Pulmonary Department, "G. Papanikolaou" General Hospital, Thessaloniki, Greece.
UniPi Department, Athens, Greece.
Postgrad Med. 2022 Nov;134(8):732-742. doi: 10.1080/00325481.2022.2109121. Epub 2022 Aug 8.
Lane-Hamilton syndrome (LHS) presents a medical emergency, with 14% mortality due to Idiopathic Pulmonary Hemosiderosis (IPH) in acute phase. Despite the clinical severity of this entity, there has been no published review in the international literature, resulting in lack of awareness and delayed diagnosis.A rigorous search of international databases yielded a total of 80 LHS cases from January 1971 to August 2020. We analyzed 44 children (8.56 ± 4.72 years, 21 boys) and 36 adults (33.61 ± 13.41 years, 12 men) to present the clinical manifestations, radiological and immunological pattern, therapeutic approaches and outcome of LHS. We also elaborated on clinical and laboratory findings' associations to propose diagnostic indexes and clarified differences based on age distribution.Celiac disease (CD) and IPH diagnosis was made concurrently in 46 patients, whereas in 21 patients, the diagnosis of LHS was delayed for 2.5y (3 months-11 years). Hemoptysis ( = 56, 70%), dyspnea ( = 47, 58.8%), anemia ( = 72, 90%), and iron deficiency ( = 54, 67.5%) were most commonly observed. Medical history revealed recurrent episodes of hemoptysis ( = 38) and persistent iron deficiency anemia ( = 25) in need of multiple blood transfusions or iron supplementation. Patchy infiltrate opacities to consolidation predominated in children, whereas bilateral diffuse ground-glass opacities in adults. Duodenal biopsy was performed in 66 cases (diagnostic 87.8%), BAL in 51 (diagnostic 74.5%), and surgical lung biopsy in 20. Anti-tTG titer was positive in all 24 (54.6%) children and 19 (52.8%) adults that documented this assay. Prednisone or methylprednisolone pulse therapy and GFD were initiated in the acute phase, whereas chronic therapy included GFD, along with long-term prednisone in refractory cases. Three cases with severe respiratory failure or hemodynamic instability were intubated and a further three succumbed.A thorough understanding of LHS may reveal further diagnostic indexes and a consensus on therapy guidelines. Screening for CD is essential in all IPH cases for timely recognition and favorable outcome.
Lane-Hamilton 综合征（LHS）表现为医学急症，14%的患者因特发性肺含铁血黄素沉着症（IPH）在急性期死亡。尽管该实体的临床严重程度很高，但国际文献中尚未发表过综述，导致人们对此认识不足，诊断延迟。我们严格检索了国际数据库，自 1971 年 1 月至 2020 年 8 月共获得了 80 例 LHS 病例。我们分析了 44 例儿童（8.56±4.72 岁，21 名男性）和 36 例成人（33.61±13.41 岁，12 名男性）的临床表现、影像学和免疫学模式、治疗方法和结果。我们还详细阐述了临床和实验室检查结果的相关性，提出了诊断指标，并根据年龄分布阐明了差异。46 例患者同时诊断为乳糜泻（CD）和 IPH，而 21 例患者 LHS 的诊断延迟了 2.5 年（3 个月至 11 年）。咯血（=56，70%）、呼吸困难（=47，58.8%）、贫血（=72，90%）和缺铁（=54，67.5%）最为常见。病史显示，38 例患者反复咯血，25 例患者持续性缺铁性贫血，需要多次输血或补铁。儿童以斑片状浸润影至实变影为主，而成人则以双侧弥漫性磨玻璃影为主。66 例（诊断率 87.8%）进行了十二指肠活检，51 例（诊断率 74.5%）进行了 BAL，20 例进行了外科肺活检。24 例（54.6%）儿童和 19 例（52.8%）成人的抗 tTG 滴度均为阳性，该试验证实了这一点。在急性期开始使用泼尼松或甲基强的松龙脉冲治疗和无麸质饮食（GFD），而慢性治疗包括 GFD 以及难治性病例的长期泼尼松治疗。3 例严重呼吸衰竭或血流动力学不稳定的患者进行了插管，另有 3 例患者死亡。深入了解 LHS 可能会发现进一步的诊断指标，并就治疗指南达成共识。所有 IPH 患者均应筛查 CD，以便及时发现并获得良好的预后。
