CRYPTOGENIC FIBROSING ALVEOLITIS - RESPONSE TO CORTICOSTEROID TREATMENT AND ITS EFFECT ON SURVIVAL
CRYPTOGENIC FIBROSING ALVEOLITIS - RESPONSE TO CORTICOSTEROID TREATMENT AND ITS EFFECT ON SURVIVAL
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DOI：
10.1136/thx.35.8.593
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发表时间：
1980-01-01
期刊：
影响因子：
10
通讯作者：
JOHNSON, A
中科院分区：
文献类型：
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作者：
TURNERWARWICK, M;BURROWS, B;JOHNSON, A
[220]对隐源性纤维性肺泡炎(CFA)患者进行了回顾性研究。对他们进行了至少4年的随访。患者[77]没有接受治疗，143人接受了皮质类固醇治疗。两组之间唯一的临床差异是发病年龄(未经治疗的平均年龄61岁+-)。11例SD；治疗后平均年龄56岁+-。11标普；0.001)。激素治疗的影响从4-8wk后的临床、放射学和生理反应以及生存期方面进行评估，采用对数秩法分析。在143例接受治疗的病例中，127例有详细的随访信息。72人(57%)的呼吸困难有明显的主观改善，但只有22人(17%)有额外的客观改善。显著区分72名主观应答者和55名无应答者的唯一因素是较年轻的出现年龄(P<；0.001)和较少的呼吸困难(P<；0.02)。生存数据分析表明，早期对类固醇有良好反应的接受治疗的患者的存活率明显好于无反应的患者(P<；0.001)。未治疗组的生存曲线介于两个类固醇组之间。激素反应性显然与预后较好的亚组特别相关，该亚组的特点是年龄较小，可能具有脱屑间质性肺炎不一定典型的细胞组织学特征。治疗似乎对存活率有额外的影响，特别是在早期疾病中，这在未治疗的病例中没有观察到。许多因素与类固醇反应或生存无关。这些因素包括相关结缔组织疾病的存在、自身抗体和出现呼吸困难的持续时间。活检信息对评估对类固醇的潜在反应是有价值的。如果类固醇治疗要有帮助，就必须在早期使用。反应是非常多变的，根据单个患者的S反应滴定类固醇剂量可能会比遵循任意的剂量时间表得到更好的结果。
Patients [220] with cryptogenic fibrosing alveolitis (CFA) were retrospectively studied. They were followed-up for at least 4 yr. Patients [77] received no treatment and 143 received cortocosteroids. The only clinical difference between the groups was the age at presentation (untreated mean age 61 yr .+-. 11 SD; treated mean age 56 yr .+-. 11 SD P < 0.001). The influence of corticosteroid treatment was assessed in terms of the clinical, radiographic and physiological response after 4-8 wk and in terms of survival, using a log rank method of analysis. Of 143 treated cases, 127 had detailed follow-up information. Seventy-two (57%) had substantial subjective improvement of breathlessness but only 22 (17%) of the total showed additional objective improvement. The only factors significantly distinguishing the 72 subjective responders from the 55 non-responders and which might be used as predictors of response, were a younger age at presentation (P < 0.001) and less dyspnea (P < 0.02). Analysis of survival data demonstrated that treated patients who showed an early good response to steroids had a markedly better survival than non-responders (P < 0.001). The survival curve for the untreated group lay between the 2 steroid groups. Steroid responsiveness apparently relates particularly to a subgroup with an inherently better prognosis, which is characterized by a younger age and probably by a cellular histology not necessarily typical of desquamative interstitial pneumonia. Treatment appears to have an additional influence on survival, especially in early disease, which is not observed in the untreated cases. A large number of factors are not related to steroid responsiveness or to survival. These include the presence of associated connective tissue diseases, autoantibodies and the duration of dyspnea at presentation. Biopsy information is valuable in assessment of potential to respond to steroids. If steroid treatment is to be helpful, it must be used at an early stage. The response is very variable and titration of the steroid dose against an individual patient''s response is likely to give better results than adherence to arbitrary dose schedules.