Steffensen I E, Rasmussen F V, Nørgaard T
Medicinsk afdeling, Centralsygehuset Hillerød.
Ugeskr Laeger. 1992 Jan 6;154(2):83-7.
Idiopathic pulmonary fibrosis (IPF) was found to have a higher incidence than previously assumed and the population involved was younger. These findings were made in a retrospective investigation during the period 1986-1990. The condition should be suspected on the basis of the radiographic characteristics, reduced lung volume (TLC), decreased DLco during testing of pulmonary function, hypoxemia at rest and also the symptoms revealed in the medical history. It is of importance for planning of treatment that the diagnosis of IPF is confirmed early by excision biopsy of pulmonary tissue. Patients with IPF were treated successfully by intravenous administration of a solution of 500 mg methylprednisolone in 500 ml isotonic glucose. This regime is administered for three consecutive days and supplemented by azathioprine as required. In order to prevent further progression of this chronic disease, patients with IPF should be followed-up at frequent intervals.
特发性肺纤维化（IPF）的发病率高于先前的推测，且患病群体更为年轻。这些发现来自于1986年至1990年期间的一项回顾性调查。根据影像学特征、肺容积减少（TLC）、肺功能测试期间DLco降低、静息时低氧血症以及病史中显示的症状，应怀疑患有此病。通过肺组织切除活检尽早确诊IPF对治疗方案的制定至关重要。特发性肺纤维化患者通过静脉注射500毫克甲泼尼龙溶于500毫升等渗葡萄糖的溶液成功得到治疗。该方案连续给药三天，并根据需要补充硫唑嘌呤。为防止这种慢性病进一步发展，特发性肺纤维化患者应定期接受随访。
