Saha Biplab K, Datar Praveen, Aiman Alexis, Bonnier Alyssa, Saha Santu, Milman Nils T
Pulmonary and Critical Care Medicine, Ozarks Medical Center, West Plains, USA.
Internal Medicine, New York Institute of Technology College of Osteopathic Medicine at Arkansas State University, Jonesboro, USA.
Cureus. 2022 Mar 25;14(3):e23482. doi: 10.7759/cureus.23482. eCollection 2022 Mar.
Idiopathic pulmonary hemosiderosis (IPH) causes diffuse alveolar hemorrhage (DAH) by a yet unknown mechanism. The coexistence of IPH and celiac disease (CD), also known as Lane-Hamilton syndrome (LHS), has been reported in both pediatric and adult patients. The objective of this study was to compare demographics, clinical and radiologic findings, treatment, and outcomes between adult patients with IPH and LHS. This is a systematic review of the literature. Multiple databases were searched using appropriate formulas to identify relevant articles. A total of 60 studies reporting 65 patients were included in the review. Forty-nine of these patients had IPH and 16 had LHS. The prevalence of anti-CD antibodies among tested patients was 13/22 (59%). The symptom onset and diagnosis of IPH occurred earlier in patients with LHS. The median delay in diagnosis was the same between the two groups (52 weeks). The classic triad was more likely to be present in patients with LHS. Only 20% of patients in the LHS cohort had any significant gastrointestinal (GI) symptoms at the time of IPH diagnosis. A gluten-free diet alone was effective in the majority of patients. Fewer patients in the LHS cohort received systemic corticosteroid than the IPH cohort. The recurrence and mortality in patients with LHS appear to be less than in the IPH cohort. The prevalence of CD is 25% in adult patients with IPH. Patients with LHS may have a milder course than patients without CD. Serologic testing for CD should be performed in all patients diagnosed with IPH.
特发性肺含铁血黄素沉着症（IPH）通过一种尚不明确的机制导致弥漫性肺泡出血（DAH）。IPH与乳糜泻（CD）共存，即所谓的莱恩 - 汉密尔顿综合征（LHS），在儿科和成年患者中均有报道。本研究的目的是比较成年IPH患者和LHS患者的人口统计学、临床和影像学表现、治疗及预后情况。这是一项系统性文献综述。使用适当公式检索多个数据库以识别相关文章。该综述共纳入60项研究报告的65例患者。其中49例为IPH患者，16例为LHS患者。检测患者中抗CD抗体的患病率为13/22（59%）。LHS患者中IPH的症状发作和诊断出现得更早。两组的诊断中位延迟时间相同（52周）。LHS患者更可能出现典型三联征。LHS队列中仅20%的患者在IPH诊断时出现任何明显的胃肠道（GI）症状。仅无麸质饮食对大多数患者有效。LHS队列中接受全身糖皮质激素治疗的患者比IPH队列少。LHS患者的复发率和死亡率似乎低于IPH队列。成年IPH患者中CD的患病率为25%。LHS患者的病程可能比无CD的患者更轻。所有诊断为IPH的患者均应进行CD的血清学检测。
