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Large-vessel vasculitis

Large-vessel vasculitis is inflammation of the wall of the aorta and of its branches. Takayasu arteritis affects mainly young women, giant cell arteritis people over the age of fifty. Since 2012 Arsène Mekinian has devoted to it a series of multicentre studies on biological treatments, prognosis and pregnancy.

What is large-vessel vasculitis?

The aorta and the large arteries that arise from it, those that supply the head, the arms, the kidneys and the intestine, can be the site of inflammation of their wall. This wall thickens, narrows, sometimes dilates. Two main diseases are grouped under the name of large-vessel vasculitis. Takayasu arteritis occurs mainly in young women and readily affects the aorta and its branches. Giant cell arteritis, also called Horton's disease, occurs after the age of fifty, often affects the branches of the external carotid arteries and can threaten sight.

There are two challenges. The disease has to be recognised before the artery is permanently narrowed, and it then has to be possible to know whether the inflammation is still active or whether what is being looked at is a fixed lesion. This second question remains difficult, because the blood markers of inflammation and the images of the artery do not always say the same thing. Corticosteroids bring the inflammation down quickly, but many patients relapse as soon as the dose is reduced, and prolonged exposure to corticosteroids has its own consequences. It is this impasse that has motivated the use of biological treatments, whose effect the works below measure.

His work on this subject

Biologics in Takayasu arteritis, from the small series to the series of 209 patients. The first work he led on this subject is a French multicentre retrospective study of 15 patients with refractory Takayasu arteritis treated with infliximab. A partial or good response was noted in 13 of the 15 patients at 3 months, 10 of 13 at 6 months and 8 of 11 at 12 months; the median corticosteroid dose fell from 20 to 6 mg per day at 12 months (p < 0.05); only one patient remained steroid-dependent at 12 months, against 8 before infliximab. Adverse events included one pulmonary tuberculosis and one severe bacterial infection (Rheumatology (Oxford), 2012, first author). The next study, published in Circulation in 2015, covered 49 patients treated with anti-TNF (80%) or tocilizumab (20%), 88% of whom had failed or were intolerant of conventional immunosuppressants: the overall response reached 75% at 6 months and 83% at 12 months, relapse-free survival at 3 years was 90.9% with biologics against 58.7% with conventional disease-modifying treatments (p = 0.0025), and no difference in efficacy was observed between anti-TNF and tocilizumab; 21% of patients had an adverse event and treatment was stopped in 6.6% of cases. The question was taken up again in 2022 in 209 patients (132 on anti-TNF, 77 on tocilizumab): the complete response at 6 months was 66% with anti-TNF and 70% with tocilizumab, 103 relapses occurred over a median follow-up of 36 months, and adverse events were comparable, 21% against 17% (p = 0.4). The study concludes that efficacy, relapse risk and treatment retention are equivalent between the two classes (Rheumatology (Oxford), 2022, first author of 54 named authors).

How to give these treatments: first line, route of administration, molecule. Three works address the modalities rather than the principle. The TOCITAKA trial, prospective, multicentre and open-label, assessed tocilizumab as first-line treatment in patients never treated before: 13 patients were included, of median age 32 years, 12 of them women; the primary endpoint, withdrawal of corticosteroids after 7 infusions, was met in 6 patients (54%); activity scores fell significantly at 6 months (NIH score from 3 to 1, p < 0.001); 11 of the 13 patients were in remission at 6 months, but 5 of them (45%) relapsed within the 12 months following withdrawal of tocilizumab. The authors conclude that maintenance treatment is needed (Arthritis Research & Therapy, 2020, first author; the declared principal investigator of the trial is Olivier Fain, Arsène Mekinian is first author of the article reporting the results). A retrospective study of 109 patients compared intravenous tocilizumab (91 patients) with subcutaneous tocilizumab (18 patients): complete response at 6 months of 70% and 69% respectively (p = 0.95), but cumulative incidence of relapse at 12 months of 10.3% with the intravenous route against 30.9% with the subcutaneous route, with a higher risk of relapse with the subcutaneous form (HR 2.55; p = 0.033) (RMD Open, 2023, first author of 57 named authors). The same comparative work was carried out on the anti-TNF side in 135 patients, 101 on intravenous infliximab and 34 on subcutaneous adalimumab: complete response at 6 months of 71% overall, 73% with infliximab and 68% with adalimumab (p = 0.8), risk of relapse at 12 months of 10.9% and 16.8%, with no significant difference in revascularisation (Rheumatology (Oxford), 2025, first author of 58 named authors).

What remains when biologics fail. Two recent works concern salvage treatments, both published as letters and not as original articles. The first concerns rituximab in Takayasu arteritis, a multicentre retrospective study for which PubMed carries no abstract (Joint Bone Spine, 2024, first author of 13 named authors). The second reports, from the BIOTAK registry, the experience of 20 patients with refractory Takayasu arteritis treated with a JAK inhibitor between January 2017 and September 2020 in referral centres in France, Italy, Spain, Armenia, Israel, Japan, Tunisia and Russia: tofacitinib in 10 patients, upadacitinib in 5, baricitinib in 4, ruxolitinib in 1; all had previously received at least three lines of immunosuppressants. A complete response at 6 months was obtained in 9 of the 18 evaluable patients (50%), the median prednisone dose falling from 13.8 to 8 mg per day and the median CRP from 18 to 4 mg/L (p < 0.05). The cumulative incidence of relapse at 24 months was 18% and two treatments were stopped for an adverse event, one thrombosis and one infection (RMD Open, 2026, first author of 22 named authors).

The prognosis of Takayasu arteritis, established on cohorts he co-signed. The central reference is a French multicentre retrospective study of 318 patients meeting the criteria of the American College of Rheumatology and of Ishikawa, published in Circulation on 19 September 2017, with online publication on 12 July 2017. Median age at diagnosis was 36 years and 276 patients (86.8%) were women. After a median follow-up of 6.1 years, 43% of the patients had relapsed, 38% had had a vascular complication and 5% had died; event-free survival was 48.2% at 5 years and 36.4% at 10 years. A progressive course from the outset and carotidynia were independently associated with event-free survival. Arsène Mekinian is 4th of 16 named authors, alongside the French Takayasu Network consortium. The same cohort of 318 patients served for the mortality analysis: 16 deaths (5%), mainly from mesenteric ischaemia (4 cases) and rupture of an aortic aneurysm (4 cases), with mortality of 1.9% at 5 years and 3.9% at 10 years and a standardised mortality ratio of 2.73 [1.69-4.22] compared with matched controls (Journal of Autoimmunity, 2019, 5th of 17). Of 320 patients, 63 (20%) had a stroke (41 cases) or a transient ischaemic attack (22 cases), in the carotid territory in 87% of them; a delay of more than one year between the first symptoms and the diagnosis was independently associated with these events (HR 2.16 [1.27-3.70]; p = 0.007) (Stroke, 2022, 10th of 18). More recently, a cluster analysis conducted on an international cohort of 852 patients identified three profiles, termed supra-aortic, abdominal and diffuse, of differing prognosis: the abdominal profile carried the highest risk of vascular complications (HR 1.79 [1.12-2.87]) and the diffuse profile the highest risk of relapse (HR 2.31 [1.81-2.95]) (Journal of Autoimmunity, 2026, 7th of 26).

Giant cell arteritis and the shared large-vessel field. A multicentre retrospective study of 417 patients with large-vessel involvement, 299 of them Takayasu arteritis and 118 giant cell arteritis, showed that the two diseases do not progress in the same way: aneurysm-free survival at 10 years was 67% in giant cell arteritis against 89% in Takayasu arteritis (p = 0.02), relapse-free survival at 5 years 47% against 69% (p < 0.001), while revascularisation-free survival at 10 years was lower in Takayasu arteritis, 55% against 76% (p < 0.001) (Journal of Autoimmunity, 2020, 6th of 12). On the giant cell arteritis side, a study of 129 patients diagnosed between September 2010 and October 2018 in two university hospitals found an ischaemic cerebral event in 18 patients (16%), more frequent in men and in older patients, and associated with markedly shorter overall survival and relapse-free survival (Journal of Autoimmunity, 2019, last author of 12). A cluster analysis of 283 patients with giant cell arteritis separated a relapsing vascular profile (23.0%), a classical profile (47.7%) and an ophthalmological profile in the very elderly (29.3%), the last of these carrying the highest mortality (QJM, 2024, 6th of 10). A national cohort study on the French national health data system (Système national des données de santé), covering 1,997 patients admitted to hospital for incident giant cell arteritis between 1 January 2012 and 31 December 2024 and who received tocilizumab (1,095 patients) or methotrexate (902 patients) in the six months following discharge, observed at two years a cumulative incidence of major cardiovascular events of 6.4% with tocilizumab against 10.7% with methotrexate, that is a hazard ratio of 0.60 [0.48-0.75] (Circulation, published online on 29 August 2026, 8th of 9). On the diagnostic side, he is last author of a systematic review with meta-analysis of 21 studies (413 patients, 299 controls) which measured the performance of FDG-PET in large-vessel vasculitis, with a pooled sensitivity of 90% and a pooled specificity of 98% for large-vessel inflammation in giant cell arteritis, and a sensitivity of 87% and a specificity of 73% for the assessment of activity in Takayasu arteritis (Medicine (Baltimore), 2015); he is first author of a review in French on the place of imaging in these diseases (La Revue de médecine interne, 2016). He is finally among the 21 named authors of the French national protocol for the diagnosis and management of giant cell arteritis and of the recommendations of the French Study Group for Large Vessel Vasculitis on the use of immunosuppressants and biologics in this disease (La Revue de médecine interne, 2025, 14th of 21 in both texts).

Pregnancy and Takayasu arteritis. As Takayasu arteritis affects women of childbearing age, a retrospective study conducted in 20 French hospitals up to August 2015 analysed 43 pregnancies in 33 women, 29 of whom already had a diagnosis of Takayasu arteritis and 4 of whom were diagnosed during pregnancy. A complication occurred during 20 pregnancies (47%): hypertension in 35% of cases (15 pregnancies), pre-eclampsia in 9% (4), HELLP syndrome in 2% (1), intrauterine growth restriction in 14% (6, leading in one case to a medically indicated termination of pregnancy). There were 42 live births (98%) at a median term of 38 weeks (range 27 to 42), 9 of them before 37 weeks (21%); median birth weight was 2,940 grams, five children (12%) were transferred to a neonatal intensive care unit and one boy born at 27 weeks died at two days of life. During pregnancy, 25 women of 43 (58%) were receiving corticosteroids, 9 of 43 (21%) azathioprine and 1 of 43 (2%) infliximab. The risk of gestational hypertension was associated with pre-existing chronic hypertension (p = 0.01) and with subdiaphragmatic vascular involvement (p = 0.04). No correlation was found between disease activity and obstetric complications, and the authors conclude that there is a high rate of adverse obstetric complications without significant effect on the live birth rate, pregnancy not appearing to influence disease activity (Clinical Rheumatology, 2020, 2nd of 34 and corresponding author). This work links this page to the one devoted to reproductive immunology.

Where care is organised

Department of internal medicine of Saint-Antoine Hospital (AP-HP), Sorbonne Université, within the medical and university department devoted to inflammation, immunopathology and biotherapies, designated DHU i2B until about 2019, then DMU 3iD and DMU i3 in the affiliations of his publications. The corresponding addresses of his publications of 2025 and 2026 attach this department to the reference centre for rare systemic autoimmune diseases and to the reference centre for autoinflammatory diseases and inflammatory amyloidosis (CeRéMAIA).

The care of these diseases is shared with radiology and nuclear medicine, which cover CT angiography, MR angiography and FDG-PET, with vascular surgery for tight or aneurysmal lesions, and with neurology for forms with cerebrovascular expression. The publications cited above carry the trace of these collaborations: nuclear medicine of the Avicenne hospital for the work on PET, vascular surgery and interventional radiology of the Pitié-Salpêtrière for the Takayasu cohorts, the neurology department of Saint-Antoine for the study of ischaemic cerebral events in giant cell arteritis.

The multicentre work is conducted within two identifiable collective frameworks. The French Takayasu network is the consortium indexed by PubMed on most of his articles devoted to Takayasu arteritis since 2017; the BIOTAK registry is named in the 2026 publication on JAK inhibitors and brings together referral centres in France, Italy, Spain, Armenia, Israel, Japan, Tunisia and Russia. For giant cell arteritis, the French Study Group for Large Vessel Vasculitis (GEFA) is the consortium of the French recommendations of 2025.

Three clinical trials in this field appear on the "Clinical research" page of the site. TOCITAKA (NCT02101333, tocilizumab as first-line treatment in Takayasu arteritis, sponsor AP-HP) is completed and its results are published; the principal investigator declared on ClinicalTrials.gov is Olivier Fain. TOGIAC (NCT04888221, tocilizumab combined with corticosteroids against placebo in giant cell arteritis with cerebrovascular involvement, phase III trial, sponsor AP-HP) has a single declared location, the neurology unit of Saint-Antoine Hospital; enrolment closed on 29 January 2025 and no results are published to date. TOCIALLON, devoted to ophthalmological involvement in giant cell arteritis, has no public identifier found as of 8 September 2026.

What is not known

There is no reliable marker of the activity of these diseases. That is the finding of his own imaging work: the 2016 review concludes that the value of the parameters used to characterise persistent arterial inflammation, and the threshold to be used, remain to be defined, and the 2015 meta-analysis notes that the specificity of FDG-PET is markedly lower in Takayasu arteritis (73%) than in giant cell arteritis (98%). The distinction between active inflammation and fixed lesion is therefore not settled.

Almost all his series on biologics in Takayasu arteritis are retrospective, without a randomised comparison arm. The equivalence observed between anti-TNF and tocilizumab in 209 patients, like the equivalence between infliximab and adalimumab in 135 patients, rests on non-randomised comparisons between groups treated according to the clinicians' choice, which exposes them to indication bias. There is to date no French randomised trial comparing these two classes in Takayasu arteritis.

The only prospective trial of this series, TOCITAKA, is open-label, without a control arm, and included only 13 patients in the publication of its results. It shows a steroid-sparing effect in slightly more than one patient in two, but 45% of the responders relapsed after withdrawal of treatment: the optimal duration of a biologic and the criteria for stopping it are not established. The work on JAK inhibitors rests on 20 patients, only 18 of whom were evaluable for the response endpoint, and it was published as a letter. The work on rituximab is also a letter, for which PubMed carries no numerical abstract. These two results have not been replicated.

On the giant cell arteritis side, the 2026 study on the French national health data system is an observational study that imitates the design of a randomised trial without being one: it observes an association between starting tocilizumab and a lower risk of major cardiovascular events, it does not establish a cause and effect relationship, and it rests on medico-administrative data and not on clinical examination. It was still in advance online publication as of 8 September 2026. The results of the TOGIAC trial, which would put the question in randomised form in cerebrovascular forms, are not published and no result can be attributed to it.

On pregnancy, the 2020 study is the most fully documented work he has signed on this subject, and it is also the most fragile by design. It is retrospective and has no comparison group: neither pregnant women without Takayasu arteritis, nor affected women who were not pregnant. Its percentages are calculated on 43 pregnancies and not on 33 women, so several women are counted more than once. Data collection stops in August 2015 and includes only one pregnancy under infliximab: it says nothing about what becomes of a pregnancy under the biologics described above. With 4 cases of pre-eclampsia and a single HELLP syndrome, the most widely quoted figures of this work rest on very few events. The absence of a correlation between disease activity and obstetric complications is a negative result obtained in a small sample: it does not allow the conclusion that no such link exists.

The international reference texts in this field are not his and are mentioned here only to place his work: the 2018 update of the EULAR recommendations for the management of large-vessel vasculitis (Annals of the Rheumatic Diseases, 2020, 10.1136/annrheumdis-2019-215672) and the 2022 American College of Rheumatology and EULAR classification criteria for Takayasu arteritis (Annals of the Rheumatic Diseases, 2022, 10.1136/ard-2022-223482). The French national diagnosis and care protocol devoted to Takayasu arteritis, coordinated by others, cites him as a collaborator and not as an author: this point has not been re-checked in the present work.

References

Published work he has taken part in, on this subject.

General information only. This page does not replace individual medical advice. Updated: 10 September 2026.