文摘

回顾性分析220例不明原因引起的fibrosing满足标准牙槽炎(CFA)参加主管布朗普顿医院1955年至1973年间进行了地铁站和患者随访4 - 21年。各种临床特征证实了先前的报道的频率。2:1在所有年龄组男性优势是相似的。演讲的平均年龄是54年±12 SD;202例(92%)患者出现呼吸困难,严重的肺活量减少相关(p < 0·003)和射线缤纷评分(p < 0·01),但其持续时间。百分之二十一的220年关节症状,10%有临床类风湿性关节炎。百分之十一的人有其他类型的结缔组织疾病。总的来说,有30%的多发性关节炎或其他免疫紊乱和孤独的CFA的70%。除了在多发性关节炎患者类风湿因子的增加,没有其他临床或生存那些差异和没有免疫疾病有关。205年百分之四十五的受试者抗核抗体,这同样发生在那些没有结缔组织紊乱有关。 One hundred and forty-seven initial radiographs were available for reclassification using the ILO/UC system and only three were normal. Small rounded opacities were seen in 16% and small irregular opacities in 84%. Pleural changes were uncommon. Histological confirmation had been obtained in 118 patients and material was still available for review using a semiquantitative analysis in 68 (biopsy 42 and necropsy 26). Of the biopsies one could be classified as desquamative interstitial pneumonia (DIP) and 17 as endstage fibrosis; the other 24 showed a mixed cellular and fibrotic pattern. The necropsy material showed much greater fibrosis and less acute inflammatory cellularity in spite of an interval between onset of symptoms and death of less than four years in 21 of 26 patients. One hundred and fifty-six patients have died (mean survival 3·2 years). Eleven (5%) are believed to be alive but have been lost to follow-up. Fifty-five per cent of deaths were attributable directly or indirectly to CFA. There was also an excess of deaths from cardiovascular disease and lung cancer. Using a life-table analysis and a log rank test, longer survival was seen in younger patients (p<0·001) and women (p<0·01). After correction for age and sex, lesser grades of dyspnoea (p<0·03) and lesser radiographic abnormality (p<0·001), absence of right axis deviation (p<0·001), and a higher Pao₂相关(p < 0·01)也不再生存。学科有更多的细胞组织学也存活时间(p < 0·02)。没有对生存的影响因素包括时间呼吸困难在演讲之前,FEV的减少程度₁、FVC和TLC,结缔组织疾病,自身抗体、吸烟史、咳嗽、痰,发出爆裂声,夜总会,ESR,或免疫球蛋白。