Division of Rheumatology, ERN RITA center, IRCCS Ospedale Pediatrico Bambino Gesù, Piazza Sant'Onofrio, 4-00165, Rome, Italy.
Pediatr Rheumatol Online J. 2021 Sep 16;19(1):144. doi: 10.1186/s12969-021-00635-w.
Pleural effusion in systemic lupus erythematous (SLE) is a common symptom, and recent studies demonstrated that IL-6 has a pivotal role in its pathogenesis.
We report a case of a 15 years old Caucasian boy with a history of persistent pleural effusion without lung involvement or fever. Microbiological and neoplastic aetiologies were previously excluded. Based on the presence of pleuritis, malar rash, reduction of C3 and C4 levels and positivity of antinuclear antibody (ANA) and anti-double stranded DNA (dsDNA), the diagnosis of juvenile SLE (JSLE) was performed. Treatment with high dose of intravenous glucocorticoids and mycophenolate mofetil was started with partial improvement of pleural effusion. Based on this and on adults SLE cases with serositis previously reported, therapy with intravenous tocilizumab (800 mg every two weeks) was started with prompt recovery of pleural effusion.
To the best of our knowledge, this is the first case of JSLE pleuritis successfully treated with tocilizumab.
红斑狼疮性胸腔积液（SLE）是一种常见症状，最近的研究表明，白细胞介素 6（IL-6）在其发病机制中起关键作用。
我们报告了一例 15 岁白人男孩，有持续胸腔积液病史，但无肺部受累或发热。先前已排除微生物和肿瘤病因。基于胸膜炎、蝶形红斑、C3 和 C4 水平降低以及抗核抗体（ANA）和抗双链 DNA（dsDNA）阳性，诊断为幼年系统性红斑狼疮（JSLE）。开始使用大剂量静脉注射糖皮质激素和吗替麦考酚酯治疗，胸腔积液部分改善。基于这一点以及之前报道的成人 SLE 伴浆膜炎的病例，开始使用静脉注射托珠单抗（每两周 800mg）治疗，胸腔积液迅速恢复。
据我们所知，这是首例成功用托珠单抗治疗的 JSLE 胸膜炎病例。
