McKinney Duncan A, Kosnik Noah R, Syed Shahzeib, Junko Gretchen
LewisGale Medical Center, Salem, Virginia.
HCA Healthc J Med. 2025 Feb 1;6(1):11-21. doi: 10.36518/2689-0216.1802. eCollection 2025.
Description Idiopathic pulmonary fibrosis (IPF) is a progressive disease characterized by gradual destruction and replacement of pulmonary parenchyma with fibrous tissue, which occurs in conjunction with chronic inflammation. It is often considered a prototypical interstitial lung disease and is both the most prevalent and perhaps the most dangerous in that family. Although the disease is uncommon in the general population, its prevalence increases with age and is typically diagnosed around the age of 65. This does not preclude the development of IPF in younger individuals, and the mean survival is 2 to 5 years post-diagnosis regardless of age. Contemporary studies have provided insight into how altered pulmonary parenchyma results in increased susceptibility to opportunistic infections. It has also been demonstrated that pulmonary insults that cause inflammation, such as pneumonia, may accelerate the progression of IPF. Eosinophilic pneumonias are a collection of pulmonary diseases in which eosinophil-mediated inflammation results in respiratory compromise. Early recognition and appropriate intervention are imperative to minimize the risk of residual pulmonary function deficits, a risk that is increased in individuals with separate pulmonary risk factors. While prompt diagnosis and pharmacologic interventions are associated with improved outcomes, patients with IPF remain at risk of deterioration to the point of requiring lung transplantation. Early screening for those at risk continues to be a topic of interest. Despite the prevalence of IPF, its pathogenesis remains poorly understood and few management options are available. In this article, we document a unique case of previously undiagnosed IPF in a young individual that acutely worsened in the setting of acute eosinophilic pneumonia and the involvement of an opportunistic organism, . The case section will serve as a transition into a discussion of each of the major pathologic factors at play, supported by a review of recent literature.
特发性肺纤维化（IPF）是一种进行性疾病，其特征是肺实质逐渐被纤维组织破坏和取代，并伴有慢性炎症。它常被视为典型的间质性肺疾病，是该类疾病中最常见且可能最危险的一种。尽管该疾病在普通人群中并不常见，但其患病率随年龄增长而增加，通常在65岁左右被诊断出来。这并不排除年轻个体患IPF的可能性，且无论年龄大小，确诊后的平均生存期为2至5年。当代研究已深入了解了肺实质改变如何导致对机会性感染的易感性增加。还已证明，诸如肺炎等引起炎症的肺部损伤可能会加速IPF的进展。嗜酸性粒细胞性肺炎是一组肺部疾病，其中嗜酸性粒细胞介导的炎症会导致呼吸功能受损。早期识别和适当干预对于将残留肺功能缺陷的风险降至最低至关重要，在存在其他肺部危险因素的个体中，这种风险会增加。虽然及时诊断和药物干预与改善预后相关，但IPF患者仍有病情恶化至需要进行肺移植的风险。对高危人群进行早期筛查仍然是一个受关注的话题。尽管IPF很常见，但其发病机制仍知之甚少，且可用的治疗方案很少。在本文中，我们记录了一例年轻个体中先前未被诊断出的IPF的独特病例，该病例在急性嗜酸性粒细胞性肺炎和机会性生物体感染的情况下急性恶化。病例部分将作为一个过渡，引入对每个起作用的主要病理因素的讨论，并辅以近期文献综述。
