Pulmonary Hemosiderosis Associated With Celiac Disease

Abstract

A 6-year-old Hispanic boy with 3 days of hemoptysis presented with hemoglobin 4.5 g/dL. He had 3 months of iron deficiency anemia unresponsive to iron supplementation, with no gastrointestinal symptoms. Chest x-ray demonstrated right lung infiltrates with small pleural effusion (Fig. 1A). Computed tomography of the chest revealed ground glass appearance of the entire right lung and left lower lobe (Fig. 1B). Bronchoalveolar lavage showed 60% hemosiderin-laden macrophages that along with the radiologic findings is diagnostic for idiopathic pulmonary hemosiderosis (1). Severe anemia was disproportionate to hemoptysis. Searching for other causes of anemia, we found elevated tissue transglutaminase (128 U/mL) and positive antiendomysial immunoglobulin A antibody. Esophagogastroduodenoscopy demonstrated edema and minimal scalloping of duodenum. Histology confirmed flattened villi with erosion and increased intraepithelial lymphocytes (Fig. 1C and D), consistent with celiac disease. After initiating a gluten-free diet, pulmonary symptoms resolved and both followup imaging and tissue transglutaminase normalized.The association of celiac disease and idiopathic pulmonary hemosiderosis has been defined as the Lane-Hamilton syndrome. Both the disorders are believed to be immunologically mediated (1,2). Because of the good prognosis with a gluten-free diet, hemoptysis associated with pulmonary hemosiderosis in the setting of disproportionately severe anemia should prompt the evaluation for celiac disease (3-5).

Association between idiopathic pulmonary hemosiderosis and celiac disease in pediatric patients: A scoping review of the literature over the past 50 years

“…The follow‐up period was variable with a mean duration of 1.2 years (Table 2). During the follow‐up, an improvement of GI symptoms, 28,35 weight and height gain, 19,22,24,29,31,36 resolution of anemia 18,21,22,27,29 and a reduction or disappearance of antibody titer 16,22,23,33,34 was reported. One patient died from sudden death 2 years after diagnosis 27 …”

“…Of the 14 patients who were treated only with GFD, no recurrence of DAH was specified in 11 patients. 16,18,21,22,23,24,27,29,[33][34][35] In two patients, the future absence of respiratory symptoms were not specified. 31,36 One patient was accidentally exposed to gluten and suffered from recurrence of hemoptysis.…”

“…The treatment for this patient cohort with LHS consisted primarily of gluten free diet (GFD) with or without immunosuppressive medications (Table 2). 14/23 (61%) patients were treated with GFD alone 16,18,21,22,23,24,25,27,29,31,33‐36 . Six patients required systemic corticosteroid, 18,19,26,28,30,32 while two was on inhaled corticosteroid 19 in addition to a GFD.…”

Lane-Hamilton Syndrome: An Illustration of Extraintestinal Manifestations of Celiac Disease

“…6,7 Since this initial report, there have been 20 pediatric case reports of CD in conjunction with IPH or LHS. 2-4,7-16…”

“…6,7 Since this initial report, there have been 20 pediatric case reports of CD in conjunction with IPH or LHS. [2][3][4][7][8][9][10][11][12][13][14][15][16] Celiac disease is an autoimmune disorder with an immune reaction to gluten protein manifested in the small intestine and extraintestinal sites. 17,18 About 1% of the population has CD, with notable geographic variation.…”