Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebron and Systemic Autoimmune Research Unit, Vall d'Hebron Research Institute, Universitat Autònoma de Barcelona, Passeig Vall d'Hebron 119-29, 08035-Barcelona, Catalonia, Barcelona, Spain.
Department of Medicine, Faculty of Medicine, Universitat Autònoma de Barcelona, Barcelona, Spain.
Rheumatol Int. 2024 Oct;44(10):2253-2261. doi: 10.1007/s00296-024-05624-2. Epub 2024 Aug 24.
Juvenile Temporal Arteritis (JTA) is a rare non-granulomatous vasculitis affecting the superficial temporal arteries, mostly in individuals under 45 years old. It is often misdiagnosed due to its benign nature and the absence of systemic symptoms. Herein, we present a case report of a 40-year-old woman who initially presented with painless nodules in the left temporal area. Following a biopsy, the patient developed additional nodules not only in the same temple but also on the contralateral side. Remarkably, these nodules underwent spontaneous regression without further treatment, highlighting the variability in JTA's course and distinctive response to intervention. In addition, through a systematic literature review of 43 case reports - 17 with bilateral involvement - we aimed to thoroughly understand the clinical and histopathological findings, diagnostic processes, and treatment responses in JTA, with an emphasis on cases with bilateral involvement. Findings indicate that JTA typically presents as painless or painful temporal nodules, rarely accompanied by other non-specific symptoms, making histopathological examination crucial for accurate diagnosis. Collectively, our work provides the most extensive account of bilateral JTA cases to date. It emphasizes the need for clinical awareness of this condition, contributes valuable data to the limited information available on this rare condition and serves as a stepping-stone for further inquiry. The main takeaway from this review is the variable nature of JTA and the importance of histopathology in diagnosis, which helps clinicians avoid excessive testing and overtreatment and anticipate possible spontaneous resolution.
青少年颞动脉炎(JTA)是一种罕见的非肉芽肿性血管炎,影响颞浅动脉,主要发生在 45 岁以下的个体。由于其良性性质和缺乏全身症状,常被误诊。在此,我们报告一例 40 岁女性,最初表现为左侧颞区无痛性结节。活检后,患者不仅在同一颞部,而且在对侧也出现了更多的结节。值得注意的是,这些结节自行消退,无需进一步治疗,突出了 JTA 病程的可变性和对干预的独特反应。此外,我们通过对 43 例病例报告的系统文献回顾 - 其中 17 例为双侧受累 - 旨在全面了解 JTA 的临床和组织病理学发现、诊断过程和治疗反应,重点关注双侧受累的病例。研究结果表明,JTA 通常表现为无痛或疼痛性颞部结节,很少伴有其他非特异性症状,因此组织病理学检查对于准确诊断至关重要。总之,我们的工作提供了迄今为止关于双侧 JTA 病例最广泛的描述。它强调了对这种疾病的临床认识的必要性,为这种罕见疾病提供了有限的信息提供了有价值的数据,并为进一步研究提供了基础。从这次审查中得出的主要结论是 JTA 的多变性和组织病理学在诊断中的重要性,这有助于临床医生避免过度检查和过度治疗,并预测可能的自发缓解。