Barlo Nicole P, van Moorsel Coline H M, van den Bosch Jules M M, van de Graaf Ed A, Kwakkel-van Erp Johanna M, Grutters Jan C
St. Antonius Ziekenhuis, afd. Longziekten, Nieuwegein, The Netherlands.
Ned Tijdschr Geneeskd. 2009;153:B425.
To describe the clinical presentation, diagnosis, and prognosis of a cohort of Dutch patients with idiopathic pulmonary fibrosis (IPF), a serious and rapidly progressive lung disease belonging to the idiopathic interstitial pneumonias.
Retrospective study of patient records.
The data from the clinical presentation, diagnosis, treatment and survival of all patients with IPF, diagnosed in the St. Antonius Hospital in Nieuwegein and University Medical Center Utrecht (UMCU), both in the Netherlands, during the period 1998-2007 were investigated. For the diagnosis, the criteria of the American Thoracic Society and the European Respiratory Society from 2002 were adhered to.
The records of 113 patients satisfied the inclusion criteria. Mean age at the time of presentation was 61.9 (SD: 12.7) years and a strong male predominance was observed (90 men vs. 23 women). The most common complaints and symptoms at presentation were dyspnoea, cough, basal crepitations and clubbing of the nails. Lung function tests revealed restrictive lung function impairment and a reduced diffusing capacity. In 72% of cases the diagnosis IPF was histologically confirmed by open lung biopsy, which revealed a pattern of usual interstitial pneumonia (UIP). In 17% of patients it concerned a familial form of the disease with diagnosis at a younger age (average 52 years; SD: 14.8). The medical treatment mostly consisted of corticosteroids, which for half of the patients were administered in combination with an immunosuppressant such as azathioprine or cyclophosphamide. After screening, 28 patients were eligible for lung transplantation. Of these, 12 patients underwent a lung transplantation in the study period, 9 died and 7 are still on the waiting list. The median survival period was 3.9 years.
In the cohort studied, IPF presented as a rapidly progressive disease with only a marginal response to medical treatment and a poor prognosis. It is important to differentiate IPF from other fibrotic interstitial lung diseases and to refer to a specialist centre, especially in the case of patients who could be eligible for a lung transplant or for participation in trials with new drugs.
描述一组荷兰特发性肺纤维化（IPF）患者的临床表现、诊断及预后情况。IPF是一种属于特发性间质性肺炎的严重且进展迅速的肺部疾病。
对患者病历进行回顾性研究。
调查了1998年至2007年期间在荷兰新韦根的圣安东尼医院和乌得勒支大学医学中心（UMCU）确诊的所有IPF患者的临床表现、诊断、治疗及生存数据。诊断遵循2002年美国胸科学会和欧洲呼吸学会的标准。
113例患者的记录符合纳入标准。就诊时的平均年龄为61.9（标准差：12.7）岁，男性占主导（90例男性对23例女性）。就诊时最常见的主诉和症状为呼吸困难、咳嗽、肺底部啰音和杵状指。肺功能测试显示限制性肺功能损害和弥散能力降低。72%的病例经开胸肺活检组织学确诊为IPF，显示为普通型间质性肺炎（UIP）模式。17%的患者为家族性疾病形式，诊断时年龄较轻（平均52岁；标准差：14.8）。药物治疗主要包括皮质类固醇，半数患者联合使用硫唑嘌呤或环磷酰胺等免疫抑制剂。筛选后，28例患者符合肺移植条件。其中，12例患者在研究期间接受了肺移植，9例死亡，7例仍在等待名单上。中位生存期为3.9年。
在所研究的队列中，IPF表现为一种进展迅速的疾病，药物治疗反应甚微且预后不良。将IPF与其他纤维化间质性肺病区分开来并转诊至专科中心很重要；尤其是对于可能符合肺移植条件或参与新药试验的患者。
