IDIOPATHIC PULMONARY HEMOSIDEROSIS, CELIA C-DISEASE AND CARDIOMYOPATHY

Background. - Idiopathic pulmonary hemosiderosis (IPH), a rare and pos sibly 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 anti bodies were present. The patient was placed on a gluten-free diet. Cas e n(o) 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 myocardi opathy. She had no manifestations, but a systematic search for coeliac disease was positive (total villous atrophy; presence of circulating gliadin and alveolar basement membrane antibodies). The patient was pl aced on a gluten-free diet, prednisone and diuretics, but she died dur ing a relapse 2 months later. Conclusion. - It is worthwhile checking for coeliac disease in all patients with IPH. The presence of myocardi opathy is a negative prognosis.