Department of Medicine, Section of Rheumatology, Helsingborg Hospital, Helsingborg, Sweden.
Scand J Rheumatol. 2009;38(4):268-75. doi: 10.1080/03009740802668554.
To assess the extent and pattern of irreversible organ damage in patients with Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), polyarteritis nodosa (PAN), and Churg-Strauss syndrome (CSS) by a cross-sectional point prevalence study within a defined geographical area.
The Vasculitis Damage Index (VDI) was recorded for 86 prevalent cases, classified as 46 patients with WG, 27 with MPA, nine with PAN, and four with CSS from a defined population in southern Sweden, with a median age of 64.8 years and a median disease duration of 9 years. The VDI was determined for all patients at the day of point prevalence (pp), 1 January 2003.
The median VDI score was 3 [interquartile range (IQR) 2-5] for all patients: 3 (2-4) for WG, 3 (1.5-4.5) for MPA, 5 (2-6) for PAN, and 1.5 (0.75-2.75) for CSS. Only 9% of patients had not been assigned a single item of damage. The most common damage was cardiovascular, followed by renal, neuropsychiatric, ear nose and throat (ENT), and musculoskeletal. Major vascular and treatment-related damage was associated with advanced age whereas ENT damage was more prevalent in younger patients. There was an almost complete separation between ENT damage and cardiac and renal damage with only two out of the 22 patients assigned ENT damage having experienced renal damage; none had been assigned cardiac damage. Patients with cardiac damage had significantly higher damage rates.
Damage remains an important problem for patients with systemic vasculitis despite effective remission-inducing drugs. Only a small fraction of patients are unmarked by their disease.
通过在瑞典南部一个特定地理区域进行横断面时点患病率研究，评估韦格纳肉芽肿病（WG）、显微镜下多血管炎（MPA）、多动脉炎（PAN）和变应性肉芽肿性血管炎（CSS）患者不可逆器官损害的程度和模式。
对 86 例现患病例进行血管炎损伤指数（VDI）评估，这些病例从瑞典南部的一个特定人群中分类为 46 例 WG、27 例 MPA、9 例 PAN 和 4 例 CSS，中位年龄为 64.8 岁，中位疾病病程为 9 年。所有患者的 VDI 均在时点患病率（pp）日，即 2003 年 1 月 1 日确定。
所有患者的 VDI 评分中位数为 3[四分位距（IQR）2-5]：WG 为 3（2-4），MPA 为 3（1.5-4.5），PAN 为 5（2-6），CSS 为 1.5（0.75-2.75）。仅有 9%的患者未被分配任何一项损伤。最常见的损伤是心血管系统，其次是肾脏、神经精神、耳鼻喉（ENT）和肌肉骨骼系统。大血管和治疗相关的损伤与年龄较大有关，而 ENT 损伤在年轻患者中更为常见。ENT 损伤与心脏和肾脏损伤几乎完全分离，22 例 ENT 损伤患者中只有 2 例有肾脏损伤，无一例有心脏损伤。有心脏损伤的患者损伤发生率明显更高。
尽管有有效的诱导缓解药物，器官损害仍然是系统性血管炎患者的一个重要问题。只有一小部分患者未受到疾病的影响。
