P0880 - Child with Pulmonary Hemorrhage and Severe Anemia: An Unusual Presentation of Celiac Disease

Introduction:
Celiac disease is an autoimmune disease affecting ~1% of the general population, in which there is an abnormal mucosal response to the gluten protein in the duodenum. Celiac disease and its manifestations completely recede when gluten is removed from the diet. It is now more common to present with atypical consequences of celiac disease than with abdominal pain and diarrhea. These consequences include, but are not limited to, neurological problems, infertility, compromised dental enamel, and pulmonary hemorrhage.

Pulmonary hemorrhage in the setting of celiac disease is known as Lane Hamilton Syndrome (LHS). This a rare presentation of celiac disease, although its true prevalence maybe unknown as chest xrays are not commonly obtained in asymptomatic patients who are mildly iron deficient.

Case description:
7 year old female presented with two weeks of extreme fatigue and recent shortness of breath on exertion. The child self-eliminated bread from her diet for the last year. Her bloodwork revealed a hemoglobin of 4g/dL (11.7-13.8) with a mean corpusclar volume of 68fL (77-95) and red blood cell distribution of 16.7% (11.5-14.5).
An extensive evaluation for blood loss was completed due to severe anemia without concurrently severe microcytosis. This included a chest xray which was concerning for pulmonary hemorrhage. Chest CT findings led to bronchoscopy, where hemosiderin-laden macrophages supported prior pulmonary hemorrhage. Extensive evaluation for conditions that result in pulmonary hemorrhage, including trauma, congenital heart disease with pulmonary hypertension, tumor, atriovenous fistula, bleeding disorders, systemic lupus erythematosus, Goodpasture syndrome, Wegener granulomatosis, Henoch-Schonlein Purpura, microscopic polyangiitis was negative. However, TTG IgA > 50 units (<20) in the setting of a normal total IgA. Histology from upper endoscopy was consistent with celiac disease. She was discharged on a gluten free diet and iron supplementation. She is asymptomatic and growing well 9 months later.

Discussion:
The atypical findings in celiac disease are more common than the classic findings of abdominal pain and diarrhea. Lane Hamilton Syndrome is rare
with less than 40 pediatric patients in literature. However, it may be under-recognized as chest xrays are not routinely obtained in patients with celiac disease and many times, iron deficiency anemia is found improves with iron supplemenation and gluten cessation.

Conclusion:
CD presents more often with extra-intestinal manifestations than gastrointestinal manifestations. Clinicians should include CD in their differential diagnosis frequently, particularly in the setting of unexplained anemia. Consideration should be given to obtaining chest x-rays or sputum for hemosiderin-laden macrophages in these cases. When treating IPH, CD should always be considered.

Resolves with cessation of gluten ingestion in children with LHS