Comparative Analysis of Adult Patients With Idiopathic Pulmonary Hemosiderosis and Lane-Hamilton Syndrome: A Systematic Review of the Literature in the Period 1971-2022

Abstract

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.

Updates in idiopathic pulmonary hemosiderosis in 2022: A state of the art review

“…Patients diagnosed with IPH should routinely undergo serologic testing for CD at the time of diagnosis regardless of GI symptoms. The risk of recurrence and death in patients with LHS showed a lower trend than patients without CD (the difference did not reach statistical significance) 42 …”

“…Despite being more common in girls, pediatric patients with LHS demonstrate a slight preference for boys (56.5%) 43 . Nearly 80% of adult patients with LHS were found to be males in one study 42 . However, another review reported a higher incidence of LHS in adult women 44 .…”

“…In contrast, nearly 80% of adult patients demonstrate all three manifestations 3 . The classic triad is present in 93.8% of adult LHS patients 42 …”

“…Lane and Hamilton in 1971, these patients are often referred to as LHS 41 . Adult patients with LHS appear to be younger than patients without CD 42 . Interestingly, pediatric patients with LHS were relatively older, the mean and median age being 10.5 and 11 years, respectively 43 .…”

The Spectrum of Autoantibodies in Adult Patients With Idiopathic Pulmonary Hemosiderosis: A Brief Review of the Literature

“…We have recently discussed the prevalence of autoantibodies in a cohort of adult IPH patients as reported in the literature [ 13 ]. Similar to pediatric patients, a majority of these patients had antibodies suggestive of celiac disease (CD), a combination known as Lane Hamilton syndrome (LHS) [ 12 , 14 , 15 ].…”