Idiopathic pulmonary haemosiderosis with celiac disease (Lane–Hamilton syndrome) in an adult – a case report

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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.

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