Co-occurrence pulmonary haemosiderosis with coeliac disease in child

Abstract

Idiopathic pulmonary haemosiderosis (IPH) is a rare and serious disorder in children of unknown aetiolopathogeny. Association of IPH and coeliac disease (CD) is even rarer. Immunological origin of IPH is now well accepted. We report the case of an 11-year-old female admitted for evaluation of recurrent streaky haemoptysis that had been evolving over the previous 9 months. Physical examination revealed weight loss with normal weight, but there was cutaneous and mucosal pallor due to severe anaemia (haemoglobin 4.6g/dl). The chest X-rays showed unilateral alveolo-intertitial infiltrate. Broncho-alveolar lavage revealed 70% haemosiderin-laden macrophages. The diagnosis of IPH was made. Since severe anaemia is disproportionate to radiologic findings, searching associated CD was performed and then confirmed by biological and histological examinations. A gluten-free diet was initiated. Evolution was favourable. Looking for especially CD in IPH should be systematic, even in the absence of gastrointestinal symptoms.

Hemorragia pulmonar asociada a enfermedad celíaca

“…8 La segunda hipótesis postula que en ambas entidades existiría una respuesta inmunitaria local inadecuada mediada por linfocitos T ante la ingesta de gluten en individuos predispuestos genéticamente. 9 Así, la presentación conjunta de hemosiderosis pulmonar idiopática y enfermedad celíaca se debería al hecho de que ambas entidades comparten una misma vía inmunopatogénica. 10 El tratamiento de esta entidad no se encuentra normatizado y deberá adecuarse a la evolución clínica de cada paciente en particular.…”

Pulmonary haemosiderosis in a 13-year-old girl with coeliac disease after 3 months on a gluten-free diet: case report and review of the literature

“…9,10 The pathogenic mechanism of this association is still unknown but, interestingly, most reported cases of CD and PH developed the latter while they had active CD and were on a glutencontaining diet. 1,2,[9][10][11] Most of them had a high titre of anti-endomesial and antigliadin antibodies at the time of pulmonary manifestations and some improved on a gluten-free diet alone. 1,[9][10][11] This is in favour of cross-reactivity between antireticulin antibodies and alveolar basal membrane antigens.…”

“…2,13 In IPH, if the severity of the anaemia is disproportionate to the radiological findings, CD should be considered. 2,9,13 Unless the bleeding is recent, haematological investigations in IPH will usually demonstrate microcytic, hypochromic anaemia. 7,13 Reticulocytosis may be present, depending on the available iron stores.…”

“…The chronic blood loss through the lungs must have played a role in the severity of the anaemia. 2,9,13 IPH might occasionally precede CD. In screening ten children with IPH for CD, Khemiri et al found the latter in three patients in whom it might have been missed initially.…”

“…1 All of the children previously reported with this association presented initially with IPH and had concurrent CD or developed it later. 2,3 In most of them, the diagnosis of CD was suspected either because of relevant gastro-intestinal symptoms [2][3][4] or because of severe anaemia. 3,5 All had serology positive for CD and improved on a gluten-free diet and/or corticosteroids.…”