[Idiopathic pulmonary hemosiderosis, celiac disease and cardiomyopathy]

Abstract

BACKGROUND--Idiopathic pulmonary hemosiderosis (IPH), a rare and possibly immune disease, is sometimes associated with coeliac disease and myocardiopathy.

Case reports: CASE NO 1--A 2 year-old boy with IPH was investigated because he suffered from frequent, soft stools. Small bowel biopsy showed partial villous atrophy. Circulating gliadin antibodies were present. The patient was placed on a gluten-free diet. CASE NO 2--An 8 year-old girl was admitted because she suffered from severe anemia (Hb: 4 g/100 ml). She was found to have IPH and myocardiopathy. She had no manifestation, but a systematic search for coeliac disease was positive (total villous atrophy; presence of circulating gliadin and alveolar basement membrane antibodies). The patient was placed on a gluten-free diet, prednisone and diuretics, but she died during a relapse 2 months later. CONCLUSION--It is worthwhile checking for coeliac disease in all patients with IPH. The presence of myocardiopathy is a negative prognosis.