Idiopathic pulmonary haemosiderosis revisited

Abstract

Idiopathic pulmonary haemosiderosis is a rare cause of diffuse alveolar haemorrhage of unknown aetiology. It occurs most frequently in children, has a variable natural history with repetitive episodes of diffuse alveolar haemorrhage, and has been reported to have a high mortality. Many patients develop iron deficiency anaemia secondary to deposition of haemosiderin iron in the alveoli.Examination of sputum and bronchoalveolar lavage fluid can disclose haemosiderinladen alveolar macrophages (siderophages), and the lung biopsy shows numerous siderophages in the alveoli, without any evidence of pulmonary vasculitis, nonspecific/ granulomatous inflammation, or deposition of immunoglobulins. Contrary to earlier reports, corticosteroids alone or in combination with other immunosuppressive agents may be effective for either exacerbations or maintenance therapy of idiopathic pulmonary haemosiderosis.

Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis

“…However, plasma ferritin level can be normal or elevated in IPH because of alveolar synthesis and release into the circulation and does not reflect the iron deposits in the body. 5 This makes the diagnosis of iron-deficiency anaemia in IPH difficult. We recommend the use of serum iron and transferrin saturation (serum iron/TIBC) instead to evaluate suspected iron-deficiency anaemia.…”

Idiopathic pulmonary hemorrhage: morphology and differential diagnosis. Case report

“…Chronic respiratory failure and cor pulmonale are related to the development of severe fibrosis. Some patients develop repeated episodes of hemoptysis, or persistent dyspnea and anemia (5,11,21,26) . The use of corticosteroids must be considered, but the response is variable (1,3,8,12) .…”

“…Presence of nodular fibrosis and/or centriacinar emphysema may be found in some cases (16,23,25,26) . The differential diagnosis of IPH also includes cases of vasculitis related to polyarteritis nodosa, Takayasu's arteritis, Behçet's syndrome, sarcoidosis, pulmonary infection, and septic emboli (4,5,7,9,11,15,17,25) . In the present report, the authors reported a case of IPH in an adult male patient referring cough and hemoptysis, after exhaustive clinical, laboratorial and radiologic investigation to exclude other possible causes of AH.…”

“…Long-term IPH may lead to interstitial fibrosis. By definition, IPH does not present renal involvement, immune complexes, and antineutrophil cytoplasmic antibody (ANCAS) (1,3,4,8,10,11,17,18,21) . IPH diagnosis is made on the basis of clinical, radiologic, laboratorial and histopathological findings.…”

“…The patients present recurrent episodes of pulmonary hemorrhage, which may occasionally determine ventilatory restriction. The clinical presentation is extremely variable, and the most common symptoms include iron deficiency anemia, diffuse pulmonary infiltrates, and hemoptysis (3,8,10,11,25) .…”