Sex-specific manifestations of Lofgren's syndrome
Sex-specific manifestations of Lofgren's syndrome
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DOI：
10.1164/rccm.200608-1197oc
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发表时间：
2007-01-01
影响因子：
24.7
通讯作者：
Eklund, Anders
中科院分区：
文献类型：
--
作者：
Grunewald, Johan;Eklund, Anders
动机：患者是否需要有结节性红斑才能被归类为洛夫格伦综合征一直存在争议。因此，在这项研究中，我们详细评估和比较了大量急性结节病和双侧肝门淋巴结病（BHL），伴或不伴结节性红斑（EN）的患者。这项研究很重要，因为它可能导致对洛夫格伦综合征更准确的定义，而准确的患者表型在现代医学研究中至关重要。背景：洛夫格伦综合征通常被认为是一种独特的临床症状。方法：我们详细评估了一大组（n 150）急性发作的结节病合并BHL的患者，其中大多数病例伴有发烧、EN和/或双侧踝关节关节炎或关节周围炎症。在该组中，87例患者有EN （EN阳性），而63例患者没有EN （EN阴性），尽管有明显的对称踝关节炎症。结果：en阳性和en阴性患者在各方面都相同，除了en阳性组的女性明显更多：en阳性组有58名女性（67%），而en阴性组只有17名女性（27%）（p < 0.0001）。在其他方面，如年龄、吸烟习惯、疾病发病的季节性聚集性、活检阳性率、胸片、肺功能、支气管肺泡灌洗液细胞分布（包括典型的CD4/CD8比值升高）和疾病的临床发展，en阳性组和en阴性组几乎相同。两组与HILA- drb1 *0301/DQB1*0201的相关性也相同，在en阳性和en阴性组中分别有60例（69.0%）和44例（69.8%）患者具有这种特殊的HILA类型。这类患者恢复的程度相同，即几乎是100%。结论：我们得出的结论是，洛夫格伦综合征的表现在男性和女性之间存在差异，EN主要发生在女性身上，而踝关节周围明显的炎症或踝关节关节炎则主要发生在男性身上。
Motivation: It has been debated whether patients need to have erythema nodosum to be classified as having Lofgren's syndrome. In this study, we have therefore in detail evaluated and compared a large number of patients with an acute onset of sarcoidosis and bilateral hilar lymphadenopathy (BHL), with or without erythema nodosum (EN). This study is important because it may lead to a more accurate definition of Lofgren's syndrome, and an exact phenotype of patients is crucial in modern medical research.Background: Lofgren's syndrome is commonly regarded as a distinct clinical entity. Methods: We have in detail evaluated a large group of patients (n 150) with an acute onset of sarcoidosis with BHL, in most cases with fever, EN, and/or bilateral ankle arthritis or periarticular inflammation. Within this group, 87 patients had EN (EN positive), whereas 63 were without EN (EN negative), though with distinct symmetric ankle inflammation.Results: EN-positive and EN-negative patients were identical in every aspect except that there were significantly more women in the EN-positive group: 58 women (67%) in the EN-positive group compared with only 17 (27%) women in the EN-negative group (p < 0.0001). In all other aspects, such as age, smoking habits, seasonal clustering of disease onset, rate of positive biopsies, chest radiography, pulmonary function, bronchoalveolar lavage cell distributions including the typically increased CD4/CD8 ratio, and clinical development of the disease, the EN-positive and EN-negative groups were close to identical. The two groups were also identically strongly associated with HILA-DRB1*0301/DQB1*0201, with 60 (69.0%) and 44 (69.8%) patients having this particular HILA type in the EN-positive and EN-negative groups, respectively. Such patients recovered to the same degree-that is, at almost 100%.Conclusions: We conclude that manifestations of Lofgren's syndrome differ between men and women, with EN found predominantly in women, whereas a marked periarticular inflammation of the ankles or ankle arthritis without EN is seen preferentially in men.