Flares in Biopsy-Proven Giant Cell Arteritis in Northern Italy: Characteristics and Predictors in a Long-Term Follow-Up Study
Flares in Biopsy-Proven Giant Cell Arteritis in Northern Italy: Characteristics and Predictors in a Long-Term Follow-Up Study
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DOI：
10.1097/md.0000000000003524
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发表时间：
2016-05-01
期刊：
影响因子：
1.6
通讯作者：
Salvarani, Carlo
中科院分区：
文献类型：
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作者：
Restuccia, Giovanna;Boiardi, Luigi;Salvarani, Carlo
本研究评估了一个大型队列的意大利活检证实的巨细胞动脉炎（GCA）患者的发作频率、时间和特征，并确定了诊断时能够预测发作发生的因素。我们评估了157例在雷焦艾米利亚医院（意大利）风湿科诊断并随访的经活检证实的透壁性GCA患者，这些患者从诊断到至少4年的随访都有足够的信息。57例患者（36.5%）发生1次发作。51（46.4%）的110总耀斑（88复发和22复发）的经验，在前2年诊断后。大多数复发发生在泼尼松10 mg/天的剂量下（82.9%），而只有3.4%的复发发生在25 mg/天的剂量下。风湿性多肌痛（46.5%）和颅脑症状（41.9%）是首次复发时最常见的表现。发作患者第一年的累积泼尼松剂量和总累积泼尼松剂量显著高于无发作患者（分别为7.82.4 vs 6.7 +/- 2.4g，P=0.02; 15.5 +/- 8.9 vs 10.0 +/- 9.2g，P=0.0001）。发作期患者泼尼松治疗的总持续时间较长（58 +/- 44 vs 30 +/- 30个月，P=0.0001）。疾病发作的患者在诊断时更常见全身表现（P=0.02）和发热38 ℃（P=0.02），血红蛋白水平显著降低（P=0.05），颞动脉活检（TAB）标本中更常见的巨细胞（P=0.04）和管腔内急性血栓形成（P=0.007），以及与无发作的患者相比更多的中度/重度动脉炎症（P=0.009）。在多变量模型中，发热38 ℃（风险比2.14; 95%置信区间，1.06-4.32，P=0.03）和炎症浸润的严重程度（中度/重度vs轻度）（风险比5.41; 95%置信区间，1.64-17.87，P=0.006）与发作风险增加显著相关。总之，在GCA中，燃烧过程是常见的，并且与长时间的GC需求相关。诊断时的发热和TAB时的炎症严重程度似乎可以预测疾病发作的发展。
This study evaluated the frequency, timing, and characteristics of flares in a large cohort of Italian patients with biopsy-proven giant cell arteritis (GCA) and to identify factors at diagnosis able to predict the occurrence of flares. We evaluated 157 patients with biopsy-proven transmural GCA diagnosed and followed at the Rheumatology Unit of Reggio Emilia Hospital (Italy) for whom sufficient information was available from the time of diagnosis until at least 4 years of follow-up. Fifty-seven patients (36.5%) experienced 1 flares. Fifty-one (46.4%) of the 110 total flares (88 relapses and 22 recurrences) were experienced during the first 2 years after diagnosis. The majority of relapses occurred with doses of prednisone 10mg/day (82.9%), whereas only 3.4% of relapses occurred for doses 25mg/day. Polymyalgia rheumatica (46.5%) and cranial symptoms (41.9%) were the most frequent manifestations at the time of the first relapse. Cumulative prednisone dose during the first year and total cumulative prednisone dose were significantly higher in flaring patients compared with those without flares (7.82.4 vs 6.7 +/- 2.4g, P=0.02; 15.5 +/- 8.9 vs 10.0 +/- 9.2g, P=0.0001, respectively). The total duration of prednisone treatment was longer in flaring patients (58 +/- 44 vs 30 +/- 30 months, P=0.0001).Patients with disease flares had at diagnosis more frequently systemic manifestations (P=0.02) and fever 38 degrees C (P=0.02), significantly lower hemoglobin levels (P=0.05), more frequent presence at temporal artery biopsy (TAB) specimens of giant cells (P=0.04) and intraluminal acute thrombosis (P=0.007), and more moderate/severe arterial inflammation (P=0.009) compared with those without flares. In the multivariate model fever 38 degrees C (hazard ratio 2.14; 95% confidence interval, 1.06-4.32, P=0.03) and the severity of inflammatory infiltrate (moderate/severe versus mild) (hazard ratio 5.41; 95% confidence interval, 1.64-17.87, P=0.006) were significantly associated with an increased risk of flares. In conclusion, a flaring course is common in GCA and it is associated with prolonged GC requirements. Fever at diagnosis and severity of inflammation at TAB appear to predict the development of disease flares.