Idiopathic Pulmonary Hemosiderosis in Adults: A Case Report and Review of the Literature

Abstract

<jats:p>Idiopathic pulmonary hemosiderosis is a very rare condition rarely affecting adults and causing recurrent episodes of diffuse alveolar haemorrhage that may lead to lung fibrosis. Due to lack of pathognomonic findings, IPH diagnosis is established upon exclusion of all other possible causes of DAH in combination with specific pathologic findings revealing bland alveolar haemorrhage with absence of vasculitis and/or accumulation of immune complexes within lung parenchyma. Here we describe a rare case of idiopathic pulmonary hemosiderosis in an otherwise healthy 27-year-old Greek male patient with relapsing episodes of fever accompanied by general fatigue and discomfort. He was at this time point a light smoker and had been hospitalised once in the past for similar symptoms. His iron deficiency anemia coupled with chest high-resolution computed tomography and bronchoalveolar lavage revealed findings compatible with diffuse alveolar hemorrhage. After excluding all other sources of bleeding through extensive gastrointestinal workup and thorough immunologic profile, video-assisted thoracic lung biopsy was performed and the diagnosis of Idiopathic Pulmonary Hemosiderosis was established. Patient was treated with high doses of oral corticosteroids, leading to clinical response. We highlight the need for vigilance by the respiratory physician for the presence of DAH, a challenging, acute condition requiring early recognition along with identification of the underlying syndrome and appropriate treatment to achieve optimal results.</jats:p>

Idiopathic pulmonary hemosiderosis in adults: review of cases reported in the latest 15 years

“…A total of 37 patients (24 males and 13 females) were included from 25 reports . The median age at diagnosis was 34 years (18–83 years), and 21 (57%) patients were diagnosed after the age of 30 years.…”

Pathological and Radiological Correlation in Prolonged Myeloperoxidase Anti-neutrophil Cytoplasmic Antibody-related Diffuse Alveolar Hemosiderosis

“…The pathological findings of hemosiderosis in idiopathic cases (4) and other diseases, including MPO-ANCA (5), have been studied very little. In this regard, the present case successfully confirmed that tiny nodules, patchy consolidation, and GGO corresponded to the accumulation of hemosiderin-laden AMs in the alveolar spaces.…”

Cryobiopsy in the diagnosis of idiopathic pulmonary hemosiderosis: a case report

“…Idiopathic pulmonary hemosiderosis is a rare disease, which is a result of an alveolar capillary bleeding and it is characterized by a high mortality rate (13,14). The disease is extremely rare in adults (15)(16)(17)(18). The clinical course usually suggests intra-alveolar bleeding with characteristic hemoptysis (19) observed in about 80% of patients (15,17,18).…”

“…The disease is extremely rare in adults (15)(16)(17)(18). The clinical course usually suggests intra-alveolar bleeding with characteristic hemoptysis (19) observed in about 80% of patients (15,17,18). In cases similar to the present one, i.e., where there are lung interstitial alterations with a concurrent anemia, IPH should always be considered in the differential diagnosis even though hemoptysis might not be reported by the patient (14,17,(19)(20)(21)(22).…”