Saha Biplab K, Aiman Alexis, Chong Woon Hean, Saha Santu, Song Jini, Bonnier Alyssa
Department of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Gainesville, Florida, USA.
New York Institute of Technology College of Osteopathic Medicine at Arkansas State University, Arkansas, USA.
Pediatr Pulmonol. 2023 Feb;58(2):382-391. doi: 10.1002/ppul.26230. Epub 2022 Nov 11.
This manuscript reports the recent advances in idiopathic pulmonary hemosiderosis (IPH), a rare cause of diffuse alveolar hemorrhage in children and adults. This narrative review of the literature summarizes different aspects of IPH, including proposed pathogenesis, patient demographics, clinical and radiological characteristics, treatment, and prognosis. Additionally, the association between Celiac Disease (CD) and IPH is carefully evaluated. IPH is a frequently misdiagnosed disease. The delay in the diagnosis of IPH is often significant but fortunately, appears to have decreased in recent years. IPH in adults and children have distinct demographic preferences. The autoantibodies are common in IPH but with a definite difference between the adult and pediatric populations. The definitive diagnosis of IPH requires lung biopsy and careful exclusion of all competing diagnoses, even with lung biopsy showing bland pulmonary hemorrhage. The presence of nonspecific inflammatory cells or lymphoid aggregates may suggest a secondary immunologic phenomenon and needs careful evaluation and follow-up. A substantial number of patients suffer from coexisting CD, also known as Lane-Hamilton syndrome (LHS), and all patients with IPH need to be evaluated for LHS by serology. Although strict gluten free diet can manage the majority of patients with LHS, other patients generally require immunosuppressive therapy. The corticosteroids are the backbone of IPH therapy. Recently utilized experimental treatment options include mesenchymal stem cell transplant, liposteroid and bronchial artery embolization. The immunosuppression should be adjusted to achieve optimal disease control. Patients may progress to end-stage lung disease despite all measures, and lung transplantation may be the only viable option.
本手稿报告了特发性肺含铁血黄素沉着症（IPH）的最新进展，这是儿童和成人弥漫性肺泡出血的罕见病因。这篇文献综述总结了IPH的不同方面，包括提出的发病机制、患者人口统计学、临床和放射学特征、治疗及预后。此外，还仔细评估了乳糜泻（CD）与IPH之间的关联。IPH是一种经常被误诊的疾病。IPH诊断的延迟往往很明显，但幸运的是，近年来这种情况似乎有所减少。成人和儿童的IPH在人口统计学上有明显的偏好。自身抗体在IPH中很常见，但成人和儿童群体之间存在明显差异。IPH的明确诊断需要进行肺活检，并仔细排除所有可能的竞争性诊断，即使肺活检显示为单纯性肺出血。非特异性炎症细胞或淋巴样聚集物的存在可能提示继发性免疫现象，需要仔细评估和随访。相当数量的患者患有并存的CD，也称为Lane-Hamilton综合征（LHS），所有IPH患者都需要通过血清学评估是否患有LHS。虽然严格的无麸质饮食可以治疗大多数LHS患者，但其他患者通常需要免疫抑制治疗。皮质类固醇是IPH治疗的主要药物。最近使用的实验性治疗方案包括间充质干细胞移植、脂质类固醇和支气管动脉栓塞。应调整免疫抑制以实现最佳的疾病控制。尽管采取了所有措施，患者仍可能进展为终末期肺病，肺移植可能是唯一可行的选择。
