Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH, UK.
BMC Pulm Med. 2019 Aug 27;19(1):161. doi: 10.1186/s12890-019-0919-2.
CTD-related pleural effusions are rare and challenging to diagnose. Our lung inflammation service (with expertise in rheumatology, interstitial lung disease and respiratory failure) works closely with the pleural team. This study aims to review the multidisciplinary approach to CTD-related pleural effusions at a tertiary centre.
All patients with CTD-related pleural effusions at St Thomas' Hospital, London were included. Retrospective data were collected from Dec 2013 to 2016.
The lung inflammation service performed an expert clinical assessment and targeted investigations. 11 patients (ages 23-77) were identified with CTD related pleural disease. 9 (82%) patients were given a new CTD diagnosis, with pleural disease as the first manifestation. The range of conditions were: rheumatoid arthritis [3] ,IgG4-related disease [2] ,adult Still's disease [2] ,vasculitis [1] ,SLE [1] ,drug-induced lupus [1] ,and Behcet's [1]. The pleural team review took place 1 day (median) after referral. 73% of diagnoses (8 patients) were achieved with local anaesthetic pleural interventions (a combination of: aspiration, drain, or percutaneous biopsy). This included 1 patient who required no pleural intervention. 1 required medical thoracoscopy, and 2 underwent thoracic surgery. Diagnoses were made by integrating all available evidence such as clinical assessment, imaging, and autoimmune serology. No diagnosis was achieved by pleural cytology or histology analysis alone. 8 (73%) were commenced on prednisolone acutely (vasculitis, SLE, drug-related lupus, 1 patient with rheumatoid arthritis, Behcet's, 2 patients with Adult Still's disease, 1 patient with IgG4-related disease). Of these 8, one patient with rheumatoid arthritis received IV methylprednisolone beforehand, one patient with IgG4-related disease was weaned off prednisolone to methothrexate, two patients with Adult Still's disease were on colchicine as well, and one patient with Behcet's was on cyclophosphamide as well. 7 (64%) were managed as outpatients; 4 required admission. The median time from pleural review to diagnosis was 53 days.
Diagnosis can be challenging in patients presenting with pleural disease as the first manifestation of a CTD. We recommend a multidisciplinary approach in management.
与 CTD 相关的胸腔积液很少见，诊断具有挑战性。我们的肺部炎症服务团队（擅长风湿病学、间质性肺病和呼吸衰竭）与胸腔团队密切合作。本研究旨在回顾伦敦圣托马斯医院的多学科方法治疗 CTD 相关胸腔积液。
纳入所有在伦敦圣托马斯医院患有 CTD 相关胸腔积液的患者。从 2013 年 12 月至 2016 年，收集回顾性数据。
肺部炎症服务团队进行了专业的临床评估和有针对性的检查。确定了 11 例（年龄 23-77 岁）与 CTD 相关胸腔疾病的患者。9 例（82%）患者被诊断为新的 CTD，胸腔疾病为首发表现。所涉及的疾病范围为：类风湿关节炎[3]、IgG4 相关疾病[2]、成人Still 病[2]、血管炎[1]、系统性红斑狼疮[1]、药物诱导性狼疮[1]和贝赫切特病[1]。胸腔团队的评估在转诊后 1 天（中位数）进行。73%（8 例）的诊断是通过局部麻醉性胸腔介入治疗（包括：抽吸、引流或经皮活检）实现的。这包括 1 例患者无需进行胸腔介入治疗。1 例患者需要进行内科胸腔镜检查，2 例患者进行了胸外科手术。诊断是通过整合所有可用的证据，如临床评估、影像学和自身免疫血清学。单纯胸腔细胞学或组织学分析不能做出诊断。8 例（73%）患者立即接受泼尼松龙治疗（血管炎、系统性红斑狼疮、药物相关狼疮、1 例类风湿关节炎、贝赫切特病、2 例成人Still 病、1 例 IgG4 相关疾病）。这 8 例中，1 例类风湿关节炎患者之前接受了静脉甲基泼尼松龙治疗，1 例 IgG4 相关疾病患者已从泼尼松龙减至甲氨蝶呤，2 例成人 Still 病患者还接受了秋水仙碱治疗，1 例贝赫切特病患者还接受了环磷酰胺治疗。7 例（64%）作为门诊患者进行管理；4 例需要住院治疗。从胸腔评估到诊断的中位时间为 53 天。
以胸腔疾病为首发表现的 CTD 患者的诊断可能具有挑战性。我们建议采用多学科方法进行治疗。
