Association of Celiac Disease With Idiopathic Pulmonary Hemosiderosis; Lane Hamilton Syndrome

Abstract

Introduction:Idiopathic Pulmonary Hemosiderosis (IPH) is a rare cause of alveolar hemorrhage, which is seen primarily in childhood. Celiac disease is defined as a chronic, immune-mediated enteropathy of the small intestine, caused by exposure to dietary gluten in genetically pre-disposed individuals. Association of IPH and celiac disease is known as Lane Hamilton syndrome. There are limited number of case reports of this syndrome in literature.Case Presentation:Although there were no growth and developmental delay and gastrointestinal symptoms like chronic diarrhea, chronic constipation, vomiting, abdominal bloating and pain in the two patients with IPH, they were diagnosed with Lane Hamilton Syndrome. After initiation of gluten-free diet, their IPH symptoms disappeared and hemoglobin levels were observed to return to normal.Conclusions:Even if there were no gastrointestinal symptoms in a patient with IPH, celiac disease should be investigated. These patients may benefit from gluten free diet and IPH symptoms may disappear.

Severe Alveolar Hemorrhage – What’s in it for the Gastroenterologist?

“…Celiac disease is usually thought of in front of a suggestive clinical scenario. Idiopathic pulmonary hemosiderosis is merely such a scenario, but CD serologic screening is recommended in these patients, irrespective of the presence of GI symptoms, as the reported cases of IPH associated with CD were GI symptom-free [10]. This is also the case of our patient, who had no history of GI complaints which could have prompted for a specific workup.…”

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.…”

Diffuse alveolar haemorrhage associated with subsequent development of ANCA positivity and emphysema in three young adults

“…IPH is a rare disease and little is known about the mechanisms involved in its development. However, its known association with coeliac disease [27], its response to immunosuppressive therapy and its possible association with autoimmune features [4, 28, 29] suggests that a contribution of immunological overactivity, even if a clear cut autoimmune disease is not identified.…”