Symptoms Of Giant Cell Arteritis Research Articles

Low incidence of late-onset giant cell arteritis during the first year in patients with polymyalgia rheumatica-a repeated imaging study.

The objective was to investigate the incidence of late-onset giant cell arteritis (GCA) within the first year in patients diagnosed with polymyalgia rheumatica (PMR). In this prospective study, treatment-naïve individuals with a new clinical diagnosis of PMR and without GCA symptoms underwent baseline assessments, including vascular ultrasonography and 2-[18F]fluoro-2-deoxy-D-glucose positron emission tomography computed tomography (FDG-PET/CT). To prevent biased inclusion, rapid referral clinics were established for all patients suspected of PMR. Additionally, the patients underwent GCA monitoring during clinical visits at weeks 8 and 10, which involved vascular ultrasonography and FDG-PET/CT scans. After one year, a follow-up visit was performed to confirm the PMR diagnosis and perform vascular ultrasonography. A final PMR diagnosis was assigned to 62 patients, excluding 2 patients with concurrent subclinical GCA and PMR at baseline, corresponding to a baseline prevalence of subclinical GCA of 3%. During the one-year follow-up, two PMR patients developed late-onset GCA corresponding to an incidence rate of 32 per 1000 person-years. One patient developed GCA 14 weeks after the PMR diagnosis, exhibiting cranial symptoms and positive vascular ultrasonography. The other patient presented with subclinical large vessel GCA at the one-year visit detected with vascular ultrasonography and confirmed by FDG-PET/CT. This study is the first to demonstrate a low incidence rate of late-onset GCA in PMR patients within the first year, employing repeated imaging to exclude GCA at baseline and diagnose GCA during follow-up. Additionally, it provides evidence of a low prevalence of subclinical GCA across the entire PMR population. ClinicalTrials.Gov, NCT04519580.

Impact of Exposure to Environmental Particulate Matter on the Onset of Giant Cell Arteritis.

The aim of this study was to investigate the association between exposure to particulate matter with an aerodynamic diameter ≤10 μm (PM10) and the development of giant cell arteritis (GCA) and its ischemic complications. This was case-crossover study on consecutive patients who received a diagnosis of GCA in three hospitals in northern Italy between 2013 and 2021. The PM10 hourly and daily average concentrations, collected in the Italian monitoring network and archived by Istituto Superiore per la Protezione e la Ricerca Ambientale, were determined using European reference. We used a Bayesian hierarchical model to determine patients' daily exposures to them. We employed conditional logistic regression to estimate the effect of exposure on GCA symptoms onset or ischemic complications. We included 232 patients. A positive association was observed between exposure to PM10 and GCA risk, with an incremental odd of 27.1% (95% confidence interval 5.8-52.6) for every 10-μg/m3 increase in PM10 concentration within a 60-day period. We did not find any significant association for shorter periods or with ischemic complications. Subgroup analysis found a significantly higher incremental risk at a 60-day lag for patients ≥70 years old. Comparing patients who were chronically exposed to high PM10 levels (26.9 ± 13.8 μg/m3) to those who were not (11.9 ± 7.9 μg/m3) revealed that only in the former group was there an association between GCA onset and increased PM10 levels in the preceding 60 days. Exposure to environmental PM10 in the preceding 60 days seems to be associated with an increased risk of developing GCA, especially in older individuals with prolonged exposure to high levels of air pollution.

Polymyalgia rheumatica: What's new?

Currently, only 25% of all polymyalgia rheumatica (PMR) patients are referred to specialists. An expert committee has recently recommended confirmation of diagnosis by specialist care. This can help to avoid misdiagnoses and hospital stays and can result in lower glucocorticoid doses.Using ultrasound, magnetic resonance imagining (MRI), or positron emission tomography-computed tomography (PET-CT), typical periarticular inflammatory changes are observed, especially in the shoulder and pelvic girdle area. However, for clinical use, ultrasound is usually sufficient.In 20-25% of newly diagnosed PMR patients without symptoms of giant cell arteritis (GCA), GCA can be detected through vascular ultrasound. These patients require higher glucocorticoid doses in analogy to GCA therapy. There is growing awareness of a joint GCA-PMR spectrum disease.Glucocorticoids remain the primary treatment. The interleukin-6 inhibitor Sarilumab has recently been approved in the USA for patients with recurrent PMR. Studies have also demonstrated the effectiveness of Tocilizumab in PMR.

The Spectrum of Isolated Retinal Artery Occlusion Secondary to Giant Cell Arteritis.

We systematically reviewed the literature to investigate the clinical features of isolated arteritic retinal artery occlusion (A-RAO) associated with giant cell arteritis (GCA). The four primary types of A-RAO were central retinal artery occlusion (CRAO), hemi-central retinal artery occlusion (hCRAO), branch retinal artery occlusion (BRAO), and cilioretinal artery occlusion (CLRAO). The most reported presentation was unilateral CRAO, followed by bilateral CRAO, unilateral CLRAO, and bilateral BRAO. Most RAOs were accompanied by typical GCA signs and symptoms, which can help distinguish them from non-arteritic RAOs. When reported, temporal artery biopsy confirmed GCA in most cases. Patients with GCA may present with a broad spectrum of isolated unilateral and bilateral A-RAOs. [Ophthalmic Surg Lasers Imaging Retina 2024;55:536-540.].

Effectiveness of janus kinase inhibitors in relapsing giant cell arteritis in real-world clinical practice and review of the literature

BackgroundA substantial proportion of patients with giant cell arteritis (GCA) relapse despite standard therapy with glucocorticoids, methotrexate and tocilizumab. The Janus kinase/signal transducer and activator of transcription (JAK/STAT) signalling pathway is involved in the pathogenesis of GCA and JAK inhibitors (JAKi) could be a therapeutic alternative. We evaluated the effectiveness of JAKi in relapsing GCA patients in a real-world setting and reviewed available literature.MethodsRetrospective analysis of GCA patients treated with JAKi for relapsing disease at thirteen centers in Spain and one center in United States (01/2017-12/2022). Outcomes assessed included clinical remission, complete remission and safety. Clinical remission was defined as the absence of GCA signs and symptoms regardless of the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) values. Complete remission was defined as the absence of GCA signs and symptoms along with normal ESR and CRP values. A systematic literature search for other JAKi-treated GCA cases was conducted.ResultsThirty-five patients (86% females, mean age 72.3) with relapsing GCA received JAKi therapy (baricitinib, n = 15; tofacitinib, n = 10; upadacitinib, n = 10). Before JAKi therapy, 22 (63%) patients had received conventional synthetic immunosuppressants (e.g., methotrexate), and 30 (86%) biologics (e.g., tocilizumab). After a median (IQR) follow-up of 11 (6-15.5) months, 20 (57%) patients achieved and maintained clinical remission, 16 (46%) patients achieved and maintained complete remission, and 15 (43%) patients discontinued the initial JAKi due to relapse (n = 11 [31%]) or serious adverse events (n = 4 [11%]). A literature search identified another 36 JAKi-treated GCA cases with clinical improvement reported for the majority of them.ConclusionsThis real-world analysis and literature review suggest that JAKi could be effective in GCA, including in patients failing established glucocorticoid-sparing therapies such as tocilizumab and methotrexate. A phase III randomized controlled trial of upadacitinib is currently ongoing (ClinicalTrials.gov ID NCT03725202).

Presence of subclinical giant cell arteritis in patients with morning stiffness of duration less than 45 minutes at the time of diagnosis of polymyalgia rheumatica.

In some patients with polymyalgia rheumatica (PMR), giant cell arteritis (GCA) is subclinical as underlying inflammation of large vessels (LV) is present without evidence of related clinical manifestations. Different factors have been proposed as predictive of subclinical GCA in PMR patients. To date, the literature reports scant data about the association between subclinical GCA and long-lasting morning stiffness (MS) in patients at the time of diagnosis of PMR. Given this background, the aim of this study was to assess the association between subclinical GCA and MS < 45 min in patients with newly diagnosed PMR. We performed an observational, retrospective, single-centre cohort study of patients consecutively referred to our public out-of-hospital rheumatologic clinic between January 2015 and December 2020, who could be classified as having PMR according to the 2012 EULAR/ACR criteria. Subclinical GCA was investigated through ultrasound examination of a core set of arteries (temporal, axillary, common carotid, and subclavian arteries), in accordance with the EULAR recommendations for the use of imaging in LV vasculitis. Patients who did not have GCA symptoms but showed halo sign in at least one of these arteries were described as having subclinical GCA. We included a total of 143 patients (35 men and 108 women). Their median age was of 71.5 years. Thirty-five had MS duration < 45 min at the time of PMR diagnosis. Subclinical GCA was found in 23 PMR patients (16.1%); 18 had a cranial and 5 an extracranial GCA. A univariate analysis highlighted that MS < 45 min was associated with a lower prevalence of GCA (OR = 0.11, 95% CI: 0.04-0.29; p < 0.0001). This association was retained in a multivariable analysis that accounted for 6 different potential covariates (OR = 0.06, 95% CI: 0.01-0.26; p < 0.0001. In our study MS < 45 min at the time of PMR diagnosis was associated with a significantly lower risk of subclinical GCA, when patients were screened by ultrasound, of approximately 90%. Identification of a more accurate MS cut-off value could improve the accuracy for subclinical GCA in patients with newly diagnosed PMR.

Subclinical giant cell arteritis increases the risk of relapse in polymyalgia rheumatica

ObjectiveThe aim of the present study was to determine the clinical significance of subclinical giant cell arteritis (GCA) in polymyalgia rheumatica (PMR) and ascertain its optimal treatment approach.MethodsPatients with PMR...

Tongue Necrosis Secondary to Giant Cell Arteritis

Giant cell arteritis (GCA) is a vasculitis of large and medium sized vessels that commonly affects extracranial branches of the internal and external carotid arteries. It is a diagnosis of the elderly that typically presents temporal tenderness, claudication of the jaw or vision loss. Lingual necrosis is unusual symptom of GCA. We report a case of GCA revealed by a tongue necrosis.

Giant cell arteritis with vertebral artery involvement-baseline characteristics and follow-up of a monocentric patient cohort.

Vertebral artery (VA) involvement in giant cell arteritis (GCA) has rarely been reported. We aimed to evaluate the prevalence, patients' characteristics, and immunotherapies used in patients with GCA and VA involvement at diagnosis and 1 year follow-up, retrospectively including patients being diagnosed between January 2011 and March 2021 in our department. Clinical features, laboratory data, VA imaging, immunotherapy, and 1 year follow-up data were analyzed. Baseline characteristics were compared to GCA patients without VA involvement. Among all 77 cases with GCA, 29 patients (37.7%) had VA involvement, as diagnosed by imaging and/or clinical signs and symptoms. Gender distribution and erythrocyte sedimentation rate (ESR) were significantly different in the groups with and without VA involvement, with more women being affected (38/48 patients, 79.2%) and a significantly higher median ESR in patients without VA involvement (62 vs. 46 mm/h; p = 0.012). MRI and/or CT showed vertebrobasilar stroke at GCA diagnosis in 11 cases. 67/77 patients (87.0%) received high-dose intravenous glucocorticosteroids (GCs) at diagnosis, followed by oral tapering. Six patients were treated with methotrexate (MTX), one with rituximab, and five with tocilizumab (TCZ). 2/5 TCZ patients achieved clinical remission after 1 year, vertebrobasilar stroke within the first year occurred in 2/5 patients. Diagnosis of VA involvement might be underrecognized in GCA patients. VA imaging should be performed in elderly patients with vertebrobasilar stroke presenting with GCA symptoms, not to miss GCA as the etiology of stroke. Efficacy of immunotherapies in GCA with VA affection and long-term outcomes need to be investigated further.

P028 Oh No-Cardiosis! A case of systemic Nocardia nova infection

Abstract Background/Aims Nocardiosis is a rare infectious disease caused by the aerobic bacteria genus Nocardia and can cause severe suppurative disease affecting virtually any organ. It tends to affect the immunocompromised, making rheumatology patients a particular risk group for this infection. It is not normal human flora and is found in soil, decaying vegetable matter and aquatic environments. It can become airborne and the most common means of entry is thought to be dust inhalation. Methods Our patient is a 75-year-old woman with giant cell arteritis (GCA) treated with prednisolone, SC methotrexate and tocilizumab (started 4 months before her acute illness). Symptoms began with a fall in the garden sustaining a nasty laceration to her leg and an injury to her back worsening some chronic lower back pain (X-rays did not show any fracture). Over the next 5 weeks due to a productive cough and sweats she was prescribed oral antibiotics with no improvement. She was then admitted to hospital due to an acute increase in the severity of her lower back pain alongside a change in character to a burning pain. Her presenting CRP was 32, likely due to having held tocilizumab due to a presumed chest infection. MRI showed a collection in the right erector spinae muscle extending from C7 to the iliac crest and another large collection in the right thigh. A mass in the left sub-mammary area led to a chest CT that showed a chest wall abscess alongside a spiculated lesion in the lung, and further nodular changes. Peripheral blood cultures were negative and eventually draining of the erector spinae abscess led to culture of Nocardiosis nova. Imipenam and Septrin were given as per sensitivities. Unfortunately, our patient has had a difficult clinical course. During her hospital stay she has had possible DRESS syndrome/Septrin-induced liver injury alongside drug induced neutropaenia and lymphopaenia and a possible stroke (this was thought less likely to represent CNS Nocardiosis). Results Her rheumatology management initially involved stopping her DMARDs however due to flares in symptoms her doses of prednisolone have been up and down. It has been difficult to separate symptoms that may be due to cervical spine collections and a possible CNS lesion from GCA symptoms. Conclusion This case demonstrates the need to thoroughly investigate immunosuppressed patients when they are not responding as expected to antibiotics or present with atypical symptoms. It also demonstrates the importance of obtaining a microbiological diagnosis. Cellular immunity is generally thought to be key in preventing Nocardia dissemination however in our patient’s case she was only taking 2.5mg prednisolone when admitted. This is one of only a few case reports of patients developing Nocardia infection whilst on tocilizumab. Disclosure R.J. Crowder: None. H. Mills: None. O. Savanovic-Abel: None. J. King: None. O. Moore: None. M. Perry: None. S. Hewagamage: None. M. Gunathilaka: None.

Long delay from symptom onset to first consultation contributes to permanent vision loss in patients with giant cell arteritis: a cohort study

ObjectivesTo characterise factors associated with permanent vision loss (PVL) and potential reasons for the therapeutic delay contributing to PVL in giant cell arteritis (GCA).MethodsRetrospective analysis of GCA patients diagnosed at...

Temporal Artery Biopsy Debate: Positive TAB Result Prolongs Steroid Use in Giant Cell Arteritis.

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.

PARACENTRAL ACUTE MIDDLE MACULOPATHY IN GIANT CELL ARTERITIS.

To describe a representative case and review the literature on paracentral acute middle maculopathy (PAMM) and giant cell arteritis (GCA). A review of the English-language ophthalmic literature was performed using the search terms of PAMM, giant cell arteritis, and temporal arteritis. A 72-year-old woman with PAMM as the presenting ophthalmic manifestation of GCA was described with a review of the prior cases from the literature. It was found that there were 26 cases of PAMM in GCA. In 19 of 26 cases, PAMM was associated with no other fundus abnormalities and was only seen on multimodal imaging including optical coherence tomography. PAMM can cause acute paracentral visual loss, and GCA should be suspected in all cases of PAMM of the elderly patients, even when isolated and not associated with constitutional symptoms of GCA.

Diplopia as the presenting symptom in giant cell arteritis

Acquired cranial nerve palsies commonly present to primary eye care clinics and are associated with a variety of etiologies. While microvascular ischemia is the most common association in adults over age fifty, cranial nerve palsies can be associated with other more insidious etiologies, like giant cell arteritis (GCA). There are traditional clinical guidelines for additional diagnostic testing in acute cranial nerve palsies to help identity the other non-microvascular causes, but the guidelines do not specifically address GCA. Although rare, clinicians need to be aware that diplopia can be associated with GCA and use systemic symptoms like jaw claudication and neck pain to screen all acute cranial nerve palsies in adults over age fifty. This case report highlights an uncommon presentation of GCA, emphasizes the importance of broadening a diplopia differential diagnosis list, and provides a brief GCA review.

Incidence of giant cell arteritis in six districts of Paris, France (2015–2017)

This prospective population-based study estimated the incidence of giant cell arteritis (GCA) in northeastern Paris. GCA cases diagnosed between 2015 and 2017 were obtained from local hospital and community-based physicians and the national health insurance system database. Criteria for inclusion were living in the study area at that time and fulfilling the 1990 American College of Rheumatology classification criteria and/or its expanded version. Cranial and large-vessel GCA cases were defined by the presence or absence of cranial signs and/or symptoms, respectively. Annual incidence was calculated by dividing the number of incident cases by the size of the study population ≥ 50years old. Completeness of case ascertainment was assessed by a three-source capture-recapture analysis. Among the 62 included cases, 42 (68%) were women, mean (± SD) age 77.3 ± 9.1years. The annual incidence ofGCA in northeastern Parisand completeness of case ascertainment were estimated at 7.6 (95% CI 5.9-9.8) per 100,000 inhabitants ≥ 50years old and 66% (95% CI 52-92%), respectively. Incidence increased with age, peaked at age 80-89years, and was almost twice as high in women versus men. Large-vessel GCA cases, mean (± SD) age 68.6 ± 11.5years, accounted for 8% of all GCA cases.In this study,GCA epidemiology was mainly driven by cases with cranial GCA signs or symptoms and incidence results were consistentwith recent European and past French studies.

Case Report: Giant Cell Arteritis presenting with 6th Nerve Palsy without Ischemic Optic Neuropathy

Purpose: Giant cell arteritis (GCA) can be a difficult condition to identify in the early stages especially in the absence of the pathognomonic arteritic anterior ischemic optic neuropathy. Optometrists serve an important role in correctly triaging and initiating appropriate work-up and treatment for this emergent condition. This case report and review serves as a refresher of the systemic and ocular signs and symptoms of GCA. Background: GCA is a systemic autoimmune condition characterized by granulomatous inflammation of medium and large arteries in patients over 50. The most well-known constellation of signs and symptoms include new onset headache, jaw claudication, scalp tenderness, temporal artery abnormality with painless vision loss secondary to arteritic anterior ischemic optic neuropathy. However, it is important to be aware of alternate presenting signs and symptoms including pain anywhere in the distribution of the external carotid (occipital, neck, tongue, throat, ear) and signs of large vessel GCA (arm/limb claudication, chest/back pain, Raynaud’s phenomenon) and Polymyalgia Rheumatica (PMR) (bilateral hip/shoulder pain and morning stiffness). Case Report: An 86-year-old Caucasian female presents for a referred exam regarding new onset diplopia with concurrent occipital headache, neck pain and sore throat originally dismissed as symptoms of her other systemic health conditions. ESR, CRP and platelets were elevated on serology and subsequent temporal artery biopsy was positive for GCA. Oral steroids were initiated, and she was lost to follow up after her ocular symptoms resolved. Conclusion: GCA can present with a large range of manifestations, and many are nonspecific and easily attributable to other causes especially when it deviates from the classic constellation of new onset headache, jaw claudication, scalp tenderness and temporal artery abnormality with painless vision loss. Optometrists as primary eye care providers may be the first point of contact and need to be cognizant of the broader set of manifestations to minimize delays in diagnosis and treatment of this life and vision threatening condition. CE Notification: This article is available as a COPE accredited CE course. You may take this course for 1-hour credit. Read the article and take the qualifying test to earn your credit. Click here to Enroll (https://www.crojournal.com/case-report-giant-cell-arteritis-presenting-with-6th-nerve-palsy-without-ischemic-optic-neuropathy) Please check COPE course expiry date prior to enrollment. The COPE course test must be taken before the course expiry date.

OP0185 TOCILIZUMAB IN COMBINATION WITH 8 WEEKS OF PREDNISONE FOR GIANT CELL ARTERITIS

BackgroundEven with the use of tocilizumab (TCZ), significant glucocorticoid exposure (usually ≥ 6 months) continues to be an important problem in giant cell arteritis (GCA).ObjectivesWe aimed to evaluate the efficacy and safety of tocilizumab (TCZ) in combination with 2 months of prednisone in a group of patients with GCA.MethodsWe conducted a prospective, single arm, open-label study of TCZ in combination with 2 months of prednisone for new-onset and relapsing GCA patients with active disease (ClinicalTrials.gov Identifier NCT03726749). GCA diagnosis required confirmation by temporal artery biopsy or vascular imaging. Active disease was defined as presence of cranial or polymyalgia rheumatica symptoms necessitating treatment within 6 weeks of baseline. All patients received TCZ 162 mg subcutaneously every week for 12 months and an 8-week prednisone taper starting between 20 mg and 60 mg daily (Figure 1). The primary endpoint, sustained prednisone-free remission, was defined as absence of relapse from induction of remission up to week 52 while adhering to the prednisone taper. Relapse was defined as the recurrence of symptoms of GCA requiring treatment intensification regardless of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels. Safety was also evaluated.Figure 1.Clinical Trial SchemaResultsBetween 11/2018 and 11/2020 we enrolled 30 patients (mean age 74 years, 60% females, 50% new-onset disease, 77% temporal artery biopsy-proven, 47% imaging-proven). The mean ESR and CRP at screening were 45 mm/hour and 48 mg/L, respectively. The initial prednisone dose was 60 mg (n = 7), 50 mg (n = 1), 40 mg (n = 7), 30 mg (n = 6) and 20 mg (n = 9). All patients entered remission within 4 weeks of baseline. The primary endpoint was achieved by 23 (77%) patients (Table 1). The mean (SD) cumulative prednisone dose in these 23 patients was 1052 (390) mg. After a mean period of 16 weeks, 7 (23%) patients relapsed (Table 1). All relapses but one occurred after the completion of the study prednisone taper. Overall, 6 of the 7 patients with relapse received a second prednisone taper over 8 weeks. Of these 6 patients, 4 achieved and maintained remission for the remainder of the trial period, and 2 withdrew from the study after having a second relapse. One patient with relapse received a second prednisone taper over 26 weeks and stayed in remission until the end of the study. The mean (SD) cumulative prednisone dose in the 7 patients with relapse was 1883 (699) mg (Table 1). Overall, 4 (13%) participants developed a serious adverse event (Table 1). No cases of ischemia-related visual symptoms including permanent vision loss occurred during the study.Table 1.Efficacy and Safety OutcomesGCA patients(n = 30)Efficacy Sustained, prednisone-free remission by week 5223.0 (76.7) Cumulative prednisone dose (mg) at week 52, mean (SD)1051.5 (390.3) Relapse7.0 (23.3) Time to relapse, weeks: mean (SD)15.8 (14.7) Prednisone dose (mg/day) at relapse, mean (SD)2.1 (5.2) Cumulative prednisone dose (mg), mean (SD)1883.1 (699.2) Clinical manifestations at relapse Cranial symptoms4 out of 7 patients Ischemic visual symptoms0 out of 7 patients PMR symptoms4 out of 7 patientsSafety Serious adverse events4.0 (13.3) Cellulitis1 COVID-191 Fragility fracture1 Cholecystitis1Values represent number and (%) unless otherwise specified. SD, standard deviation; PMR, polymyalgia rheumaticaConclusionThese results suggest that 12 months of TCZ in combination with 8 weeks of prednisone could be efficacious for inducing and maintaining disease remission in patients with GCA. Confirmation of these findings in a randomized controlled trial is required.Disclosure of InterestsSebastian Unizony Consultant of: Kiniksa, Sanofi, Janssen, GSK, Grant/research support from: Janssen, Genentech, Mark Matza: None declared, Adam Jarvie: None declared, Ana Fernandes: None declared, John H. Stone Consultant of: Roche/Genentech, Grant/research support from: Roche/Genentech

Vascularite des artères utérines révélant une artérite à cellules géantes : cas clinique et revue de la littérature

IntroductionGenital vasculitis are uncommon. They may be localized or be a manifestation of a systemic vasculitis. We report a patient with a giant cell arteritis (GCA) involving uterine arteries and a literature review on genital vasculitis. Case reportA 65-year-old woman was referred to a gynecologist for a cervical intraepithelial neoplasia (CIN) associated with an ovarian mass. An unexpected diagnosis of GCA involving small to medium sized uterine arteries was made through the anatomopathological analysis while the patient was asymptomatic. Two weeks later, she presented typical cranial symptoms of giant cell arteritis (GCA). PET-scanner confirmed the diagnosis of GCA with an involvement of the ascending aorta, and the axillary and the subclavian arteries. ConclusionGynecologic vasculitis are rare and usually an asymptomatic manifestations of GCA.

Giant cell arteritis with cervical radiculopathy mimicking polymyalgia rheumatica and elderly-onset rheumatoid arthritis: a case report

BackgroundGiant cell arteritis has a wide variety of clinical symptoms, one of them being cervical radiculopathy, which mainly involves the C5 nerve root. If the patient does not develop typical clinical symptoms of giant cell arteritis but has C5 radiculopathy, it may be misdiagnosed as polymyalgia rheumatica or elderly-onset rheumatoid arthritis due to old age, high serum inflammatory markers, and difficulty in raising both upper limbs.Case presentationA 72-year-old Japanese man with a month-long history of dyspnea on exertion and with difficulty in raising both upper limbs was referred to our hospital because of elevated serum C-reactive protein (12.62 mg/dL). He had no typical symptoms of giant cell arteritis such as headache, jaw claudication, visual loss, and fever. The patient tested negative for rheumatoid factor and anti-cyclic citrullinated peptide antibody, and matrix metalloproteinase-3 was within the normal range (54.3 ng/mL). Musculoskeletal ultrasound examination showed absence of tenosynovitis, bursitis, and synovitis, and the patient did not meet the classification criteria of polymyalgia rheumatica or rheumatoid arthritis; hence, those two diseases were unlikely. A precise neurological examination suggested bilateral C5 and C6 anterior radiculopathy and left C4 radiculopathy. Since cervical magnetic resonance imaging showed no mechanical causality, cervical radiculopathy of unknown origin was suggested. Fluorodeoxyglucose positron emission tomography/computed tomography revealed increased fluorodeoxyglucose lineal uptake along the vessel walls, including temporal arteries, vertebral arteries, and axillary arteries. Results of the biopsy of the left superficial temporal artery were compatible with giant cell arteritis. He was successfully treated with 30 mg of prednisolone, and both upper limbs could be elevated.ConclusionsIf the patient was misdiagnosed with polymyalgia rheumatica or elderly-onset rheumatoid arthritis based on only clinical symptoms and laboratory data, his symptoms might not improve due to insufficient steroid dose and vascular complications may occur later. Although rare, peripheral neuropathy in giant cell arteritis may include cervical radiculopathy. The musculoskeletal ultrasound and precise neurological examination were the turning points for the diagnosis of this case, and making a careful diagnosis using these methods was important.

Ustekinumab for the Treatment of Giant Cell Arteritis

To evaluate the efficacy and safety of ustekinumab (UST) in giant cell arteritis (GCA). We conducted a prospective, open-label trial of UST in patients with active new-onset or relapsing GCA. Active disease was defined as the presence of GCA symptoms and elevation of erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) level within 6 weeks of baseline. All patients received a 24-week prednisone taper and subcutaneous UST 90 mg at baseline and at weeks 4, 12, 20, 28, 36, and 44. The primary endpoint, prednisone-free remission, was defined as the absence of relapse through week 52 and normalization of the ESR and CRP level. Relapse was defined as the recurrence of GCA symptoms requiring treatment intensification. A sensitivity analysis excluding ESR/CRP level normalization from the prednisone-free remission definition was performed. The study enrolled 13 patients (target sample size 20). Enrollment was closed prematurely after 7 of the initial 10 patients relapsed. Five patients (39%) had new-onset disease. The initial prednisone doses were 20 mg (1 patient), 40 mg (9 patients), and 60 mg (3 patients). All patients entered disease remission within 4 weeks of baseline. Only 3 (23%) achieved the primary endpoint. Of the 10 patients (77%) who failed to achieve the primary endpoint, 7 relapsed after a mean period of 23 weeks. The remaining 3 patients met the alternative definition of prednisone-free remission that did not require ESR/CRP level normalization. One serious adverse event occurred. UST combined with 24 weeks of prednisone was associated with a high rate of treatment failure in this prospective GCA trial.