and C-reactive protein' and "Acute phase reactants", section on 'Clinical use'.) Imaging surveillance for patients with large vessel involvement Different imaging modalities (CT, CT angiography [CTA], magnetic resonance imaging [MRI], MR angiography [MRA], and ultrasound) may be used for long-term monitoring of structural damage, particularly to detect stenosis, occlusion, dilatation, and/or aneurysms. The frequency of surveillance, as well as the imaging method applied, should be decided on an individual basis. Repeat imaging of a https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 11/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate newly discovered or enlarging aortic aneurysm should be considered at six months of follow-up; if stable, the interval between imaging studies can be lengthened to an annual basis. The choice of an imaging modality will vary by institution and individual patient (see "Diagnosis of giant cell arteritis", section on 'Imaging modalities'). There are not enough data to recommend the routine use of any of the different imaging modalities for monitoring disease activity during follow-up of patients with large vessel GCA. As an example, in a large retrospective cohort study of patients diagnosed with large vessel GCA, positron emission tomography (PET)/CT grading showed moderate accuracy in distinguishing between clinically active and inactive disease, with a sensitivity of 51 and specificity of 83 percent [41]. Furthermore, PET/CT was not able to predict subsequent relapse. However, in selected cases in which a flare is suspected, imaging might be helpful to confirm or exclude it. Prevention of treatment-associated toxicity Immunosuppressive therapy with high-dose glucocorticoids with or without a glucocorticoid-sparing agent has both infectious and noninfectious toxicities that warrant additional prophylactic measures. The specific regimens are discussed in detail separately: Prevention of opportunistic infections Reports of Pneumocystis jirovecii pneumonia (PCP) in GCA are rare [42,43]. In a retrospective cohort study of 1168 patients with GCA, no cases of PCP were identified over 547 patient-years of follow-up [44]. However, prednisone use at 16 mg daily or more for eight weeks has been shown to be associated with an increased risk of PCP [45], and to date, the risk of PCP in patients on prednisone and tocilizumab is unknown. While the absolute risk of PCP in GCA is small, some experts recommend PCP prophylaxis for patients with GCA given the risk of morbidity and mortality associated with PCP pneumonia. Ultimately, the decision to prescribe PCP prophylaxis should be individualized based on patient risk factors and preference and following a careful discussion of risks and benefit. (See "Treatment and prevention of Pneumocystis pneumonia in patients without HIV", section on 'Indications'.) Osteoporosis prevention Because of the length of the course of glucocorticoid treatment for GCA, osteoporosis prevention should be aggressively pursued at the start of therapy. Adequate dietary calcium and vitamin D intake should be encouraged. Determination of bone mineral density near the time that treatment is begun is essential for guiding management of bone loss. (See "Prevention and treatment of glucocorticoid- induced osteoporosis".) Age-appropriate vaccinations Age-appropriate vaccinations should be up to date in immunosuppressed patients. (See "Immunizations in autoimmune inflammatory rheumatic disease in adults".) https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 12/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate Screening for latent tuberculosis Screening tests of latent tuberculosis should be performed at the start of therapy. (See "Tuberculosis infection (latent tuberculosis) in adults: Approach to diagnosis (screening)".) DISEASE RELAPSE Incidence and clinical features Reports on the incidence of relapses in giant cell arteritis (GCA) vary widely, from 34 to 74 percent [46-50]. This range is due in part to a lack of consensus regarding the definition of what constitutes a relapse. In some analyses, asymptomatic rises in the acute phase reactants were counted as a flare of GCA and in others, a recrudescence of symptoms only, unaccompanied by rises in the acute phase reactants, was included. All studies construed the appearance of polymyalgia rheumatica (PMR) as a relapse of GCA. Late relapses (and recurrences) of GCA are described, leading to protracted glucocorticoid treatment. In one study, one-half of patients were still on treatment after five years [49]. Other estimates of the total duration of glucocorticoid treatment are in the range of one to two years [51,52], which is more consonant with clinical practice. The majority of relapses in GCA occur at doses of prednisone below 20 mg/day and are most prevalent during the first year of treatment. Headache and PMR are the most common symptomatic expressions of relapse. Other symptoms include jaw claudication, the development of ischemic limb symptoms, and the recurrence of constitutional symptoms. Elevations of the erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) do not always indicate a disease flare, but their occurrence should trigger close clinical follow-up and questioning of patients about symptoms of recurring disease. In addition, if elevations in ESR and CRP are accompanied by constitutional symptoms, diagnostic imaging for underlying large vessel vasculitis may be considered. The occurrence of sight loss after an initial course of high-dose glucocorticoid treatment, administered daily, is exceptional. Management Increase glucocorticoid dose For patients who experience a relapse of GCA, we increase the glucocorticoid dose appropriate to the nature of the relapse. For visual symptoms attributable to GCA (generally occurring within the first weeks of the initial diagnosis of disease), an increase of the prednisone dose to 40 to 60 mg/day and even pulse doses may be needed. For other symptoms, an increase of the daily dose of glucocorticoids to the last effective dose or 5 to 15 mg above this dose is appropriate. Smaller increments in the daily dose of prednisone, between 5 to 7.5 mg/day, are usually appropriate for PMR symptoms. https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 13/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate Addition of a glucocorticoid-sparing agent For patients who are not already on a glucocorticoid-sparing agent at the time of relapse, a glucocorticoid-sparing agent, preferably tocilizumab, should be added. The rationale for this approach is based on data from the clinical trials of tocilizumab for GCA that included patients with relapsing disease. These trials are discussed in detail above. (See 'Tocilizumab' above.) For patients with relapsing disease while already on tocilizumab, in addition to increasing the glucocorticoid dose, some experts may modify the tocilizumab dose or route of administration when possible. This approach is based on clinical experience, as there are no data to support this approach. As an example, if a patient relapses while on tocilizumab subcutaneous injections every other week, the dosing frequency may be increased to weekly injections. As another example, if a patient relapses while receiving tocilizumab 6 mg/kg intravenously once every four weeks, they may have their dose increased to 8 mg/kg once every four weeks. Additionally, switching from subcutaneous administration to intravenous administration may be attempted. Entry into an investigational trial may also be an option at some institutions for patients with relapsing disease while already on tocilizumab. UNPROVEN OR INEFFECTIVE AGENTS Several other glucocorticoid-sparing agents have been reported as having efficacy as adjunctive treatment for giant cell arteritis (GCA), but their use cannot be endorsed because of small effect, potential toxicity, lack of controls, or small numbers of patients studied. Abatacept A trial of abatacept, a blocker of T-cell costimulation, was proposed on the basis of the presence of activated CD4+ T cells in the typical inflammatory infiltrate of the temporal artery in GCA [53] (see "Pathogenesis of giant cell arteritis"). In a phase 2 randomized, double-blind study of patients with newly diagnosed or relapsing GCA, 49 patients were enrolled and treated with prednisone and intravenous abatacept, administered on days 1, 15, 29, and 56. At week 12, 41 patients were in remission and were randomized to continue treatment with monthly abatacept or placebo. Prednisone was tapered by a standardized schedule and discontinued by week 28. The rate of sustained remission at 12 months in patients treated with abatacept compared with placebo was of borderline statistical significance, 48 versus 31 percent. There was no difference in adverse events, including infection, between the two groups. Further study is needed to determine a possible role for abatacept as adjunctive treatment for GCA. https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 14/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate Baricitinib Preclinical findings and case reports have demonstrated the biologic plausibility that agents selectively targeting Janus kinase (JAK) 1/JAK2 hold potential promise in GCA. In a prospective, open-label, proof-of-concept study with a tiered glucocorticoid entry and accelerated taper in patients with relapsing GCA, the oral selective JAK1/JAK2 inhibitor baricitinib at 4 mg/day was well tolerated and allowed discontinuation of glucocorticoids in most patients [54]. Larger randomized clinical trials are needed to determine the safety and efficacy of JAK inhibition in GCA. Mavrilimumab Preclinical research has implicated granulocyte-macrophage colony- stimulating factor (GM-CSF) in the pathogenesis of GCA. Mavrilimumab, an immunoglobulin (Ig) G4 monoclonal antibody, blocks GM-CSF signaling by binding to the alpha chain of the receptor. In a phase 2 trial enrolling patients with GCA, mavrilimumab plus 26 weeks of prednisone was superior to placebo for time to flare by week 26 and sustained remission [55]. Further studies are needed to define the potential role of mavrilimumab in the treatment of GCA. Azathioprine In a study from a single center of 31 patients with GCA, polymyalgia rheumatica (PMR), or both, a double-blind randomized controlled study of azathioprine, 150 mg/day, versus placebo showed a small but statistically significant reduction in mean prednisolone dose at 52 weeks (1.9 mg/day 0.84 versus 4.2 mg/day 0.58) [56]. Only 20 patients completed the study. Ustekinumab T helper (Th) 1 and Th17 cells are believed to play key roles in the pathogenesis of GCA [57] (see "Pathogenesis of giant cell arteritis"). Ustekinumab blocks interleukin (IL) 12, a Th1-promoting cytokine, and IL-23, a Th17-promoting cytokine, providing a theoretical basis for its use in the treatment of GCA. In an open-label study of ustekinumab in 14 patients with refractory GCA, the prednisolone dose was decreased from a median of 20 mg/day to 5 mg/day; four patients discontinued glucocorticoid therapy entirely [58]. However, a subsequent prospective, open-label trial of ustekinumab in patients with active new-onset or relapsing GCA was closed prematurely after 7 of the initial 10 patients relapsed [59]. Cyclophosphamide Cyclophosphamide has been widely used in the treatment of systemic vasculitis. A few small, uncontrolled studies have suggested that it may be useful in GCA in patients at high risk of glucocorticoid-related adverse effects who have not responded adequately to other immunosuppressive or immunomodulatory glucocorticoid- sparing treatments [60-62]. A systematic review identified 103 published cases for analysis [62]. The major indications for cyclophosphamide, administered either orally or intravenously, included glucocorticoid dependency or relapsing disease. Most of the https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 15/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate reported patients (86 percent) responded, but 22 percent relapsed despite maintenance immunosuppressive therapy. Adverse effects were described in one-third of the patients, and 12.5 percent discontinued the therapy because of infections and cytopenias. One death due to hepatitis was reported. Others Small, uncontrolled, retrospective series have proposed benefit for dapsone [63], leflunomide [64], and IL-1 blockade [65] for the management of GCA. Lack of benefit of anti-TNF therapy Because GCA is characterized by granulomatous inflammation, tumor necrosis factor (TNF) inhibition would appear to be an appropriate approach to treatment. However, several small randomized trials of TNF inhibition have found that infliximab, etanercept, and adalimumab are ineffective in patients with GCA [66- 68]. As an example, 44 patients were studied in a multicenter, randomized, placebo- controlled trial of infliximab for the maintenance of remission [66]. After prednisone- induced remission, patients were randomly assigned in a 2:1 ratio to infliximab 5 mg/kg or placebo. An interim analysis at week 22 demonstrated that infliximab did not reduce the proportion of patients with relapses (43 versus 50 percent on placebo). In addition, infliximab did not increase the proportion of patients whose prednisone dose could be tapered to 10 mg/day without relapse (61 versus 75 percent). Consequently, the trial was stopped early. Through the follow-up period, no differences between the treatment groups were observed in the proportion of relapse-free patients, the cumulative dose of prednisone, or the incidence of adverse events. PROGNOSIS Giant cell arteritis (GCA) is a disease of variable duration. In some, it may have a course of one to two years, while in many others, the disease is more chronic. The glucocorticoid dose can eventually be reduced and discontinued in the majority of patients, although some patients require low doses of prednisone for a number of years to control symptoms. GCA does not adversely affect overall survival, excepting the subset of patients with aortic involvement and dissection [69-71]. SOCIETY GUIDELINE LINKS Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Giant cell arteritis and polymyalgia rheumatica".) https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 16/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate INFORMATION FOR PATIENTS UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." th th The Basics patient education pieces are written in plain language, at the 5 to 6 grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more th th sophisticated, and more detailed. These articles are written at the 10 to 12 grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon. Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.) Basics topic (see "Patient education: Polymyalgia rheumatica and giant cell arteritis (The Basics)") Beyond the Basics topics (see "Patient education: Vasculitis (Beyond the Basics)" and "Patient education: Polymyalgia rheumatica and giant cell arteritis (Beyond the Basics)") SUMMARY AND RECOMMENDATIONS Initial management Systemic glucocorticoids For all patients with giant cell arteritis (GCA), we recommend initial treatment with high-dose systemic glucocorticoids to preserve vision (Grade 1C), as well as to treat other clinical symptoms associated with GCA (Grade 2B). Treatment should be initiated promptly once the diagnosis is confirmed or there is a high index of suspicion for GCA. Our practice regarding the specific dose and route of administration of glucocorticoids for newly diagnosed GCA is as follows (see 'Glucocorticoids' above): No visual loss at diagnosis Prednisone 40 to 60 mg/day or equivalent, given in a single daily dose. Threatened or established visual loss at diagnosis Methylprednisolone 500 to 1000 mg intravenous daily, for three days (followed by 40 to 60 mg/day of oral prednisone). https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 17/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate Glucocorticoid-sparing agent in selected patients For patients with newly diagnosed GCA with an increased risk of glucocorticoid-related adverse effects, we suggest the addition of tocilizumab to glucocorticoid therapy (Grade 2B). Patients who are at increased risk of developing glucocorticoid-related side effects or complications include those with osteoporosis, diabetes, cardiovascular disease, or glaucoma. Tocilizumab has demonstrated a glucocorticoid-sparing effect in clinical trials. (See 'Glucocorticoid-sparing agent in selected patients' above and 'Indications and rationale' above and 'Tocilizumab' above.) Whether a glucocorticoid-sparing agent should be used in the routine care of all patients with GCA is uncertain. The American College of Rheumatology (ACR) recommends the use of adjunctive tocilizumab for all patients with GCA. Methotrexate may be used as an alternative to tocilizumab for patients who are unable to use the medication due to factors such as availability, cost, recurrent infections, or a history of gastrointestinal perforations or diverticulitis; however, limited available data and clinical experience suggest methotrexate is less effective. (See 'Methotrexate' above.) Patients with large vessel involvement Patients with large vessel GCA are managed in a manner similar to those without large vessel involvement. Additional considerations include antiplatelet therapy in selected patients as well as possible surgical interventions for patients with vascular complications. (See 'Patients with large vessel involvement' above.) Subsequent management Glucocorticoid tapering Symptoms and signs of GCA usually respond quickly to glucocorticoids, permitting the initiation of a taper after one to two weeks. The approach to glucocorticoid tapering varies generally depends on whether patients are also receiving tocilizumab. (See 'Glucocorticoid tapering' above.) Patients on glucocorticoid monotherapy For patients with GCA receiving monotherapy with glucocorticoids, we use a standard glucocorticoid tapering regimen ( table 1). Patients on glucocorticoids plus tocilizumab For most patients with GCA receiving glucocorticoids in combination with tocilizumab (or another glucocorticoid-sparing agent), we use an accelerated glucocorticoid-tapering regimen ( table 2). https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 18/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate Routine monitoring of disease activity Monitoring of disease activity requires careful clinical follow-up to evaluate the patient's response to therapy and assess for toxicity of the treatment regimen. For patients who are not on tocilizumab, the erythrocyte sedimentation rate (ESR) and the C-reactive protein (CRP) can be useful adjuncts to clinical decision making. Tocilizumab completely normalizes the ESR and CRP levels, and they are thus not useful for monitoring disease activity in such patients. Clinicians must rely on clinical evaluation and, in the case of large vessel involvement, periodic imaging studies for patients on tocilizumab. (See 'Routine monitoring of disease activity' above.) Imaging surveillance for patients with large vessel involvement Different imaging modalities (CT, CT angiography [CTA], MRI, MR angiography [MRA], and ultrasound) may be used for long-term monitoring of structural damage, particularly to detect stenosis, occlusion, dilatation, and/or aneurysms. The frequency of surveillance, as well as the imaging method applied, should be decided on an individual basis. (See 'Imaging surveillance for patients with large vessel involvement' above.) Prevention of treatment-associated toxicity Immunosuppressive therapy with high- dose glucocorticoids with or without a glucocorticoid-sparing agent has both infectious and noninfectious toxicities that warrant additional prophylactic measures including prevention of opportunistic infections, osteoporosis prevention, age-appropriate vaccinations, and screening for latent tuberculosis. (See 'Prevention of treatment- associated toxicity' above.) Disease relapse Incidence and clinical features Relapses of disease activity are most common at prednisone doses less than 20 mg/day and are most prevalent during the first year of treatment. Headache and polymyalgia rheumatica (PMR) are the most common symptomatic expressions of relapse. Relapses usually do not result in major adverse events such as visual loss. (See 'Incidence and clinical features' above.) Management of relapse For patients who experience a relapse of GCA, we increase the glucocorticoid dose appropriate to the nature of the relapse. For patients who are not already on a glucocorticoid-sparing agent at the time of relapse, we suggest adding tocilizumab (Grade 2B). (See 'Management' above.) https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 19/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate Prognosis GCA is a disease of variable duration. Length of treatment may extend from one to multiple years. Glucocorticoid treatment can eventually be discontinued in the majority of patients. (See 'Prognosis' above.) ACKNOWLEDGMENTS The UpToDate editorial staff acknowledges Gene G Hunder, MD and William P Docken, MD, who contributed to earlier versions of this topic review. Use of UpToDate is subject to the Terms of Use. REFERENCES 1. Crowson CS, Matteson EL, Myasoedova E, et al. 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Topic 8240 Version 40.0 https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 25/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate GRAPHICS Narrowing of the left subclavian artery in giant cell arteritis Angiogram of the left subclavian artery in GCA, showing several areas of severe vascular narrowing but a proliferation of small blood vessels forming excellent collateral circulation. The long, smooth arterial tapering in large vessel GCA is often not amenable to revascularization interventions such as angioplasty or stent placement. Those types of interventions are rarely necessary. GCA: giant cell arteritis. Courtesy of John H Stone, MD, MPH. Graphic 73553 Version 8.0 https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 26/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate 52-week glucocorticoid tapering regimen for giant cell arteritis Taper week Dose (mg/day) 1 60 2 50 3 40 4 35 5 30 6 25 7 20 8 17.5 9 17.5 10 15 11 15 12 12.5 13 10 14 10 15 10 16 10 17 9 18 9 19 9 20 9 21 8 22 8 23 8 24 8 25 7 26 7 27 7 28 7 29 6 https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 27/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate 30 6 31 6 32 6 33 5 34 5 35 5 36 5 37 4 38 4 39 4 40 4 41 3 42 3 43 3 44 3 45 2 46 2 47 2 48 2 49 1 50 1 51 1 52 1 Tapering doses listed refer to oral prednisone (or equivalent) typically administered once daily in the morning. Data from: Stone JH, Tuckwell K, Dimonaco S, et al. Trial of tocilizumab in giant-cell arteritis. New Engl J Med 2017; 377:317. Graphic 139301 Version 2.0 https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 28/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate Accelerated glucocorticoid tapering regimen for patients with giant cell arteritis on tocilizumab Taper week Dose (mg/day) 1 60 2 50 3 40 4 35 5 30 6 25 7 20 8 15 9 12.5 10 12.5 11 10 12 9 13 8 14 7 15 6 16 6 17 5 18 5 19 4 20 4 21 3 22 3 23 2 24 2 25 1 26 1 Tapering doses listed refer to oral prednisone (or equivalent) typically administered once daily in the morning. https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 29/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate Data from: Stone JH, Tuckwell K, Dimonaco S, et al. Trial of tocilizumab in giant-cell arteritis. New Engl J Med 2017; 377:317. Graphic 139298 Version 2.0 https://www.uptodate.com/contents/treatment-of-giant-cell-arteritis/print 30/31 7/7/23, 11:22 AM Treatment of giant cell arteritis - UpToDate Contributor Disclosures Carlo Salvarani, MD No relevant financial relationship(s) with ineligible companies to disclose. Francesco Muratore, MD No relevant financial relationship(s) with ineligible companies to disclose. Jonathan Trobe, MD No relevant financial relationship(s) with ineligible companies to disclose. Kenneth J Warrington, MD Grant/Research/Clinical Trial Support: Eli Lilly [Giant cell arteritis]; GSK [Giant cell arteritis]; Kiniksa [Giant cell arteritis]. Other Financial Interest: Chemocentryx [Honoraria ANCA-associated vasculitis]. All of the relevant financial relationships listed have been mitigated. Philip Seo, MD, MHS No relevant financial relationship(s) with ineligible companies to disclose.