Is It Time to Call Idiopathic Pulmonary Hemosiderosis by the Correct Name: Immune-Mediated Pulmonary Hemosiderosis?

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is an uncommon cause of diffuse alveolar hemorrhage (DAH). This rare disease has an estimated incidence of 0.24–1.23 cases per million in children, and more than eighty percent of reported cases occurred in the pediatric population. There have been approximately forty adult cases reported in the past two decades. 1 Chen X.-.Y. Sun J.-.M. Huang X.-.J. Idiopathic pulmonary hemosiderosis in adults: review of cases reported in the latest 15 years. Clin Respir J. 11. 2017: 677-681 Google Scholar Due to the rarity of the disease and relative unawareness among clinicians, a diagnosis of IPH is often delayed, sometimes by years. The classic presentation includes the constellation of hemoptysis (varies significantly in quality and quantity), radiologic chest abnormalities, and iron deficiency anemia. However, the classic presentation is rather uncommon, and patients might present with other respiratory symptoms, such as dry cough, chest pain, and shortness of breath. Fatigue and fever are common. Massive hemoptysis with respiratory failure is rare, but when present, it can be rapidly fatal. A definitive diagnosis of IPH requires compatible symptoms and exclusion of competing diagnoses. DAH is the hallmark of IPH and is diagnosed by bronchoscopy. Progressively bloody fluid return on serial aliquots during bronchoalveolar lavage (BAL) is diagnostic of DAH. Microscopic evaluation of the BAL shows the predominance of hemosiderin-laden macrophages. Bronchoscopy is also helpful in excluding microbiologic causes of DAH. A lung biopsy is often performed (surgical, transbronchial or cryo lung biopsy), especially in adults, to rule out antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), Goodpasture syndrome and other immunologic causes of DAH.