Diffuse alveolar haemorrhage with suspected idiopathic pulmonary hemosiderosis and decrease in lung diffusing capacity and chronic respiratory failure

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown aetiology that causes recurrent episodes of diffuse alveolar haemorrhage (DAH). A male patient in his 50s had repeatedly experienced hemoptysis for the past 6 years, along with a decrease in the pulmonary diffusing capacity and chronic respiratory failure. After a 6-year follow-up, the patient experienced sudden exacerbation of hemoptysis and respiratory failure, and he was hospitalised. A CT of the chest revealed diffuse pulmonary infiltrates, whereas the bronchoalveolar lavage revealed hemosiderin-laden macrophages. Thus, the patient was diagnosed with DAH. As all diseases that cause DAH other than IPH were negative, the patient was suspected of IPH. He was treated with a combination of glucocorticoids and azathioprine, and his hemoptysis and chronic respiratory failure improved; however, the decrease in the pulmonary diffusing capacity did not improve. Treating adult-onset IPH with glucocorticoids and azathioprine might not improve pulmonary diffusing capacity.

Keywords: lung function; respiratory medicine; respiratory system.

Figure 1

Chest X-ray on admission. Patchy opacity is revealed from the middle lung field to the lower lung field.

Figure 2

CT of the chest on admission. In addition to existing emphysematous changes, diffuse pulmonary infiltrates were disseminated throughout the overall lung field.

Figure 3

Bronchoalveolar lavage fluid (BALF) analysis. After inserting a bronchofiberscope into the right middle lobe (B4), 150 mL of physiological saline was administered and 44 mL of BALF was recovered. In BALF analysis (Berlin blue stain, ×60), hemosiderin-laden macrophages were revealed. BALF cell counts: 2.0×10⁵/mL (macrophages 97%, neutrophils 1% and lymphocytes 2%).

Figure 4

CT of the chest after administering 8 mg/day of prednisolone and 100 mg/day of azathioprine. There is residual emphysematous change, but diffuse pulmonary infiltrates have disappeared.