Treatment Of Giant Cell Arteritis Research Articles

Polymyalgia rheumatica and giant cell arteritis: diagnosis and management.

There have been advances in the diagnosis and treatment of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR). Themes in PMR and GCA include classification criteria, ultrasound imaging of temporal and axillary arteries replacing biopsies for diagnosis of GCA, faster diagnosis and treatment with rapid access clinics for suspected GCA, and expanding treatment options with the goal of rapid suppression of inflammation and sparing steroids. Treatment is aimed at suppressing inflammation quickly in both GCA and PMR. Randomized trials have demonstrated success in reducing glucocorticoids when adding advanced therapies such as interleukin 6 (IL6) inhibitors. Other treatments including Janus kinase (JAK) inhibitors (especially a phase 3 trial of upadacitinib at 15 mg daily and secukinumab (an IL17 inhibitor) are being tested. Some uncontrolled GCA protocols are limiting glucocorticoids to initial IV pulse therapy only or rapid tapering of oral glucocorticoids with upfront treatment with tocilizumab. There is uncertainty of who should have an advanced therapy and how long to use it for and what order to consider advanced therapies when treatment fails. In PMR, studies are performed when patients cannot taper glucocorticoids effectively, whereas in GCA, advanced therapies are started with disease onset or with recurrent GCA.

Safety and Efficacy of Long-Term Tocilizumab in a Cohort of Patients with Giant Cell Arteritis: An Italian Monocentric Retrospective Study.

Tocilizumab (TCZ) is the only biologic drug approved for the treatment of giant cell arteritis (GCA), having clinical trials and real-life studies proved its efficacy and safety. However, the optimal duration of the treatment has yet to be determined, being its early interruption associated with an increased risk of relapse. Conversely, prolonged schemes of therapy may rise safety concerns. The aim of the study was to evaluate the incidence of adverse events (AEs) and remission/relapse rate in a cohort of GCA patients treated with TCZ and an accelerated steroid tapering scheme, followed for 24 months. We retrospectively included patients referring to our clinic from January 2019 to November 2021 who were diagnosed with GCA and started subcutaneous TCZ treatment (162 mg/week). They also received up to 62,5 mg of prednisone (PDN), tapered following an accelerated six-month scheme. We collected 38 patients, with a mean age of 76,4 years, treated with TCZ for an average of 22,3 months. AEs occurred in 11 (29%) subjects, and only one serious AE was reported; 7 (18%) patients permanently discontinued TCZ. At the end of the follow-up, all the patients continuing treatment showed clinical remission, with a PDN dosage <5mg. We registered 3 (8%) minor relapses under TCZ, after an average of 15 months. Our data support the evidence of a safe and effective long-term use of TCZ in GCA patients, especially when combined with moderate GCs doses for the shortest possible duration.

Experience with olokizumab in the treatment of polymyalgia rheumatica and giant cell arteritis: A series of cases

Glucocorticoids are the basis for the treatment of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR), however, their long-term use is associated with a number of well-known side effects. Despite the fact that a significant number of patients with these diseases require long-term treatment, approaches to long-term therapy remain insufficiently developed. The role of traditional anti-inflammatory drugs remains uncertain due to insufficient efficacy and inconclusive evidence base. A possible solution of this problem is the use of biological agents, in particular, affecting the interleukin (IL-6) axis. This series of clinical observations presents the experience of using the IL-6 inhibitor olokizumab in the treatment of GCA and PMR. The data obtained are consistent with the positive international experience of using drugs of this group and demonstrate both efficiency of disease activity control and a pronounced steroid-sparring effect. Olokizumab is a promising drug for the treatment of GCA and PMR.

Pharmacotherapy of giant cell arteritis and polymyalgia rheumatica: Prospects for the use of monoclonal antibodies to interleukin 6

Giant cell arteritis (GCA) and polymyalgia rheumatica (RPM) are immune-mediated rheumatic disease (IMRDs), which typically develop in people over 50 years of age. Currently, GCA and PMR are considered within a single complex autoimmune-autoinflammatory pathology, defined as “GCA-PMR spectrum disease”. This was reflected in the development of general recommendations for pharmacotherapy within the framework of the “treat-to-tar get” strategy, although specific approaches to the treatment of patients with GCA and RPM differ depending on the clinical and pathogenetic characteristics of each nosological form. Glucocorticoids (GCs) are central to the treatment of GCA and RPM. Attention is drawn to the paradoxical discrepancy between the high effectiveness of GCs in the short term and the increasing severity of pathology associated with persistent inflammatory activity and the accumulation of organ damage induced by GCs in the long term, which indicates the need to improve therapy, primarily in the direction of optimizing the use of GCs. New opportunities for pharmacotherapy of GCA and RPM (as well as other IMRDs) are associated with the use of biologic agents that block the activity of cytokines involved in the immunopathogenesis of diseases, and in recent years, JAK (Janus kinase) inhibitors. Among pharmacological “targets,” special attention is drawn to interleukin (IL) 6, a pleiotropic cytokine involved in the development of inflammation, immune response, immunometabolism, cancerogenesis, vascular wall remodeling, etc. Currently, several biologic agents have been developed that are specific to both IL-6 receptor and IL-6: humanized monoclonal antibodies (mAbs) to the IL-6 receptor (tocilizumab), and human mAbs to IL-6 receptor (sarilumab and levilimab (BIOCAD)), humanized mAbs to IL-6 olokizumab (R-PHARM), etc. The article summarizes data regarding the effectiveness and safety of tocilizumab in GCA and RPM, recommendations for the use of IL-6 inhibitors in these diseases and discusses the prospects for further research.

Pharmacological advances in giant cell arteritis treatment

Pharmacological advances in giant cell arteritis treatment

Treatment of giant cell arteritis with ultra-short glucocorticoids and tocilizumab: results from the extension of the TOPAZIO study.

To assess the maintenance of efficacy of one year of tocilizumab (TCZ) monotherapy after its discontinuation in large vessel-GCA (LV-GCA). 17 patients with active LV-GCA were previously treated with 3 boluses of intravenous methylprednisone and weekly subcutaneous TCZ in monotherapy for 52 weeks. Patients in relapse-free clinical remission at week 52 discontinued TCZ and entered part two, which was a 26-week observational follow-up period. PET/CT was performed in all patients at the end of the 26-week observational period (week 78). End points were the variation in PET vascular activity score (PETVAS) at week 78 compared with baseline and with week 52, and the proportion of patients with relapse-free clinical remission at week 78 and at the end of the follow-up. Compared with baseline, a significant reduction in PETVAS was observed at week 78, mean (95% CI) change -6.6 (-9.5 to -3.7). However, compared with week 52, PETVAS significantly increase 6 months after TCZ discontinuation (week 78), mean (95% CI) change 4.6 (0.7-8.5). The proportion of patients with relapse-free clinical remission at weeks 78 and at the end of the follow-up (median time from TCZ discontinuation 148 weeks) was 11/17 (65%, 95% CI 38-86) and 8/17 (47%, 95% CI 23-72), respectively. Age and sex-adjusted HR (95% CI) for each unit increase of PETVAS indicating subsequent relapse was 1.36 (0.92-2.00). One year of TCZ monotherapy was effective in maintaining drug-free clinical remission in LV-GCA. Changes in PETVAS early after TCZ discontinuation may predict subsequent relapses. ClinicalTrials.gov, NCT05394909.

Faster steroid-free remission with tocilizumab compared to methotrexate in giant cell arteritis: a real-life experience in two reference centres.

Glucocorticoids (GCs) are still the mainstay of treatment of giant cell arteritis (GCA). Although GCs are highly effective in GCA, the high burden of toxicity of GCs as well as the disease relapse during GC tapering is well documented. To compare the efficacy and rapidity of TCZ and MTX as steroid-sparing agents in a real-life cohort of GCA patients. A retrospective analysis was conducted including patients with newly diagnosed GCA from the Rheumatology Units of Udine and Rome. The inclusion criterion was the treatment with TCZ or MTX as first steroid-sparing drug. 112 GCA patients (81 females) with a median age of 70 (IQ 65-75) years were collected. Thirty-one out of 112 (27.7%) patients were treated with TCZ (162mg/week), while 81/112 (72.3%) patients received MTX (up to 20mg/week) as a GC-sparing agent. At month 6 after GCA onset, 5/31 (16.1%) patients in TCZ group and none in MTX group were in GC-free sustained remission (p value = 0.001). Similarly, at month 12, 64.5% (20/31) and 11.1% (9/81) of patients were in sustained GC-free remission in TCZ and MTX group, respectively (p value <0.001). At month 24 of follow-up, at least one relapse of the disease occurred in 7/31 (22.6%) in TCZ-treated and 28/81 (34.6%) in MTX-treated patients, respectively (p value = 0.22). TCZ allowed a faster discontinuation of steroid therapy than MTX in GCA patients, without increasing the risk of relapse.

Taper versus discontinuation of tocilizumab in patients with giant cell arteritis: Real-world experience from a tertiary center

IntroductionFollowing the approval of tocilizumab (TCZ) for giant cell arteritis (GCA), recent studies have shown a high relapse frequency after abrupt discontinuation of TCZ. However, a thorough exploration of TCZ tapering compared to abrupt discontinuation has never been undertaken. Likewise, adverse events have only been scarcely investigated in routine care. This study aimed to compare the incidence of relapses in GCA patients undergoing TCZ tapering compared to abrupt discontinuation. MethodsWe performed a single-center retrospective cohort study from 2012 to 2022. Data from GCA patients treated with TCZ was obtained from the Electronic Patients Record. Relapse-free survival is reported in Kaplan-Meier plots and tapering versus abrupt discontinuation were compared using a Wilcoxon-Brewlos-Gehan test. ResultsWe included 155 patients receiving TCZ treatment for GCA, of which 104 discontinued TCZ. Among the 104 patients discontinuing TCZ, 42 (40 %) experienced a relapse within the first year. A total of 57 patients underwent taper with 6/38 (16 %) and 2/19 (11 %) relapsing while receiving TCZ every second or third week, respectively. In comparison, 59 patients underwent abrupt discontinuation with 27 (46 %) relapsing during follow-up. The patients undergoing abrupt TCZ discontinuation demonstrated a significantly shorter time to relapse compared to all tapered patients (p = 0.02) as well as patients tapered from weekly TCZ treatment to every second week (p < 0.01). Furthermore, 15 % of patients discontinued TCZ due to adverse events. ConclusionThis is the first study indicating that TCZ taper induced longer relapse-free survival than abrupt discontinuation, implying that taper may be favored over discontinuation in patients with GCA.

E076 Incidence of adrenal insufficiency in patients with giant cell arteritis tapering glucocorticoids with the Norwich prednisolone regimen

Abstract Background/Aims Glucocorticoids continue to be the main treatment for giant cell arteritis (GCA). It is widely recognised that treatment with oral glucocorticoids is associated with adverse events including adrenal insufficiency. Reports suggest the risk of adrenal insufficiency is dependent on the dose and the duration of therapy. The Norwich regimen has been devised to deliver prednisolone at a rate 1mg/kg of lean body mass tapering over 100 weeks. We report the incidence of adrenal insufficiency related to the Norwich regimen. Methods The Norwich regimen has been used in all patients diagnosed with GCA from 01/01/2012. A notes review of all patients diagnosed after that date was undertaken to look for evidence of adrenal insufficiency. We routinely check 9am cortisol when symptoms of adrenal insufficiency were reported. The 9am cortisol was recorded as normal, indeterminate, or low. Patients with a low 9am cortisol remained on long term prednisolone; no further test was required. Patients with an indeterminate result were referred for a short synacthen test (SST). If the results of the SST were normal patients were weaned off prednisolone, patients with an inadequate SST result remained on long term prednisolone. Results From 01/01/2012-31/05/2022 353 patients diagnosed with GCA were treated with the Norwich regimen. 9am cortisol was checked in 76 patients (21.5%). Of these, 34 patients (9.6%) had a normal result, 35 patients (9.9%) had an indeterminate result requiring SST. 7 patients (2.0%) had a low 9am cortisol. Of the 35 patients referred for SST: 27 patients (7.6%) had an adequate result, 8 patients (2.3%) had an inadequate result resulting in long term prednisolone. Conclusion We report the incidence of adrenal insufficiency in patients diagnosed with GCA, tapering prednisolone using the Norwich regimen. In total 15/353 (4.3%) patients developed adrenal insufficiency because of long-term glucocorticoid use. Disclosure G. Ducker: None. C.B. Mukhtyar: None.

E009 An audit looking into bisphosphonate co-prescription and compliance in patients with giant cell arteritis

Abstract Background/Aims The treatment of giant cell arteritis includes glucocorticoid use to reduce inflammation. There are known drawbacks of long-term glucocorticoid use, including osteopenia and osteoporosis. The British Society for Rheumatology giant cell arteritis guidelines recommend that we should consider appropriate pharmacological bone protection at the time of presentation to counteract this. Glucocorticoid toxicity risks increases with both dosage and duration of steroid use. To reduce steroid burden, we consider introduction of the steroid sparing agents such as tocilizumab and methotrexate. This audit was to look into whether we were locally achieving bone protection consideration and to whether steroid sparing agents were helping in terms of compliance of bisphosphonates. Methods We retrospectively looked at the clinical letters of the patients locally diagnosed with giant cell arteritis in the previous 3 years to ascertain whether there was documentation of decisions around starting bisphosphonates at the time of diagnosis and whether the patients tolerated bisphosphonate therapy for the recommended duration. Patients were classified into 3 groups, as to whether their treatment was glucocorticoids with tocilizumab (TOC), glucocorticoids with methotrexate (MTX) or glucocorticoid monotherapy (MONO). Results 49 Patients were identified. 3 of these patients were excluded for various reasons. 17 were in the TOC group, 11 in the MTX group, and 18 in the MONO group. Documentation of consideration of bisphosphonates at diagnosis was 94% in TOC, 91% in MTX and 100% in MONO. Overall this was 96%. Compliance of bisphosphonate therapy for the recommended duration was 88% in TOC, 90% in MTX and 86% in MONO. Overall compliance was 88%. There was no statistically significant difference between the steroid sparing groups and the monotherapy group both in terms of consideration of initiation of bisphosphonate treatment and compliance of bisphosphonate treatment, with p values of 0.52 and 0.8 respectively. Conclusion Overall, we found that locally there was good consideration of bisphosphonate therapy at diagnosis and that compliance remained good throughout treatment. This can help reduce the side effect profile of glucocorticoid toxicity. Although, there was no statistically significant difference in our study of concordance of bisphosphonates between the two groups, steroid sparing agents are useful in our arsenal at reducing the risks of glucocorticoid toxicity. Disclosure A.A. Bolger: None. L. Hutton: None.

Comment on: Treatment of giant cell arteritis with ultra- short glucocorticoids and tocilizumab: the role of imaging in a prospective observational study. Reply.

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Comment on: Treatment of giant cell arteritis with ultra-short glucocorticoids and tocilizumab: the role of imaging in a prospective observational study.

Comment on: Treatment of giant cell arteritis with ultra-short glucocorticoids and tocilizumab: the role of imaging in a prospective observational study.

Diagnostics and treatment of large vessel vasculitis

Giant cell arteritis (GCA) and Takayasu arteritis (TAK), as the main representatives of large vessel vasculitis, are rheumatological autoimmune disorders associated with inflammatory vessel wall changes in the arterial system that can lead to many types of organ damage. In this review the current scientific evidence on the diagnostics and treatment of large vessel vasculitis is evaluated and discussed. In addition to the medical history and clinical presentation, imaging techniques nowadays represent the core of large vessel vasculitis diagnostics and have largely replaced the histological confirmation of GCA. After the diagnosis, acute treatment with glucocorticoids should be initiated as rapidly as possible but in the long term this should be tapered out or replaced by asteroid-sparing basic treatment. In contrast to GCA with already available options and other biologic disease-modifying antirheumatic drugs (DMARDs) about to be approved, there are still no approved biologic DMARD treatment options available for the less common TAK. In contrast to the substantial progress in imaging diagnostics of large vessel vasculitis and with respect to the treatment of GCA, the much rarer TAK still requires intensive research efforts, especially to improve the treatment situation.

The Meteoritics Trial: efficacy of methotrexate after remission-induction with tocilizumab and glucocorticoids in giant cell arteritis—study protocol for a randomized, double-blind, placebo-controlled, parallel-group phase II study

BackgroundGlucocorticoids (GC) are the standard treatment for giant cell arteritis (GCA), even though they are associated with adverse side effects and high relapse rates. Tocilizumab (TCZ), an interleukin-6 receptor antagonist, has shown promise in sustaining remission and reducing the cumulative GC dosage, but it increases the risk of infections and is expensive. After discontinuation of TCZ, only about half of patients remain in remission. Additionally, only few studies have been conducted looking at remission maintenance, highlighting the need for alternative strategies to maintain remission in GCA. Methotrexate (MTX) has been shown to significantly decrease the risk of relapse in new-onset GCA and is already a proven safe drug in many rheumatologic diseases.MethodsThis study aims to evaluate the efficacy and safety of MTX in maintaining remission in patients with GCA who have previously been treated with GC and at least 6 months with TCZ. We hypothesize that MTX can maintain remission in GCA patients, who have achieved stable remission after treatment with GC and TCZ, and prevent the occurrence of relapses. The study design is a monocentric, randomized, double-blind, placebo-controlled, parallel-group phase II trial randomizing 40 GCA patients 1:1 into a MTX or placebo arm. Patients will receive 17.5 mg MTX/matching placebo weekly by subcutaneous injection for 12 months, with the possibility of dose reduction if clinically needed. A 6-month follow-up will take place. The primary endpoint is the time to first relapse in the MTX group versus placebo during the 12-month treatment period. Secondary outcomes include patient- and investigator-reported outcomes and laboratory findings, as well as the prevalence of aortitis, number of vasculitic vessels, and change in intima-media thickness during the study.DiscussionThis is the first clinical trial evaluating remission maintenance of GCA with MTX after a previous treatment cycle with TCZ. Following the discontinuation of TCZ in GCA, MTX could be a safe and inexpensive drug.Trial registrationClinicalTrials.gov, NCT05623592. Registered on 21 November 2022.EU Clinical Trials Register, 2022-501058-12-00.German Clinical Trials Register DRKS00030571.

Challenges and perspectives for the treatment of giant cell arteritis

Early diagnosis and prompt initiation of treatment are crucial to avoid severe complications in giant cell arteritis (GCA). The European Alliance of Associations for Rheumatology (EULAR) recommendations for the use of imaging in large vessel vasculitis have helped better define the role of different techniques for diagnosing and monitoring the disease. Regarding the treatment, corticosteroids remain the standard, and tocilizumab is the preferred corticosteroid-sparing treatment. New corticosteroid-sparing treatments and "ultra-light" corticosteroid usage regimens are also under study and could represent valid therapeutic alternatives in the future.

Recent Developments of USG Score for GCA

The 2022 European League Against Rheumatism (EULAR) guidelines have introduced a significant advancement in the diagnostic approach to Giant Cell Arteritis (GCA). According to these updated guidelines, Ultrasound (US) examinations of the cranial and axillary arteries should be considered the primary imaging method for evaluating patients who are suspected to have GCA, provided that the examinations are performed by adequately trained specialists using high-quality equipment. This recommendation marks a notable shift in the traditional management practices for GCA, as it acknowledges the effectiveness of ultrasound as a non-invasive, efficient, and reliable point-of-care test for individuals with suspected GCA. As a result, this development has paved the way for the establishment of fast-track clinics dedicated to GCA diagnosis and treatment across various parts of the globe. However, it is important to note that the use of ultrasound for diagnosing GCA comes with certain challenges. The highly technical nature of the ultrasound procedure, combined with its dependence on the expertise of the operator, often leads to criticism regarding its reliability as a clinical tool. This can create confusion among clinicians when they attempt to interpret formal ultrasound reports, especially if they are not trained in sonography themselves. In this presentation, we will thoroughly examine the evidence supporting the use of ultrasonography in the diagnosis of Giant Cell Arteritis. The discussion will encompass several key concepts, including: 1. The validity of ultrasonography as a diagnostic tool for GCA. 2. The diagnostic performance of ultrasound in identifying the condition. 3. The practical utility of ultrasound in an Australian Fast Track Clinic setting. 4. The assessment of treatment response and sensitivity to change, as measured by ultrasound findings. 5. The various scoring tools utilized in ultrasound evaluations, such as the Halo Count, the Halo Score, and the OMERACT GCA Ultrasound Score. 6. A review of the EULAR guidelines and how they have evolved over time. 7. Critical information that clinicians who are not trained in sonography need to understand regarding the use of ultrasound in GCA. Through this comprehensive review, we aim to clarify the role of ultrasonography in the diagnosis and management of Giant Cell Arteritis, empowering clinicians with the knowledge they need to effectively utilize this diagnostic tool in their practice.

Association of clinical, imaging and laboratory parameters with adverse effects of glucocorticoid therapy in patients with giant cell arteritis.

Giant cell arteritis (GCA) is characterized by inflammation of large and medium vessels. First-line therapy for the treatment of GCA are glucocorticoids, which are effective while potential adverse effects should be considered, especially during long-term use. The aim was to investigate the incidence of glucocorticoids' adverse effects and potential predictors for them. 138 GCA patients were retrospectively evaluated for newly developed glucocorticoid adverse effects in 2020. Potential predictors, defined as initial glucocorticoid pulse therapy, relapse of GCA and concomitant polymyalgia rheumatica as well as parameters of inflammation and endothelial dysfunction, including pulse-wave velocity and intima-media-thickness, were measured in 2012. Potential new glucocorticoid adverse effects per patient was 1 (25th-75th 0-3) of which chronic kidney disease progression (29%), bone fractures (23.2%), cataracts (18.1%), dementia, and arterial hypertension (each at 12.3%) were most commonly recorded. Significant associations were found between occurrence of any relapse and new diabetes mellitus and between initial glucocorticoid pulse therapy and new dementia (all with p < 0.05). In multivariate regression analysis, any relapse was a predictor for developing diabetes mellitus (OR 9.23 [95% CI 1.33-64.05], p = 0.025). However, no correlations were observed between endothelial dysfunction or inflammatory parameters and development of new glucocorticoid adverse effects. GCA relapses may be associated for development of diabetes mellitus potentially by increasing glucocorticoid doses. Parameters of inflammation and endothelial dysfunction are not suited predictors for glucocorticoid adverse effects.

Diagnosis, Treatment, and Follow-Up of Giant-Cell Arteritis: A Retrospective Multicenter Study.

Giant-cell arteritis (GCA) is the most common type of vasculitis in the elderly and is associated with high risks of visual loss and recurrence. Owing to its rarity in Asian populations, the current clinical interventions for these patients are not well known. Here we aimed to characterize the current management status of patients with GCA using Korean multicenter data. This retrospective study analyzed medical records of patients with GCA at six Korean university hospitals from February 2009 to November 2022. GCA had originally been diagnosed based on the 1990 American College of Rheumatology (ACR) criteria, and cases were selected for inclusion in this study based on the 2022 ACR/European Alliance of Associations for Rheumatology criteria. We evaluated treatments, follow-up periods, and outcomes (relapse, remission, and adverse drug reactions) in patients with GCA with or without arteritic anterior ischemic optic neuropathy (AAION). This study analyzed 18 patients with a median age of 75.5 years that included 12 females (66.7%). Seven patients (38.8%) had AAION. All patients initially received prednisolone treatment, while four (22.2%) underwent adjuvant treatment with methotrexate and azathioprine during prednisolone tapering. During the median follow-up of 3.5 months (interquartile range: 2.0-23.2 months), 4 patients (22.2%) had prednisolone-related adverse reactions, 2 (11.1%) relapsed, and 13 (72.3%) dropped out. Nine patients (50.0%) experienced remission, with this being sustained in four (36.4%). This study observed high dropout rates and short follow-ups. Adverse effects of prednisolone were common, and relapses occurred in approximately one-tenth of Korean patients with GCA. Thus, optimizing GCA treatment necessitates regular monitoring and long-term follow-up.

Treatment for giant cell arteritis with 8 weeks of prednisone in combination with tocilizumab: a single-arm, open-label, proof-of-concept study

Even after the approval of tocilizumab, substantial glucocorticoid exposure (usually ≥6 months) and toxicity continue to be important problems for patients with giant cell arteritis. We aimed to assess the outcomes of a group of patients with giant cell arteritis treated with tocilizumab in combination with 8 weeks of prednisone. This prospective, single arm, proof-of-concept study was conducted at Massachusetts General Hospital (Boston, MA, USA). Individuals aged 50 years or older who had new-onset or relapsing giant cell arteritis with active disease were eligible for inclusion. Participants received 12 months of tocilizumab 162 mg weekly subcutaneously in combination with 8 weeks of prednisone. The primary endpoint was sustained prednisone-free remission at week 52. Adverse events were also evaluated. This trial is registered with ClinicalTrials.gov (NCT03726749), and is complete. Between Nov 28, 2018, and Nov 2, 2020, we enrolled 30 patients (mean age 73·7 years [SD 8·1], 18 [60%] women and 12 [40%] men, 30 [100%] White race, 15 [50%] new-onset disease, 23 [77%] temporal artery biopsy-proven, 14 [47%] imaging-proven). The initial prednisone doses were 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 from baseline. 23 (77%) of 30 patients were in sustained prednisone-free remission at week 52 and seven (23%) patients relapsed, with a mean time to relapse of 15·8 weeks (SD 14·7). Overall, four (13%) participants developed a serious adverse event, including one related or probably related to prednisone exclusively, two related or probably related to tocilizumab exclusively, and one related or probably related to prednisone, tocilizumab, or both. Two of the non-responder patients stopped tocilizumab and withdrew from the study prematurely after having a second disease relapse. No cases of giant cell arteritis-related permanent vision loss occurred during the study. These results suggest that 12 months of tocilizumab in combination with 8 weeks of prednisone could induce and maintain remission in patients with giant cell arteritis. Confirmation of these findings in a randomised controlled trial is required. Genentech.

Giant cell arteritis in patients with systemic sclerosis: a case series.

Giant cell arteritis (GCA) in patients with systemic sclerosis (SSc) is rare, and optimal treatment strategies for this group of patients have not been defined. We aim to describe the first case series of GCA/SSc overlap. A single-institution retrospective study was performed reviewing all patients that had diagnosis codes for both SSc and GCA between January 1, 1996, and December 31, 2020. Demographic characteristic, clinical presentation, diagnostic modality, treatment, and outcome data were abstracted. Diagnosis of both SSc and GCA by a rheumatologist was required for inclusion. Eight patients were retrospectively identified, all of which were female. Seven patients fully met both respective ACR/EULAR classification criteria sets. One patient fulfilled GCA criteria and had 8/9 points for SSc criteria plus an oesophagogram which was consistent with clinical diagnosis of SSc. Three patients had a previous history of scleroderma renal crisis (SRC) prior to glucocorticoid initiation for GCA. No episodes of SRC occurred following initiation of glucocorticoids. Three patients were treated with tocilizumab. One patient developed a diverticular perforation while on tocilizumab requiring colonic resection and colostomy, one patient discontinued tocilizumab after a medication-unrelated complication and one patient has remained on treatment and in remission. Herein we present the largest single-institution series of patients with a history of GCA and SSc, an uncommon combination. Glucocorticoid treatment for GCA did not precipitate SRC, even in those with prior history of SRC. Further investigation regarding the benefit of tocilizumab in patients with SSc and GCA is required.