scholarly journals Disease Activity in Takayasu Arteritis Affects Long-Term Graft-Related Outcomes

2018 ◽  
Vol 67 (2) ◽  
pp. e31
Author(s):  
Anthony H. Chau ◽  
Victor J. Davila ◽  
William W. Sheaffer ◽  
William M. Stone ◽  
Richard J. Fowl ◽  
...  
Keyword(s):  
Disease Activity ◽  
Takayasu Arteritis

scholarly journals AB0509 SUSPENSIVE EFFICACY OF TOCILIZUMAB IN TREATMENT-NAÏVE PATIENTS WITH TAKAYASU ARTERITIS: TOCITAKA FRENCH PROSPECTIVE MULTICENTER OPEN-LABELLED TRIAL.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1552.3-1552
Author(s):  
A. Mekinian ◽  
D. Saadoun ◽  
J. C. N. F. [email protected] ◽  
I. Q. M. F. [email protected] ◽  
P. Jégo ◽  
...  
Keyword(s):  
Disease Activity ◽  
Takayasu Arteritis ◽  
Immunosuppressive Drugs ◽  
Relapse Free Survival ◽  
Research Support ◽  
Free Survival ◽  
Treatment Naïve ◽  
Steroid Sparing

Objectives:To assess long term efficacy of tocilizumab in treatment-naive patients with Takayasu arteritis (TAK).Methods:In this multicenter, prospective, open-labelled trial, we aim to evaluate the benefit of adding tocilizumab to steroids in treatment-naïve patients with TAK, on discontinuation of steroids after 6 months of tocilizumab treatment, and to assess relapse-free survival following tocilizumab discontinuation.Results:Thirteen patients with TAK were included, with a median age of 32 years [19-45] and 12 (92%) females. Six (54%) patients met the primary end-point. Among 11 (85%) patients which achieved remission at 6 months, 6 (54%) have reached primary endpoint.. Among the 5 remaining patients which continued steroids, 3 had a prednisone-equivalent dosage < 5mg/day. A significant decrease of disease activity was observed after 6 months of tocilizumab therapy: decrease of median NIH scale (3 [3-4] at baseline, versus 1 [0-2] after 6 months; p <0.001), ITAS-2010 score (5 [2-7] versus 3 [0-8]; p = 0.002), and ITAS-A score (7 [4-10] versus 4 [1-15]; p = 0.0001)]. All patients discontinued tocilizumab after 7 infusions, and no other immunosuppressive drugs was introduced, except for 1 patient which received methotrexate. After 9 and 12 months, respectively 7 (54%) and 6 (50%) patients achieved remission with less than 7.5 mg/day of prednisone, and 9 (69%) and 9 (75%) with doses <10 mg/day. During the 12 months follow-up after tocilizumab discontinuation, a relapse occurred among 5 patients (45%) out of 11 in which achieved remission after 6 months of tocilizumab.No severe AEs were considered related to study treatment and none required tocilizumab interruption or dose reduction. No deaths have occurred during the study period.Conclusion:Tocilizumab seems an effective steroid sparing therapy in TAK but its effect appears to be suspensive.Disclosure of Interests:Arsene Mekinian: None declared, david Saadoun: None declared, [email protected] [email protected]: None declared, [email protected] [email protected]: None declared, Patrick Jégo: None declared, [email protected] [email protected]: None declared, wxv wxv: None declared, Jacques-Eric Gottenberg Grant/research support from: BMS, Pfizer, Consultant of: BMS, Sanofi-Genzyme, UCB, Speakers bureau: Abbvie, Eli Lilly and Co., Roche, Sanofi-Genzyme, UCB, Mathieu Vautier: None declared, [email protected]>; [email protected]>;: None declared, Patrice cacoub: None declared, olivier fain: None declared

scholarly journals Tocilizumab in treatment-naïve patients with Takayasu arteritis: TOCITAKA French prospective multicenter open-labeled trial

2020 ◽  
Vol 22 (1) ◽  
Author(s):  
Arsene Mekinian ◽  
◽  
David Saadoun ◽  
Eric Vicaut ◽  
Sara Thietart ◽  
...  
Keyword(s):  
Maintenance Therapy ◽  
Disease Activity ◽  
Primary Endpoint ◽  
Takayasu Arteritis ◽  
Number Of Patients ◽  
Treatment Naïve ◽  
Secondary Endpoints ◽  
Steroid Sparing

Abstract Objectives To assess long-term efficacy of tocilizumab in treatment-naive patients with Takayasu arteritis (TAK). Methods Prospective open-labeled trial in naïve patients with TAK who received steroids at the dose of 0.7 mg/kg/day and 7 infusions of 8 mg/kg/month of tocilizumab. The primary endpoint was the number of patients who discontinued steroids after 7 infusions of tocilizumab. Secondary endpoints included disease activity and the number of relapses during 18-month follow-up. Results Thirteen patients with TAK were included, with a median age of 32 years [19–45] and 12 (92%) females. Six (54%) patients met the primary end-point. A significant decrease of disease activity was observed after 6 months of tocilizumab therapy: decrease of median NIH scale (3 [3, 4] at baseline, versus 1 [0–2] after 6 months; p < 0.001), ITAS-2010 score (5 [2–7] versus 3 [0–8]; p = 0.002), and ITAS-A score (7 [4–10] versus 4 [1–15]; p = 0.0001)]. During the 12-month follow-up after tocilizumab discontinuation, a relapse occurred among 5 patients (45%) out of 11 in which achieved remission after 6 months of tocilizumab. Conclusion Tocilizumab seems an effective steroid sparing therapy in TAK, but maintenance therapy is necessary. Trial registration ClinicalTrials.gov NCT02101333. Registered on 02 April 2014.

scholarly journals Tocilizumab in treatment-naïve patients with Takayasu arteritis: TOCITAKA French prospective multicenter open-labelled trial

2020 ◽  
Author(s):  
Arsene Mekinian ◽  
David Saadoun ◽  
Eric Vicaut ◽  
Sarah Thietart ◽  
Bertrand Lioger ◽  
...  
Keyword(s):  
Maintenance Therapy ◽  
Disease Activity ◽  
Primary Endpoint ◽  
Takayasu Arteritis ◽  
Number Of Patients ◽  
Treatment Naïve ◽  
Secondary Endpoints ◽  
Steroid Sparing

Abstract ObjectivesTo assess long term efficacy of tocilizumab in treatment-naive patients with Takayasu arteritis (TAK).MethodsProspective open-labelled trial in naïve patients with TAK who received steroids at the dose of 0.7 mg/kg/day and 7 infusions of 8 mg/kg/month of tocilizumab. The primary endpoint was the number of patients which discontinued steroids after 7 infusions of tocilizumab. Secondary endpoints included disease activity and the number of relapses during an 18-month follow-up.ResultsThirteen patients with TAK were included, with a median age of 32 years [19-45] and 12 (92%) females. Six (54%) patients met the primary end-point. A significant decrease of disease activity was observed after 6 months of tocilizumab therapy: decrease of median NIH scale (3 [3-4] at baseline, versus 1 [0-2] after 6 months; p <0.001), ITAS-2010 score (5 [2-7] versus 3 [0-8]; p = 0.002), and ITAS-A score (7 [4-10] versus 4 [1-15]; p = 0.0001)]. During the 12 months follow-up after tocilizumab discontinuation, a relapse occurred among 5 patients (45%) out of 11 in which achieved remission after 6 months of tocilizumab.ConclusionTocilizumab seems an effective steroid sparing therapy in TAK but maintenance therapy is necessary.

scholarly journals A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab

2021 ◽  
Vol 10 (6) ◽  
pp. 1329
Author(s):  
Johanna Backhus ◽  
Christian Neumann ◽  
Lukas Perkhofer ◽  
Lucas A Schulte ◽  
Benjamin Mayer ◽  
...  
Keyword(s):  
Disease Activity ◽  
Clinical Activity ◽  
Inflammatory Disorder ◽  
International Consensus ◽  
Steroid Pulse ◽  
Related Disease ◽  
Igg4 Related Disease

Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity. Methods: We retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019. Results: Patients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX. Conclusion: RTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6–9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy.

scholarly journals OP0146 LONG TERM OUTCOME AND PROGNOSIS FACTORS OF ISOLATED AORTITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 93.1-93
Author(s):  
Y. Ferfar ◽  
S. Morinet ◽  
O. Espitia ◽  
C. Agard ◽  
M. Vautier ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Giant Cell Arteritis ◽  
Takayasu Arteritis ◽  
Vascular Complications ◽  
Event Free Survival ◽  
Prognosis Factors ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Free Survival

Background:Aortitis is a group of disorders characterized by the inflammation of the aorta. The most common causes of aortitis are the large-vessel vasculitis i.e. giant cell arteritis (GCA) and Takayasu arteritis (TA). However, aortitis may be isolated. Because of the wide variation in the course of aortitis, predicting outcome is challenging. The optimal management strategy of isolated aortitis (IA) is still unclear as IA is poorly defined, with data consisting of small retrospective and case control studies.Objectives:To assess the long-term outcome and prognosis factors for vascular complications in patients with isolated aortitis.Methods:Retrospective multicenter study of 353 patients with non-infectious aortitis including 136 giant cell arteritis (GCA), 96 Takayasu arteritis (TA) and 73 isolated aortitis (IA). Factors associated with event-free survival, vascular event-free survival and revascularization-free survival were assessed. Risk factors for vascular complications were identified in multivariate analysis.Results:After a median follow up of 52 months, vascular complications were observed in 32.3 %, revascularization in 30 % and death in 7.6%. The 5-year cumulative incidence of vascular complications was 58% (41; 71), 20% (13; 29), and 19 % (11; 28) in IA, GCA and TA, respectively. In multivariate analysis, IA [HR, 1.85 (1.19 to 2.88), p=0.017] and male gender [1.77 (1.26 to 2.49), p<0.0001] were independently associated with vascular events. The 5-year surgery-free survival was 45% (31; 65), 71% (62; 81) and 76% (68; 86) in IA, TA and GCA, respectively.Conclusion:IA has a worse vascular prognosis than GCA and TA. Sixty percent of IA patients will experience a vascular complication within 5 years from diagnosis.Disclosure of Interests:None declared

scholarly journals POS0815 CLINICAL CHARACTERISTICS, IMAGING PHENOTYPE, AND LONG-TERM OUTCOMES OF TAKAYASU ARTERITIS PATIENTS WITH HYPERTENSION

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 661.1-661
Author(s):  
Y. Sun ◽  
L. Ma ◽  
H. Chen ◽  
C. Rongyi ◽  
L. Jiang
Keyword(s):  
Blood Pressure ◽  
Adverse Events ◽  
Clinical Features ◽  
Takayasu Arteritis ◽  
Pressure Control ◽  
Free Survival ◽  
The World ◽  
Cluster 2

Background:Hypertension occurred in 30-80% of TAK patients around the world. The occurrence of hypertension might severely worsen TAK prognosis. Nevertheless, data describing the specific imaging features in hypertensive TAK patients and the associations between hypertensive severity, blood pressure control status and long-term outcome were still lacking.Objectives:To investigate the characteristics and associations of hypertensive characteristics with adverse events-free survival in Takayasu arteritis (TAK) patients with hypertension.Methods:This research was based on a prospectively on-going observational cohort-East China Takayasu Arteritis (ECTA) cohort. In all, 618 TAK patients, who registered in the ECTA cohort up to December 2019, were enrolled. The main outcome was the adverse-events-free survival among hypertensive TAK patients during the follow-up ended on August 2020.Results:Totally, 204 (33.0%) patients suffered from hypertension, with 48 (23.5%), 62 (30.4%), and 94 (46.1%) mild, moderate, and severe hypertension, respectively. Cluster analysis indicated three imaging phenotypes for hypertensive TAK patients: Cluster 1: involvement of the abdominal aorta and/or renal artery (n=56, 27.5%); Cluster 2: involvement of the ascending aorta, thoracic aorta, and the aortic arch and its branches (n=38, 18.6%); Cluster 3: combined involvement of Cluster 1 and Cluster 2 (n=111, 54.4%). By the end of the follow-up, the blood pressure control rate was 50.8%, while the adverse-events-free survival was 67.9% in the entire hypertensive population. Multivariate Cox regression analysis indicated that well-controlled blood pressure (HR=2.13, 95%CI 1.32–3.78, p=0.047), co-existence of severe aortic valve regurgitation (HR=0.87, 95%CI 0.64–0.95, p=0.043), Cluster 1 (HR=0.69, 95%CI 0.48–0.92, p=0.017) and Cluster 3 (HR=0.72, 95%CI 0.43–0.94, p=0.048) imaging phenotype was associated with the adverse-events-free survival.Conclusion:Patients with controlled hypertension showed better adverse-events-free survival, while those with the Cluster 1 imaging phenotype were more likely to suffer from worse adverse-events-free survival. Hypertension occurred in 30-80% of TAK patients around the world. The occurrence of hypertension might severely worsen TAK prognosis.References:[1]Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol 2002; 55:481–6.[2]Watanabe Y, Miyata T, Tanemoto K. Current clinical features of new patients with Takayasu arteritis observed from a cross-country research in Japan: age and sex specificity. Circulation 2015; 132:1701–9.[3]Yilmaz N, Can M, Oner FA, et al. Impaired quality of life, disability and mental health in Takayasu’s arteritis. Rheumatol. (Oxford) 2013; 52:1898–904.[4]Laurent A, Julien H, Nicolas L, et al. Takayasu arteritis in France: a single-center retrospective study of 82 cases comparing white, North African, and black patients. Medicine 2010; 89:1–17.[5]Mwipatayi BP, Jeffery PC, Beningfield SJ, et al. Takayasu arteritis: clinical features and management: report of 272 cases. ANZ J Surg 2005; 75:110–7.Disclosure of Interests:None declared

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