Pulmonary hemorrhage: not only vasculitis

A 54-year-old man was admitted to our Unit for evaluation of a 4-week history of worsening dyspnea, mild productive cough, fatigue and chest pain. He also reported some episodes of haemoptysis in the prior month. The patient was affected by congenital bicuspid aortic valve disease associated with aortic root aneurysm, mild-severe aortic regurgitation and arterial hypertension. He was an active smoker (around fifteen cigarettes/day). Home therapy was ramipril and acetylsalicylic acid (voluntarily discontinued 2 weeks before admission). The physical examination revealed tachypnea, peripheral cyanosis, hypotension, and fever. He also reported severe fatigue. Arterial blood gas analysis showed hypocapnic respiratory failure. Electrocardiography signs of sinus tachycardia and incomplete right branch block were also present. Laboratory data showed a mild normocytic normochromic anemia (Hb 10.6 g/dl, MCV 92.1 fL, MCH 29.9 pg), leukocytosis (13.7 × 109/L) with neutrophilia (12.11 × 109/L), an increased erythrocyte sedimentation rate (ESR) (83 mm/h) and C-reactive protein (CRP) levels (40 mg/L), and mild acute renal failure (GFR 53 ml/min). Oxygen (2 L/min), i.v. hydration and empiric antimicrobial therapy were promptly administered. A preliminary thoracic computerised tomography (CT scan) was performed, and pulmonary embolism or neoplasms were ruled out. However, it did show apical peripheral bullous pulmonary emphysema with sparing of the medullary or central portion of the lungs, hilar lymphadenopathies, and multiple bilateral heterogeneous parenchymal opacities, with a ground-glass appearance, some of them with interlobular septal thickening and air bronchograms (Fig. 1). The Ziehl-Neelsen test on sputum was negative for Mycobacterium tuberculosis. Serological tests for atypical pathogens, and both Legionella pneumophila and Streptoccocus pneumoniae urinary antigens were also negative. The patient did not have any previous history of COPD. At this point, a fibre optic bronchoscopy was performed, demonstrating significant tracheal and bronchial bleeding in the absence of proliferative or traumatic lesions. A bronchoalveolar lavage fluid (BALF) was collected and tested for microbiology, cytology and lymphocyte immunophenotyping analysis. No evidence of infectious disease was detected in BALF, and the search for pandemic influenza virus H1N1 was negative. Only a few copies of HSV1 DNA were detected, probably due to oral contamination.