Idiopathic pulmonary hemosiderosis and alveolar hemorrhage syndrome: case report and review of the literature

Abstract

The presence of widespread hemorrhage from the microvasculature of the lung into the alveolar spaces defines what is called the "alveolar hemorrhage syndrome", which can occur in association with a wide variety of clinical disorders. The cardinal manifestations of this syndrome include: hemoptysis, unexplained anemia and diffuse alveolar infiltrates on chest roentgenograms. Since the pulmonary features are similar, the diagnosis usually depends on the clinical, laboratory and pathologic evaluations. Early diagnosis and treatment is crucial since the occurrence of pulmonary hemorrhage in this syndrome may represent a catastrophic event with fatal consequences. Idiopathic pulmonary hemosiderosis (IPH) has been identified as a cause of alveolar hemorrhage in a small number of cases, mainly by exclusion criteria. We report a case of a 70-year old man who presented with a 40-year history of intermittent hemoptysis and bilateral upper lobes alveolar infiltrates proved to be secondary to idiopathic pulmonary hemosiderosis. Although the lung apices are frequently spared in IPH, they were the site of the infiltrates in our case. To our knowledge, our patient had the longest survival time ever reported in the literature in adult IPH. A brief review of some of the disorders commonly associated with alveolar hemorrhage is also presented.