An Adult Case of Idiopathic Pulmonary Hemosiderosis Associated with Pulmonary Fibrosis and Emphysematous Change

Abstract

A 48-year-old woman was admitted to our hospital with acute respiratory failure. Chest computed tomography showed ground-glass opacity and patchy emphysematous lesions in both lungs. Corticosteroid therapy was effective; however, the disease worsened with the tapering of corticosteroids. Bronchoalveolar lavage revealed hemosiderin-laden macrophages, and video-assisted thoracic surgery showed diffuse interstitial fibrosis with diffuse alveolar hemorrhage (DAH). There was no evidence of vasculitis nor autoimmune diseases. This patient was diagnosed with idiopathic pulmonary hemosiderosis (IPH) that progressed to end-stage pulmonary fibrosis despite treatment. Autopsy demonstrated DAH with pulmonary fibrosis and emphysematous change, suggesting IPH-related pulmonary lesions.

Keywords: diffuse alveolar hemorrhage; emphysematous change; idiopathic pulmonary hemorrhage; pulmonary fibrosis.

Figure 1.

Chest HRCT on admission shows ground-glass opacity and patchy emphysema-like low attenuation areas in both lungs (A) with partial consolidation in the lower lobes (B). HRCT: high-resolution computed tomography

Figure 2.

Changes in the findings on chest CT from initial admission to bronchoscopy. Corticosteroid treatment improved the ground-glass opacity in both lungs one month later (PSL 40 mg/day); however, the findings gradually worsened two months later (at bronchoscopy, PSL 20 mg/day) with tapering of corticosteroids. CT: computed tomography, PSL: prednisolone

Figure 3.

A histological examination of video-assisted thoracoscopic surgery shows alveolitis, hyperplasia of type 2 pneumocytes, thickening of intra-alveolar septa [A: Hematoxylin and Eosin (H&E) staining], and cystic changes with fibrosis in some specimens (B: H&E staining), along with the accumulation of hemosiderin-laden macrophages in the alveolar space (C: H&E staining and D: iron staining). There is no evidence of vasculitis.

Figure 4.

Clinical course after video-assisted thoracoscopic surgery. AZA: azathioprine, FVC: forced vital capacity, Hb: hemoglobin, KL-6: Krebs von den Lungen-6, MEPM: meropenem, mo: month, mPSL: methylprednisolone, PSL: prednisolone, SP-D: surfactant protein-D, VATS: video-assisted thoracoscopic surgery

Figure 5.

Gross findings on autopsy show diffuse pulmonary sclerosis and cystic changes on the central side (A). Diffuse thickening of alveolar septa by collagen and hyperplasia of alveolar epithelium are seen with extensive hemorrhage, and fibrin and hemosiderin-laden macrophages are seen within the alveolar spaces [B: Hematoxylin and Eosin (H&E) staining, C: EVG staining, D: iron staining]. Emphysematous lesions are adjacent to fibrotic lesions, some with fibrous thickening (E: H&E staining and F: H&E staining). EVG: Elastica van Gieson