Improved Outcome in 445 Patients With Wegener's Granulomatosis in a German Vasculitis Center Over Four Decades
Improved Outcome in 445 Patients With Wegener's Granulomatosis in a German Vasculitis Center Over Four Decades
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DOI：
10.1002/art.27763
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发表时间：
2011-01-01
影响因子：
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通讯作者：
Reinhold-Keller, Eva
中科院分区：
文献类型：
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作者：
Holle, Julia U.;Gross, Wolfgang L.;Reinhold-Keller, Eva
Objective.确定在一所专门从事风湿病学的学术性医院中40年以上韦格纳肉芽肿（WG）患者的长期结局。我们纳入了290例患者，将其分为2个队列，并将其与155例患者的历史队列进行比较。回顾性比较疾病的表现，治疗，死亡率和恶性肿瘤的发生率。历史队列（队列1）包括1966 - 1993年诊断的155例患者，队列2包括1994 - 1998年诊断的123例患者，队列3包括1999 - 2002年诊断的167例患者。随着时间的推移，首次症状和诊断之间的间隔缩短了一半（从8个月到4个月）。3个队列的器官表现相似，超过80%的患者仍需要环磷酰胺（CYC）;然而，中位累积剂量显著降低（从队列1的67 gm降至队列2的36 gm，再降至队列3的24 gm）。标准化死亡率（SMR）下降（从队列1的2.1至队列2的1.41和队列3的1.03），与WG和/或治疗相关的死亡较少（队列1为86.4%，队列2为76.9%，队列3为50%），复发率降低（队列1为63.9%，队列2为51.2%，队列3为35.3%），恶性肿瘤发生率未增加。与年轻女性相比，年轻男性的SMR明显更高（8.87 [95%可信区间4.05-16.8]），肾脏表现更频繁（54.4%比33.8%）。WG患者的死亡率在过去40年中下降，可能是由于诊断和治疗程序的改进以及对WG认识的提高，这导致了早期诊断和治疗，复发率降低，累积CYC剂量降低，与治疗相关的死亡减少。
Objective. To determine the long-term outcome in patients with Wegener's granulomatosis (WG) over 4 decades in an academic hospital unit specializing in rheumatology.Methods. We included 290 patients, divided them into 2 cohorts, and compared them with the historical cohort of 155 patients. Comparisons were retrospective regarding disease manifestations, therapy, mortality, and incidence of malignancies. The historical cohort (cohort 1) included 155 patients diagnosed between 1966 and 1993, cohort 2 included 123 patients diagnosed between 1994 and 1998, and cohort 3 included 167 patients diagnosed between 1999 and 2002.Results. Over time, the interval between first symptoms and diagnosis was reduced by half (from 8 months to 4 months). Organ manifestations were similar in the 3 cohorts, and more than 80% of patients still required cyclophosphamide (CYC); however, the median cumulative dose was reduced significantly (from 67 gm in cohort 1 to 36 gm in cohort 2 and to 24 gm in cohort 3). The standardized mortality ratios (SMRs) declined (from 2.1 in cohort 1 to 1.41 in cohort 2 and to 1.03 in cohort 3), with fewer deaths related to WG and/or therapy (86.4% in cohort 1, 76.9% in cohort 2, 50% in cohort 3), decreasing relapse rates (63.9% in cohort 1, 51.2% in cohort 2, 35.3% in cohort 3), and no increased rate of malignancies. Compared with young females, young males had a considerably higher SMR (8.87 [95% confidence interval 4.05-16.8]) and more frequent renal manifestations (54.4% versus 33.8%).Conclusion. Mortality of WG patients declined over the last 4 decades, probably due to improved diagnostic and therapeutic procedures and increased awareness of WG, which led to earlier diagnosis and therapy, reduction in relapse rates, and lower cumulative CYC dose with fewer deaths related to therapy.