Wells Athol U
Interstitial Lung Diseases Unit, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK.
BMC Med. 2016 Feb 10;14:23. doi: 10.1186/s12916-016-0562-1.
In principle, accurate guideline recommendations should lead to optimal management based on a secure diagnosis. However, current IPF diagnostic guidelines do not meet the needs of a major sub-group (possibly the majority) of patients with idiopathic pulmonary fibrosis (IPF). A great many IPF patients have HRCT appearances of "possible UIP". A surgical biopsy is very often impracticable due to age, disease severity, co-morbidities or patient refusal. A guideline-based diagnosis cannot be made in these patients, although the diagnosis is often obvious. Inflexible diagnostic criteria, although essential for treatment trials, must necessarily be structured around an inflexible diagnostic algorithm. With this approach, non-standardised information (i.e. not available in all patients) must be omitted, including observed disease behaviour prior to and on treatment, findings on bronchoalveolar lavage, likelihoods in relation to age and a wealth of ancillary clinical information. However, when a diagnosis cannot be made using guideline criteria, a probable or highly probable "working diagnosis" of IPF can and should be made in most IPF patients by means of clinical reasoning, integrating all available non-standardised information.
原则上,准确的指南建议应基于可靠的诊断得出最佳治疗方案。然而,目前的特发性肺纤维化(IPF)诊断指南无法满足大部分(可能是大多数)特发性肺纤维化患者的需求。许多IPF患者的高分辨率计算机断层扫描(HRCT)表现为“可能的寻常型间质性肺炎(UIP)”。由于年龄、疾病严重程度、合并症或患者拒绝,手术活检往往不可行。尽管诊断通常很明显,但这些患者无法依据指南进行诊断。虽然严格的诊断标准对于治疗试验至关重要,但必须围绕固定的诊断算法构建。采用这种方法时,必须省略非标准化信息(即并非所有患者都具备的信息),包括治疗前和治疗期间观察到的疾病行为、支气管肺泡灌洗结果、与年龄相关的可能性以及大量辅助临床信息。然而,当无法依据指南标准进行诊断时,大多数IPF患者可以且应该通过临床推理,整合所有可用的非标准化信息,做出可能或极有可能的IPF“暂定诊断”。