Blanco Ricardo, Aldasoro Vicente, Maiz Olga, Melero Rafael, Romero-Yuste Susana, de Miguel Eugenio, Ferraz-Amaro Iván, López-Gutiérrez Fernando, Castañeda Santos, Loricera Javier
Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Immunopathology Group, IDIVAL, Santander, Spain.
Department of Rheumatology, Complejo Hospitalario de Navarra, Pamplona, Spain.
Rheumatology (Oxford). 2025 May 1;64(5):2928-2936. doi: 10.1093/rheumatology/keae666.
The spectrum of GCA includes various vascular phenotypes. Tocilizumab (TCZ) is the only biologic therapy currently approved, regardless of phenotype. We aimed to assess the effectiveness of TCZ in various phenotypes.
This is a multicentre observational study of GCA patients treated with TCZ. They were divided into three phenotypes: (i) cranial (cGCA), (ii) extracranial GCA (ecGCA) and (iii) mixed GCA (mixGCA). Outcomes included clinical remission, EULAR complete remission, relapses, absence of inflammation as shown using imaging techniques, and safety.
We studied 471 patients (342 women; mean age 74.0 ± 9.0 years). The phenotypic distribution was: cGCA (n = 217; 46%), mixGCA (174; 37%) and ecGCA (80; 17%). Patients with ecGCA were younger (66.5 ± 10.1 years) than those with cGCA (74.8 ± 8.1) and those with mixGCA (71.4 ± 8.5), and had a longer delayed GCA diagnosis {median [interquartile range (IQR) [6 (1-14)] vs 1 (1-3) vs 2 (1-6) months, respectively}. Systemic manifestations were similar in the three groups, while ischaemic manifestations were more frequent in cGCA. Combined TCZ, in addition to glucocorticoids, was used more frequently in ecGCA (36%). Clinical remission was observed in 51%/43%/47% in cGCA/ecGCA/mixGCA, respectively, after the first month, and in 79%/81%/89% after 24 months. Complete EULAR remission in 35%/27%/28% (after 1 month) and 72%/73%/67% (after 24 months). Absence of inflammation being shown in the imaging techniques was 15%/26% after 12 months, and 22%/7% (ecGCA/mixGCA) (after 24 months). Relevant adverse events were observed in 109 (23.1%) patients.
TCZ shows rapid and maintained effectiveness in all GCA phenotypes in clinical remission and EULAR complete remission. By contrast, absence of inflammation as shown using imaging techniques was much lower in ecGCA and mixGCA.
巨细胞动脉炎（GCA）的谱系包括各种血管表型。托珠单抗（TCZ）是目前唯一获批的生物疗法，无论表型如何。我们旨在评估TCZ在各种表型中的有效性。
这是一项对接受TCZ治疗的GCA患者的多中心观察性研究。他们被分为三种表型：（i）颅型（cGCA），（ii）颅外型GCA（ecGCA）和（iii）混合型GCA（mixGCA）。结局包括临床缓解、欧洲抗风湿病联盟（EULAR）完全缓解、复发、影像学检查显示无炎症以及安全性。
我们研究了471例患者（342例女性；平均年龄74.0±9.0岁）。表型分布为：cGCA（n = 217；46%），mixGCA（174例；37%）和ecGCA（80例；17%）。ecGCA患者（66.5±10.1岁）比cGCA患者（74.8±8.1岁）和mixGCA患者（71.4±8.5岁）更年轻，且GCA诊断延迟时间更长{中位数[四分位间距（IQR）][分别为6（1 - 14）] vs 1（1 - 3）vs 2（1 - 6）个月}。三组的全身表现相似，而缺血性表现在cGCA中更常见。除糖皮质激素外，联合使用TCZ在ecGCA中更频繁（36%）。第一个月后，cGCA/ecGCA/mixGCA的临床缓解率分别为51%/43%/47%，24个月后为79%/81%/89%。EULAR完全缓解率在1个月后为35%/27%/28%，24个月后为72%/73%/67%。影像学检查显示12个月后无炎症的比例为15%/26%，24个月后为22%/7%（ecGCA/mixGCA）。109例（23.1%）患者观察到相关不良事件。
TCZ在所有GCA表型的临床缓解和EULAR完全缓解方面均显示出快速且持续的有效性。相比之下，ecGCA和mixGCA中影像学检查显示无炎症的比例要低得多。
