hayreh et al. 1998b). Recently, Poole and colleagues presented a unique case of occult GCA with normal ESR and CRP (Poole et al. 2003). Their patient had transient episodes of monocular visual loss and evidence of ocular ischaemia with ophthalmoplegia, cotton wool spots and a markedly delayed choroidal perfusion on fluorescein angiography. These findings raised the possibility of GCA, which was confirmed with a positive TAB. As in our case, this highlights the importance of TAB as the gold-standard diagnostic test in GCA since inflammatory markers can be normal if the disease process is at an early stage, there is only localized arteritis or there is an inability to mount an acute-phase serologic response. GCA is a heterogeneous disease with a wide range of clinical manifestations. It remains a challenging diagnosis, requiring a high index of clinical suspicion and a low threshold for TAB.

ObjectiveIt is urgent to diagnose giant cell arteritis (GCA) as quickly as possible to prevent irreversible blindness. The traditional gold standard for diagnosing GCA is temporal artery biopsy (TAB). However, TAB lacks diagnostic performance and carries out risks of surgical intervention. The noninvasive color Doppler ultrasound (CDU) seems to be a promising alternative. This study is designed to assess the diagnostic value of CDU in daily clinical practice.MethodsIn this prospective cohort study, patients with a clinical suspicion of active GCA were included and underwent a CDU of the temporal arteries. If deemed necessary by the referrer, a TAB and/or 18F‐fluorodeoxyglucose positron emission tomography with computed tomography was performed. The retrospective clinical diagnosis was determined 1 year after inclusion by two physicians experienced in the field of vasculitis.Results242 patients were included and GCA was diagnosed in 73 (30%) patients by the defined retrospective clinical diagnosis. Compared with the retrospective diagnosis, CDU has a sensitivity of 60% (48–72), specificity of 94% (89–97), positive predictive value (PPV) of 81% (70–89), negative predictive value (NPV) of 85% (80–88), and an accuracy of 84% (78–88). A total of 84 (35%) patients underwent TAB. TAB has a sensitivity of 66% (51–79), specificity of 100% (90–100), PPV of 100% (100), NPV of 67% (58–75), and an accuracy of 80% (70–88).ConclusionThis study shows comparable diagnostic performance for CDU and TAB and even better CDU results with a bilateral halo present. Considering the advantages of the noninvasive CDU, it is the diagnostic tool of choice.