Bleeding Lung and Bloating Gut: Lane Hamilton Syndrome

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare condition of unknown etiology characterized by hemoptysis, diffuse alveolar infiltrates and iron-deficiency anemia. Lane-Hamilton syndrome (LHS), a condition in which IPH is associated with celiac disease is an extremely rare condition. CASE PRESENTATION: 49-year-old male with no known past medical history presented with progressively worsening shortness of breath for 3 days. No associated fever, chest pain, cough or hemoptysis were noted. On presentation, temperature: 99F, RR: 34/min, O2 saturation of 85% on room air, HR: 130/min and BP: 100/60mmHg. Physical examination revealed diffuse crackles on chest auscultation. Blood work was significant for leukocytosis at 23.61, Hb of 9, MCV 76 and iron deficiency anemia. Liver function test showed mild transaminitis. Chest xray and CT chest showed diffuse ground glass opacities. He was started on broad spectrum antibiotics. Patient's respiratory status worsened requiring mechanical ventilation. Bronchoscopy and lavage showed diffuse alveolar hemorrhage, elevated white count and hemosiderin laden macrophages. Cultures and cytology were negative. A preliminary diagnosis of pulmonary hemosiderosis was made. Patient was started on pulse dose steroids. Rheumatologic workup was negative. On further review of chart, patient had frequent outpatient visits with bloating and diarrhea. Due to association of celiac disease and alveolar hemorrhage, further work up was sent. Anti-tissue glutaminase and Anti-Gliadin antibody were positive with gliadin IgA titers of 68.5 and IgG titers of 34.2. The probable diagnosis of LHS was made. Patient responded to steroids with clearing of infiltrates and decreasing oxygen requirement. DISCUSSION: LHS is an extremely rare combination of IPH and celiac disease. Both diseases are immunologically mediated and respond well to immunosuppression. Several hypotheses have been postulated to explain this association including deposition of circulating immune complexes from food allergens on the basement membrane of alveolar capillaries, the cross-reaction between the antireticulin antibodies and the alveolar basement membrane antigens. Lane-Hamilton syndrome was first seen in 1971. The association is mostly seen in children below 15 years but is very rare among adults. CONCLUSIONS: LHS should remain one of the differentials in patients presenting with diffuse alveolar hemorrhage. Concurrent screening for celiac disease should therefore be undertaken as disease can be successfully treated with a gluten-free diet once the diagnosis has been made.