Transition to severe phenotype in systemic lupus erythematosus initially presenting with non-severe disease: implications for the management of early disease

Transition to severe phenotype in systemic lupus erythematosus initially presenting with non-severe disease: implications for the management of early disease
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DOI:
10.1136/lupus-2020-000394
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发表时间:
2020-01-01
影响因子:
3.9
通讯作者:
Fanouriakis, Antonis
Fanouriakis, Antonis
中科院分区:
医学3区
文献类型:
--
作者:
Nikolopoulos, Dionysis S.;Kostopoulou, Myrto;Fanouriakis, Antonis

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SLE患者护理的客观变化要求重新评估其自然病史以及疾病恶化和损害累积的危险因素。我们试图破译在最初表现为非严重疾病的患者中预测表型恶化(‘过渡’)的因素。根据不列颠群岛狼疮评估小组的表现和医生的判断,确诊时的疾病被分类为轻度、中度或重度。严重程度的“转换”被定义为从诊断到最后一次随访的任何时间严重程度的增加。多变量Logistic回归分析确定与这一转变相关的基线因素。结果462名患者获得了中位数36(120)个月的随访。确诊时,超过一半(56.5%)的患者有轻微的表型。在病程中,44.2%的患者过渡到更严重的形式,导致在最后一次随访时严重程度的分布相似(轻度28.4%,中度33.1%,重度38.5%)。起病时神经精神损害(OR 6.33,95%CI 1.22~32.67)、男性(OR 4.53,95%CI 1.23~16.60)和病程较长(每1年OR 1.09,95%CI 1.04~1.14)与轻、中、重度疾病的转归独立相关。病程<gt;=3年,进展为较严重疾病的患者发生不可逆转损害的风险增加20倍以上。结论最初非严重疾病的患者中,近一半进展为较严重形式的SLE,尤其是男性和发病时抗双链DNA阳性或神经精神疾病受累的患者。这些数据可能会对轻度狼疮的治疗产生影响。
Objective Changes in the care of patients with SLE dictate a re-evaluation of its natural history and risk factors for disease deterioration and damage accrual. We sought to decipher factors predictive of a deterioration in phenotype ('transition') in patients initially presenting with non-severe disease.Methods Patients from the 'Attikon' cohort with disease duration >= 1 year were included. Disease at diagnosis was categorised as mild, moderate or severe, based on the British Isles Lupus Assessment Group manifestations and physician judgement. 'Transition' in severity was defined as an increase in category of severity at any time from diagnosis to last follow-up. Multivariable logistic regression was performed to identify baseline factors associated with this transition.Results 462 patients were followed for a median (IQR) of 36 (120) months. At diagnosis, more than half (56.5%) had a mild phenotype. During disease course, transition to more severe forms was seen in 44.2%, resulting in comparable distribution among severity patterns at last follow-up (mild 28.4%, moderate 33.1%, severe 38.5%). Neuropsychiatric involvement at onset (OR 6.33, 95% CI 1.22 to 32.67), male sex (OR 4.53, 95% CI 1.23 to 16.60) and longer disease duration (OR 1.09 per 1 year, 95% CI 1.04 to 1.14) were independently associated with transition from mild or moderate to severe disease. Patients with disease duration >= 3 years who progressed to more severe disease had more than 20-fold increased risk to accrue irreversible damage.Conclusion Almost half of patients with initially non-severe disease progress to more severe forms of SLE, especially men and patients with positive anti-doublestranded DNA or neuropsychiatric involvement at onset. These data may have implications for the management of milder forms of lupus.