Shang Trisha, Kaelber David C, Chaaban Mohamad R
School of Medicine, Case Western Reserve University, Cleveland, OH 44106, USA.
The Center for Clinical Informatics Research and Education, The MetroHealth System, Cleveland, OH 44109, USA.
J Clin Med. 2025 Apr 25;14(9):2962. doi: 10.3390/jcm14092962.
Granulomatosis with polyangiitis (GPA) is an autoimmune vasculitis, often presenting first with sinonasal symptoms diagnosed as vasculitis chronic rhinosinusitis (CRS). Patients with limited (L) GPA do not have renal involvement and often have more local sinonasal disease. Few studies have examined systemic progression in LGPA patients presenting with local sinonasal disease. Our objective was to compare GPA disease progression and activity in LGPA patients with and without CRS. Using the US Collaborative Network of the TriNetX platform, we conducted a retrospective study of adults with LGPA and CRS versus those without CRS. Outcomes were measured 1 month-5 years after patients met inclusion criteria. Primary outcomes were acute sinusitis, end-organ damage, and major GPA disease activity. Secondary outcomes were end-organ damage and major disease activity for each organ system and mortality. There were = 1097 in the LGPA with CRS cohort and n = 3331 in the LGPA without CRS cohort, with n = 1023 in each cohort after 1:1 propensity matching on age, gender, ethnicity, and race. We found a significantly greater risk of acute sinusitis (risk ratio: 4.80, 95% confidence interval: [2.89,7.99]), end-organ damage (2.99 [2.41, 3.70]), and major disease activity (2.41 [1.73, 3.35]) comparing patients with CRS to those without. LGPA patients with CRS had no significant difference in mortality compared to those without CRS (0.94, [0.64,1.38]). Patients with LGPA and CRS have greater risk of developing disease progression and increased organ system disease activity compared to LGPA without CRS.
肉芽肿性多血管炎（GPA）是一种自身免疫性血管炎，通常最初表现为被诊断为血管炎慢性鼻窦炎（CRS）的鼻窦症状。局限性（L）GPA患者无肾脏受累，且往往有更多局部鼻窦疾病。很少有研究探讨表现为局部鼻窦疾病的LGPA患者的全身病情进展。我们的目的是比较有和没有CRS的LGPA患者的GPA疾病进展和活动情况。利用TriNetX平台的美国协作网络，我们对患有LGPA和CRS的成人与没有CRS的成人进行了一项回顾性研究。在患者符合纳入标准后1个月至5年测量结果。主要结局是急性鼻窦炎、终末器官损伤和主要GPA疾病活动。次要结局是每个器官系统的终末器官损伤和主要疾病活动以及死亡率。LGPA合并CRS队列中有n = 1097例，LGPA无CRS队列中有n = 3331例，在按年龄、性别、种族和民族进行1:1倾向匹配后，每个队列中有n = 1023例。我们发现，与没有CRS的患者相比，患有CRS的患者发生急性鼻窦炎的风险显著更高（风险比：4.80，95%置信区间：[2.89,7.99]）、终末器官损伤（2.99 [2.41, 3.70]）和主要疾病活动（2.41 [1.73, 3.35]）。与没有CRS的LGPA患者相比，患有CRS的LGPA患者在死亡率方面无显著差异（0.94，[0.64,1.38]）。与没有CRS的LGPA患者相比，患有LGPA和CRS的患者发生疾病进展和器官系统疾病活动增加的风险更高。
