Ferriani Mariana Paes Leme, Silva Marco Felipe Castro, Pereira Rosa Maria Rodrigues, Terreri Maria Teresa, Saad Magalhães Claudia, Bonfá Eloisa, Pastorino Antônio C, Carolina Dos Santos Maria, Appenzeller Simone, Ferriani Virginia Paes Leme, Len Claudio Arnaldo, Sallum Adriana Maluf Elias, Libório Jonatas, Monteiro de Castro Tânia Caroline, Silva Clovis Artur
Unit of Pediatric Rheumatology, Faculdade de Medicina da Universidade de Sx00E3;o Paulo (FMUSP), Sx00E3;o Paulo, Brazil.
Int Arch Allergy Immunol. 2015;167(3):186-92. doi: 10.1159/000438723. Epub 2015 Aug 25.
Data regarding the prevalence of chronic spontaneous urticaria (CSU) in childhood-onset systemic lupus erythematosus (cSLE) patients and possible associated factors are limited to a few case reports. The objectives of this study were to assess CSU in a large cSLE population, in order to evaluate the demographic data, clinical manifestations, disease activity/damage, laboratory abnormalities and treatment.
A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services and included 852 cSLE patients. CSU was diagnosed according to the guidelines of the European Academy of Allergy and Clinical Immunology, the Global Allergy and Asthma European Network, the European Dermatology Forum and the World Allergy Organization. Patients with CSU (evaluated at urticaria diagnosis) and patients without CSU (evaluated at the last visit) were assessed for lupus clinical/laboratory features and treatment.
CSU was observed in 10/852 cSLE patients (1.17%). The median of cSLE duration at urticaria diagnosis was 0 (-3 to 5) years. Comparison of cSLE patients with and without CSU revealed a greater frequency of constitutional symptoms (40 vs. 8%, p = 0.006), reticuloendothelial system involvement (30 vs. 3%, p = 0.003), mucocutaneous (90 vs. 28%, p < 0.0001) and musculoskeletal manifestations (50 vs. 6%, p < 0.0001) and methylprednisolone pulse therapy use (60 vs. 9%, p < 0.0001) in the former group. The frequency of immunosuppressive treatment was lower in patients with CSU (p = 0.018). The median SLE Disease Activity Index 2000 (12 vs. 2, p < 0.0001) and erythrocyte sedimentation rate (40 vs. 19 mm/1st hour, p = 0.024), was higher in patients with CSU.
To our knowledge, this is the first study with evidence that CSU may be linked to cSLE. We also demonstrated that this particular skin manifestation occurs predominantly at disease onset and is associated with lupus moderate/high disease activity without major organ involvement.
关于儿童期系统性红斑狼疮（cSLE）患者慢性自发性荨麻疹（CSU）的患病率及可能的相关因素的数据仅限于少数病例报告。本研究的目的是评估一大群cSLE患者中的CSU情况，以评估人口统计学数据、临床表现、疾病活动/损害、实验室异常及治疗情况。
在10个儿科风湿病服务机构进行了一项回顾性多中心队列研究（巴西cSLE组），纳入了852例cSLE患者。CSU根据欧洲变态反应和临床免疫学会、全球变态反应和哮喘欧洲网络、欧洲皮肤病学论坛及世界变态反应组织的指南进行诊断。对CSU患者（在荨麻疹诊断时评估）和无CSU患者（在最后一次就诊时评估）进行狼疮临床/实验室特征及治疗情况的评估。
在852例cSLE患者中有10例（1.17%）观察到CSU。荨麻疹诊断时cSLE病程的中位数为0（-3至5）年。对有和无CSU的cSLE患者进行比较发现，前一组中全身症状（40%对8%，p = 0.006）、网状内皮系统受累（30%对3%，p = 0.003）、皮肤黏膜（90%对28%，p < 0.0001）和肌肉骨骼表现（50%对6%，p < 0.0001）以及甲泼尼龙冲击疗法的使用（60%对9%，p < 0.0001）的频率更高。CSU患者免疫抑制治疗的频率较低（p = 0.018）。CSU患者的2000年系统性红斑狼疮疾病活动指数中位数（12对2，p < 0.0001）和红细胞沉降率（40对19 mm/第1小时，p = 0.024）更高。
据我们所知，这是第一项有证据表明CSU可能与cSLE相关的研究。我们还证明了这种特殊的皮肤表现主要发生在疾病发作时，且与狼疮中度/高度疾病活动相关而无主要器官受累。
