Kelly David, Makkuni Damodar, Ail Dhiraj
Department of Medicine, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK.
Department of Rheumatology, James Paget University Hospital, Norwich, UK.
BMJ Case Rep. 2017 Aug 3;2017:bcr-2017-219235. doi: 10.1136/bcr-2017-219235.
A previously healthy 21-year-old young woman presented with worsening dyspnoea and haemoptysis. Imaging was suggestive of widespread pulmonary haemorrhage. There was no other organ system involvement in particular no evidence of renal involvement. Raised antimyeloperoxidase titres allowed diagnosis of isolated diffuse alveolar haemorrhage (DAH) secondary to microscopic polyangiitis (MPA). The patient rapidly deteriorated with worsening respiratory failure despite invasive mechanical ventilation and required extracorporeal membrane oxygenation (ECMO). This maintained the patient long enough to allow aggressive therapy in the form of immunosuppression and plasma exchange. She made a remarkable recovery and is asymptomatic 2 years on. Isolated DAH in the absence of renal disease is an atypical presentation of MPA and can lead to diagnostic uncertainty. A literature review reveals increasing reports of successful use of ECMO in severe DAH due to pulmonary vasculitis. Despite this, the need for systemic anticoagulation in the presence of pre-existing haemorrhage remains a challenging dilemma.
一名既往健康的21岁年轻女性出现进行性加重的呼吸困难和咯血。影像学检查提示广泛的肺出血。无其他器官系统受累,尤其是没有肾脏受累的证据。抗髓过氧化物酶滴度升高有助于诊断继发于显微镜下多血管炎(MPA)的孤立性弥漫性肺泡出血(DAH)。尽管进行了有创机械通气,患者仍因呼吸衰竭迅速恶化,需要体外膜肺氧合(ECMO)。这使患者维持了足够长的时间,以便进行免疫抑制和血浆置换等积极治疗。她恢复得非常好,2年后无症状。无肾脏疾病的孤立性DAH是MPA的非典型表现,可导致诊断不确定性。文献综述显示,越来越多的报告称ECMO成功用于治疗因肺血管炎导致的严重DAH。尽管如此,在已有出血存在的情况下进行全身抗凝的必要性仍然是一个具有挑战性的难题。