Celiac disease and idiopathic pulmonary hemosiderosis: a literature review of the Lane–Hamilton syndrome

ABSTRACT

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 (n = 56, 70%), dyspnea (n = 47, 58.8%), anemia (n = 72, 90%), and iron deficiency (n = 54, 67.5%) were most commonly observed. Medical history revealed recurrent episodes of hemoptysis (n = 38) and persistent iron deficiency anemia (n = 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.

KEYWORDS:

• Celiac disease
• idiopathic pulmonary hemosiderosis
• Lane–Hamilton syndrome
• hemoptysis
• anemia

Acknowledgments

None stated.

This study was announced at the CHEST Congress in June 2022 (EP372, AS24 Pulmonary Manifestations of Systemic Disease).

Declaration of funding

No funding was received for the production of this manuscript.

Disclosure of any financial/other conflicts of interest

The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Statement of Ethics

Research involving human participants: There are no records about human involvement according to GDPR (General Data Protection Regulation – 2016/679).