Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ, USA.
Mod Pathol. 2022 May;35(5):589-593. doi: 10.1038/s41379-022-01054-2. Epub 2022 Feb 24.
Usual interstitial pneumonia (UIP) is a concept that is deeply entrenched in clinical practice and the prognostic significance of UIP is well established, but the field continues to suffer from the lack of a true gold standard for diagnosing fibrotic interstitial lung disease (ILD). The meaning and usage of UIP have shifted over time and this term is prone to misinterpretation and poor diagnostic agreement. For pathologists, it is worth reflecting on the limitations of UIP and our true role in the care of patients with ILD, a controversial topic explored in two point-counterpoint editorials published simultaneously in this journal. Current diagnostic guidelines are ambiguous and difficult to apply in clinical practice. Further complicating matters for the pathologist is the paradigm shift that occurred with the advent of anti-fibrotic agents, necessitating increased focus on the most likely etiology of fibrosis rather than simply the pattern of fibrosis when pulmonologists select appropriate therapy. Despite the wealth of information locked in tissue samples that could provide novel insights into fibrotic ILDs, pulmonologists increasingly shy away from obtaining biopsies, likely because pathologists no longer provide sufficient value to offset the risks of a biopsy procedure, and pathologic assessment is insufficiently reliable to meaningfully inform therapeutic decisionmaking. To increase the value of biopsies, pathologists must first recognize the problems with UIP as a diagnostic term. Second, pathologists must realize that the primary goal of a biopsy is to determine the most likely etiology to target with therapy, requiring a shift in diagnostic focus. Third, pathologists must devise and validate new classifications and criteria that are evidence-based, biologically relevant, easy to use, and predictive of outcome and treatment response. Only after the limitations of UIP are understood will pathologists provide maximum diagnostic value from biopsies to clinicians today and advance the field forward.
特发性间质性肺炎（UIP）是临床实践中根深蒂固的概念，UIP 的预后意义已得到充分确立，但该领域仍缺乏诊断纤维化间质性肺疾病（ILD）的真正金标准。随着时间的推移，UIP 的含义和用法发生了变化，这个术语容易被误解，且诊断一致性较差。对于病理学家来说，值得反思 UIP 的局限性以及我们在ILD 患者护理中的真正作用，这是两个观点对立的社论在本期刊上同时发表，探讨了这个有争议的话题。目前的诊断指南存在歧义，难以在临床实践中应用。对于病理学家来说，进一步复杂化的是随着抗纤维化药物的出现而发生的范式转变，这需要更加关注纤维化的最可能病因，而不是当肺科医生选择合适的治疗方法时仅仅关注纤维化模式。尽管组织样本中蕴藏着大量信息，可以为纤维化 ILD 提供新的见解，但肺科医生越来越回避获取活检，可能是因为病理学家不再提供足够的价值来抵消活检程序的风险，而且病理评估的可靠性不足以对治疗决策产生有意义的影响。为了增加活检的价值，病理学家必须首先认识到 UIP 作为诊断术语存在的问题。其次，病理学家必须认识到活检的主要目的是确定最有可能的病因进行靶向治疗，这需要改变诊断重点。第三，病理学家必须设计和验证新的分类和标准，这些标准是基于证据、具有生物学相关性、易于使用，并可预测结果和治疗反应。只有在了解 UIP 的局限性之后，病理学家才能为今天的临床医生提供来自活检的最大诊断价值，并推动该领域向前发展。
