P105 自身炎症性特发性浆膜炎：一种特征不足的现象？,Rheumatology

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P105 自身炎症性特发性浆膜炎：一种特征不足的现象？
Rheumatology ( IF 4.7 ) Pub Date : 2021-04-26 , DOI: 10.1093/rheumatology/keab247.102
Kavya Pillai ₁ , James Perera ₁ , Dominik Kurzeja ₁ , Sanaah Amin ₂ , Maahia Choudhury ₂ , Richard Stratton ₁

Affiliation

背景/目的浆膜炎是一个广义的术语，包括心包、胸膜或腹膜的炎症。浆膜炎可继发于各种疾病，包括系统性红斑狼疮、炎症性肠病和家族性地中海热。由于多种诱因，特发性浆膜炎是一种排除性诊断，需要大量的医学检查。本病例系列详细介绍了在过去 12 年中在伦敦三级风湿病学中心皇家自由医院发生炎症性浆膜炎但没有明确潜在诱因的患者。方法通过电子病历检索2008-2020年的门诊信函和出院信函，对患者进行回顾性识别。检索词包括浆膜炎、心包炎、胸膜炎和腹膜炎。收集的数据包括他们的主要人口统计细节、实验室和病理学调查以及治疗过程和随访数据，包括复发率、并发症和生存结果。有潜在风湿病、免疫或恶性原因的患者被排除在外。结果 12 岁以上浆膜炎患者共 420 例，其中 22 例患者被归类为特发性、自身炎症性浆膜炎。患者人口统计数据表明，平均年龄为 61 岁（范围 19-87 岁）和高加索种族（68.2%，15/22）的女性占 5:2 的优势。对患者的潜在原因进行了调查，包括筛查感染和自身免疫状况，并进行了成像，包括计算机断层扫描和正发射断层扫描。自身免疫筛查显示 18.2% (4/22) 患者的弱阳性自身抗体（抗中性粒细胞抗体和类风湿因子）敏感性低。治疗选择各不相同，通常是通过类固醇治疗（口服泼尼松龙或静脉注射甲基泼尼松龙）急性开始免疫抑制，这取决于在进展为类固醇保留药物之前疾病表现的严重程度，包括霉酚酸酯（50%，11/22）、环磷酰胺（13.6 %, 3/22) 和硫唑嘌呤 (9.1%, 2/22)。使用了其他疗法，包括秋水仙碱 (40.9%, 9/22)。一旦患者使用类固醇节约剂，复发相对少见（21.7%，5/22）。然而，Anakinra（白细胞介素-1 受体拮抗剂）用于复发性复发（9.1%，2/22）且反应良好的患者。一名患者在环磷酰胺治疗后死于支气管肺炎。值得注意的是，3/22 (13.6%) 患者入院期间血流动力学受损的患者需要进行心包剥离术，其中 1 名患者随后需要 Anakinra 治疗。结浆膜炎发作。这种情况似乎对类固醇、类固醇节约剂和阿那白滞素的免疫抑制反应良好。需要进一步的工作来描述和分类这种情况，并制定治疗指南。披露 K. Pillai：没有。J.佩雷拉：没有。D. Kurzeja：没有。S.阿明：没有。M.乔杜里：没有。R. 斯特拉顿：没有。

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P105 Auto-inflammatory idiopathic serositis: an under-characterised phenomenon?

Background/Aims Serositis is a broad term encompassing inflammation of the pericardium, pleural or peritoneum. Serositis can be secondary to various conditions ranging from systemic lupus erythematous, inflammatory bowel disease and familial Mediterranean fevers. Due to the multitude of precipitating causes, idiopathic serositis is a diagnosis of exclusion and requires extensive medical work-up. This case series details patients who have developed inflammatory serositis without a clear underlying precipitating cause over the past 12 years at the Royal Free Hospital, a tertiary rheumatology centre based in London. Methods Patients were identified retrospectively through electronic records by searching through clinic letters and discharge letters from 2008 to 2020. Search terms including serositis, pericarditis, pleuritis and peritonitis were used. Data collected included their key demographic details, laboratory and pathology investigations as well as the course of treatment and follow up data, including rate of recurrence, complication and survival outcomes. Patients with underlying rheumatological, immunological, or malignant causes were excluded. Results Four hundred and twenty patients over 12 years with serositis were identified with 22 patients classified as idiopathic, auto-inflammatory serositis. Patient demographics indicate a 5:2 female preponderance for the condition with an average age of 61 (range 19-87) and Caucasian ethnicity (68.2%, 15/22). Patients were investigated for underlying causes, including screening for infection and autoimmune conditions, and imaging was performed including computer tomography and positive emission tomography. Autoimmune screening revealed weakly positive autoantibodies of low sensitivity (anti-neutrophil antibodies and rheumatoid factor) in 18.2% (4/22) patients. Treatment options varied, typically with immunosuppression being initiated acutely with steroid therapy, either oral prednisolone or intravenous methylprednisolone, depending on severity of disease presentation before progression to steroid-sparing agents, including mycophenolate motefil (50%, 11/22), cyclophosphamide (13.6%, 3/22) and azathioprine (9.1%, 2/22). Other therapies including colchicine (40.9%, 9/22) were used. Relapse was relatively uncommon once patients were established on steroid-sparing agents (21.7%, 5/22). However, Anakinra (interleukin-1 receptor antagonist) was initiated in patients with recurrent relapses (9.1%, 2/22) with good response. One patient died through bronchopneumonia following cyclophosphamide therapy. Of note, pericardial stripping was required in patients with haemodynamic compromise during admission in 3/22 (13.6%) with 1 patient in this subset subsequently requiring Anakinra therapy. Conclusion Auto-inflammatory idiopathic serositis appears to be distinct category of serositis affecting predominantly middle aged to elderly female Caucasian patients, without underlying connective tissue disease, requiring prolonged therapy with immunosuppressive agents, which can be characterised by involvement of multiple serous membranes and/or recurrent episodes of serositis. This condition appears to respond well to immune suppression with steroids, steroid-sparing agents and Anakinra. Further work will be required to characterise and classify this condition and to establish treatment guidelines. Disclosure K. Pillai: None. J. Perera: None. D. Kurzeja: None. S. Amin: None. M. Choudhury: None. R. Stratton: None.

更新日期：2021-04-26

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