Duke Pediatric Rheumatology, Box 3212 2301 Erwin Rd., Durham, NC, 27705, USA.
Curr Allergy Asthma Rep. 2020 Apr 4;20(5):13. doi: 10.1007/s11882-020-00907-7.
Idiopathic pulmonary hemosiderosis (IPH) is one of the rarest and least understood causes of pulmonary hemorrhage in children. Illustrated by a complex case presentation, we discuss the clinical manifestations, diagnosis, pathology, proposed etiologies, and treatment of this rare disease. We also compare IPH with anti-glomerular basement membrane antibody syndrome (anti-GBM disease), another rare causes of pediatric pulmonary hemorrhage.
Recent retrospective studies regarding IPH along with advanced immunotherapy have led to an improved understanding of how to best treat this condition, potential associations, and improved prognosis. Pathogenesis remains unknown, but several reports have suggested involvement of the alveolar capillary basement membrane. IPH is a poorly understood disease of unknown etiology that is a diagnosis of exclusion. Our patient was diagnosed with IPH after an exhaustive workup, including lung biopsy, into other immune-mediated causes of disease. While the pathogenesis of this rare disease remains elusive, our patient's immunofluorescent staining along the alveolar basement membrane without evidence of circulating antibody to type IV collagen raises the question of an immune-mediated pathogenesis of the disease with involvement of the alveolar basement membrane.
特发性肺含铁血黄素沉着症（IPH）是儿童肺部出血最罕见和了解最少的原因之一。通过一个复杂的病例介绍，我们讨论了这种罕见疾病的临床表现、诊断、病理学、提出的病因和治疗方法。我们还将 IPH 与抗肾小球基底膜抗体综合征（抗 GBM 病）进行了比较，后者也是儿童肺部出血的另一种罕见原因。
最近关于特发性肺含铁血黄素沉着症的回顾性研究以及先进的免疫疗法使人们对如何最好地治疗这种疾病、潜在的关联和改善预后有了更好的理解。发病机制仍不清楚，但有几份报告表明肺泡毛细血管基底膜参与其中。特发性肺含铁血黄素沉着症是一种病因不明的不明原因疾病，是一种排除性诊断。我们的患者在进行了详尽的检查后被诊断为特发性肺含铁血黄素沉着症，包括肺活检，以排除其他免疫介导的疾病原因。虽然这种罕见疾病的发病机制仍不清楚，但我们患者的肺泡基底膜免疫荧光染色没有循环抗Ⅳ型胶原抗体的证据，这引发了一个问题，即疾病的发病机制可能是免疫介导的，涉及肺泡基底膜。
