L'hemosiderose pulmonaire idiopathique est une affection caracterisee par des hemorragies intra-alveolaires conduisant a une fibrose pulmonaire. Ce travail decrit l'evolution de 8 patients atteints d'hemosiderose pulmonaire idiopathique. Il s'agit d'une etude retrospective portant sur 12 ans, d'enfants hospitalises dans deux services, l'un de pneumologie pediatrique et l'autre de pediatrie. Les enfants encore vivants ont eu un bilan immuno-allergologique et une etude de la muqueuse intestinale. Sur la periode de 12 ans, 8 cas d'hemosiderose pulmonaire ont ete diagnostiques. La duree moyenne d'evolution etait de 6 ans 1/2. Sur les 8 patients etudies, deux sont decedes, deux ont evolue vers une insuffisance respiratoire chronique et un autre presentait un trouble ventilatoire restrictif. Une manifestation extra pulmonaire etait associee a l'hemosiderose pulmonaire dans la moitie des cas : il s'agissait d'un vitiligo, d'une cardiomyopathie, d'une glomerulonephrite endocapillaire et d'une maladie cœliaque. L'evolution de l'hemosiderose pulmonaire idiopathique de l'enfant est severe. Son pronostic imprevisible justifie la recherche approfondie d'un agent causal.

Idiopathic pulmonary hemosiderosis is characterized by alveolar hemorrhage with secondary pulmonary fibrosis, Outcomes in eight retrospectively studied patients seen over a 12-year period In two hospital departments (pediatric pneumology and pediatrics) are reported. Tests for immunoallergy and an intestinal biopsy were done in survivors, Mean follow-up was six and a half years, Two patients died, two developed chronic respiratory failure, and one developed a restrictive ventilatory defect. Four patients had an extrapulmonary manifestation (one case each of vitiligo, cardiomyopathy, endocapillary glomerulonephritis, and celiac disease). The unpredictable but often grim prognosis of pediatric idiopathic pulmonary hemosiderosis warrants extensive investigations for a cause.

Pulmonary haemosiderosis in children is usually idiopathic and has a poor prognosis. It therefore justifies thorough aetiological investigation. Six children suffering from so-called idiopathic pulmonary haemosiderosis were submitted to a detailed clinical interview looking for triggering factors or an atopic predisposition. They were also submitted to an allergological assessment (skin tests, total serum IgE, specific IgE), an immunological assessment (IgA, IgC, IgM, serum complement, antinuclear, antimitochondrial, antismooth-muscle and antigliadin antibodies), biopsy of the small intestine and determination of the HLA group. This assessment demonstrated an association of idiopathic pulmonary haemosiderosis and coeliac disease in one case. This patient had raised IgE and a positive RAST to egg white and gluten. Antigliadin antibodies were positive in 4 cases. No case of cow's milk intolerance was revealed. The immunological assessment did not reveal any abnormalities. In conclusion, food sensitization must be systematically investigated in children with idiopathic pulmonary haemosiderosis. Ultrastructural and immunohistological study of the lung would further our understanding of the pathogenesis of this disease.