Childhood-Onset Takayasu Arteritis: Clinical Features of Disease and Relapse Risk Factors

<b>Background:</b> Takayasu’s arteritis (TA) is a systemic vasculitis that primarily affects the aorta and major arteries. Despite aggressive treatment with glucocorticoids (GCs) and non-biological disease-modifying antirheumatic drugs (nbDMARDs), about 30% of patients experience resista...

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Main Authors: Vera Podzolkova, Galina Lyskina, Olga Shpitonkova, Angelina Polyanskaya, Svetlana Chebysheva, Marina Shakhnazarova, Jinbo Zhao, Aleksandr Suvorov, Vera Khudoroshkova, Natalia Geppe
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Language:English
Published: MDPI AG 2025-01-01
Series:Children
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Online Access:https://www.mdpi.com/2227-9067/12/1/70
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author Vera Podzolkova
Galina Lyskina
Olga Shpitonkova
Angelina Polyanskaya
Svetlana Chebysheva
Marina Shakhnazarova
Jinbo Zhao
Aleksandr Suvorov
Vera Khudoroshkova
Natalia Geppe
author_facet Vera Podzolkova
Galina Lyskina
Olga Shpitonkova
Angelina Polyanskaya
Svetlana Chebysheva
Marina Shakhnazarova
Jinbo Zhao
Aleksandr Suvorov
Vera Khudoroshkova
Natalia Geppe
author_sort Vera Podzolkova
collection DOAJ
description <b>Background:</b> Takayasu’s arteritis (TA) is a systemic vasculitis that primarily affects the aorta and major arteries. Despite aggressive treatment with glucocorticoids (GCs) and non-biological disease-modifying antirheumatic drugs (nbDMARDs), about 30% of patients experience resistance to therapy or relapse. This study aimed to identify risk factors associated with refractory and relapse TA in pediatric patients. <b>Methods:</b> A retrospective, open-label, case–control study was conducted with 56 pediatric patients with TA diagnosed between February 2011 and October 2022. Fourteen patients were excluded due to insufficient data in their medical records, leaving 42 for further analysis. The patients were divided into two groups: Group 1 (18 patients) with no evidence of relapse and Group 2 (24 patients) with relapse despite first-line treatment at the end of the follow-up period. Clinical, laboratory, and instrumental data were collected and analyzed using R v4.2 and Python v3.10. <b>Results:</b> The median time to relapse was 18 [IQR: 13; -] months according to the Kaplan–Meier curve. Patients with ITAS.A with a diagnosis of TA ≥ 12 had a higher probability of relapse, according to the log-rank criterion (<i>p</i> = 0.006). Symptoms of critical ischemia, such as limb claudication, were more common in Group 2 at diagnosis (<i>p</i> = 0.047), and a trend toward a longer diagnostic delay was observed (<i>p</i> = 0.067). <b>Conclusions:</b> Pediatric patients with an initial ITAS.A score above 12 have a higher risk of relapse when treated with a combination of GCs and nbDMARDs as first-line treatment. Further research is needed to identify high-risk patients more accurately and optimize therapeutic strategies.
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spelling doaj-art-6c4d699af3644ce4881f8bdb80fb60f52025-01-24T13:27:11ZengMDPI AGChildren2227-90672025-01-011217010.3390/children12010070Childhood-Onset Takayasu Arteritis: Clinical Features of Disease and Relapse Risk FactorsVera Podzolkova0Galina Lyskina1Olga Shpitonkova2Angelina Polyanskaya3Svetlana Chebysheva4Marina Shakhnazarova5Jinbo Zhao6Aleksandr Suvorov7Vera Khudoroshkova8Natalia Geppe9Department of Children’s Diseases, N.F. Filatov Clinical Institute of Children’s Health, I.M. Sechenov First Moscow State Medical University, 119435 Moscow, RussiaDepartment of Children’s Diseases, N.F. Filatov Clinical Institute of Children’s Health, I.M. Sechenov First Moscow State Medical University, 119435 Moscow, RussiaDepartment of Children’s Diseases, N.F. Filatov Clinical Institute of Children’s Health, I.M. Sechenov First Moscow State Medical University, 119435 Moscow, RussiaDepartment of Children’s Diseases, N.F. Filatov Clinical Institute of Children’s Health, I.M. Sechenov First Moscow State Medical University, 119435 Moscow, RussiaDepartment of Children’s Diseases, N.F. Filatov Clinical Institute of Children’s Health, I.M. Sechenov First Moscow State Medical University, 119435 Moscow, RussiaDepartment of Children’s Diseases, N.F. Filatov Clinical Institute of Children’s Health, I.M. Sechenov First Moscow State Medical University, 119435 Moscow, RussiaDepartment of Internal, Occupational Diseases and Rheumatology, N.V. Sklifosovsky Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University, 119435 Moscow, RussiaWorld-Class Research Center “Digital Biodesign and Personalized Healthcare”, I.M. Sechenov First Moscow State Medical University, 119048 Moscow, RussiaN.V. Sklifosovsky Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University, 119048 Moscow, RussiaDepartment of Children’s Diseases, N.F. Filatov Clinical Institute of Children’s Health, I.M. Sechenov First Moscow State Medical University, 119435 Moscow, Russia<b>Background:</b> Takayasu’s arteritis (TA) is a systemic vasculitis that primarily affects the aorta and major arteries. Despite aggressive treatment with glucocorticoids (GCs) and non-biological disease-modifying antirheumatic drugs (nbDMARDs), about 30% of patients experience resistance to therapy or relapse. This study aimed to identify risk factors associated with refractory and relapse TA in pediatric patients. <b>Methods:</b> A retrospective, open-label, case–control study was conducted with 56 pediatric patients with TA diagnosed between February 2011 and October 2022. Fourteen patients were excluded due to insufficient data in their medical records, leaving 42 for further analysis. The patients were divided into two groups: Group 1 (18 patients) with no evidence of relapse and Group 2 (24 patients) with relapse despite first-line treatment at the end of the follow-up period. Clinical, laboratory, and instrumental data were collected and analyzed using R v4.2 and Python v3.10. <b>Results:</b> The median time to relapse was 18 [IQR: 13; -] months according to the Kaplan–Meier curve. Patients with ITAS.A with a diagnosis of TA ≥ 12 had a higher probability of relapse, according to the log-rank criterion (<i>p</i> = 0.006). Symptoms of critical ischemia, such as limb claudication, were more common in Group 2 at diagnosis (<i>p</i> = 0.047), and a trend toward a longer diagnostic delay was observed (<i>p</i> = 0.067). <b>Conclusions:</b> Pediatric patients with an initial ITAS.A score above 12 have a higher risk of relapse when treated with a combination of GCs and nbDMARDs as first-line treatment. Further research is needed to identify high-risk patients more accurately and optimize therapeutic strategies.https://www.mdpi.com/2227-9067/12/1/70Takayasu’s arteritissystemic vasculitisITAS.A score
spellingShingle Vera Podzolkova
Galina Lyskina
Olga Shpitonkova
Angelina Polyanskaya
Svetlana Chebysheva
Marina Shakhnazarova
Jinbo Zhao
Aleksandr Suvorov
Vera Khudoroshkova
Natalia Geppe
Childhood-Onset Takayasu Arteritis: Clinical Features of Disease and Relapse Risk Factors
Children
Takayasu’s arteritis
systemic vasculitis
ITAS.A score
title Childhood-Onset Takayasu Arteritis: Clinical Features of Disease and Relapse Risk Factors
title_full Childhood-Onset Takayasu Arteritis: Clinical Features of Disease and Relapse Risk Factors
title_fullStr Childhood-Onset Takayasu Arteritis: Clinical Features of Disease and Relapse Risk Factors
title_full_unstemmed Childhood-Onset Takayasu Arteritis: Clinical Features of Disease and Relapse Risk Factors
title_short Childhood-Onset Takayasu Arteritis: Clinical Features of Disease and Relapse Risk Factors
title_sort childhood onset takayasu arteritis clinical features of disease and relapse risk factors
topic Takayasu’s arteritis
systemic vasculitis
ITAS.A score
url https://www.mdpi.com/2227-9067/12/1/70
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