title: Idiopathic pulmonary haemosiderosis with celiac disease (Lane-Hamilton syndrome) in an adult - a case report.
abstract: Idiopathic pulmonary haemosiderosis (IPH) is a rare disorder of unknown cause characterised by haemoptysis, diffuse alveolar infiltrates and iron-deficiency anaemia. IPH predominantly affects children; it is rare in adults, in whom it usually manifests before 30 years. In adults, course is protracted with a better prognosis, in contrast to children. Even rarer is the Lane-Hamilton syndrome, a condition in which IPH is associated with celiac disease. Only 15 cases of Lane-Hamilton syndrome affecting adults are reported in literature. Treatment of IPH is based on anecdotal case reports and case series because of its rare occurrence. High-dose steroids reportedly reduce morbidity and mortality and delays or stops disease progression; more effectively in adults than children. In Lane-Hamilton syndrome, a gluten-free diet for the celiac disease in addition to steroids for IPH, is the mainstay of therapy. The optimal treatment duration of steroid therapy is not known but anecdotally a more prolonged course results in improved outcome. We report a case of a young woman who presented with exertional dyspnoea, intermittent haemoptysis, severe anaemia and lung infiltrates but no gastrointestinal complaints. After extensive work-up, she was diagnosed with Lane-Hamilton syndrome based on a diagnosis of IPH made from lung biopsy and concomitant celiac disease because of positive anti-gliadin antibody and endomyosial antibody and jejunal biopsy. She was treated with sustained low-dose steroid therapy for a year and a gluten-free diet with resolution of her symptoms, anaemia and lung infiltrates. At 4 years of follow-up, she remains stable, without recurrence.
authors.affiliation: Division of Pulmonary-Critical Care Medicine, University of CT Health Center, Farmington, CT, USA.
authors.forename: Natalie
authors.initials: N
authors.lastname: Berger
authors.affiliation: Division of Pulmonary-Critical Care Medicine, University of CT Health Center, Farmington, CT, USA.
authors.forename: Julianne
authors.initials: J
authors.lastname: Nichols
authors.affiliation: Division of Pulmonary-Critical Care Medicine, University of CT Health Center, Farmington, CT, USA. ddatta@uchc.edu.
authors.forename: Debapriya
authors.initials: D
authors.lastname: Datta
mesh_terms: D000328:Adult; D002446:Celiac Disease / Q000175:diagnosis* / Q000178:diet therapy*; D015897:Comorbidity; D003937:Diagnosis, Differential; D055050:Diet, Gluten-Free; D005260:Female; D006486:Hemosiderosis / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis* / Q000188:drug therapy*; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome; D000097545:Hemosiderosis, Pulmonary
chemical_list: D013256:Steroids
keywords: alveolar haemorrhage; anaemia; lung infiltrates; pulmonary haemosderosis