A 68-year-old woman with systemic sclerosis and interstitial lung disease presented with progressive cough and dyspnea. She was in respiratory distress, with a heart rate of 110 beats per minute, respiratory rate 35, and oxygen saturation 63 % on ambient air, which improved to 94 % with 15 l of 100 % oxygen by face mask. Lungs had diffuse crackles throughout. Lab results revealed hemoglobin of 7.2 g/dL. Her chest x-ray showed multifocal bilateral airspace opacities (Fig. 1). She was intubated; subsequent bronchoscopy revealed progressively hemorrhagic lavage fluid (Fig. 2) indicative of diffuse alveolar hemorrhage (DAH). She was started on high-dose intravenous corticosteroid and cyclophosphamide. Due to ongoing DAH, bleeding was temporized with intravenous aminocaproic acid and intrabronchial activated factor VIIa (rFVIIa).1–3 Ultimately, she developed ventilator-associated pneumonia and died.

Fig. 1
figure 1

Chest x-ray showing bilateral multifocal patchy airspace opacities

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Fig. 2
figure 2

Bronchoalveolar lavage (BAL) demonstrating sequential progressive hemorrhagic fluid indicative of diffuse alveolar hemorrhage (DAH). Left-most tube represents the initial lavage fluid, and right-most tube the final lavage fluid

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DAH is characterized by bleeding into the alveolar spaces from disruption of the alveolar–capillary barrier.4 Cough, dyspnea and hemoptysis are common, although one-third of patients may not have hemoptysis.5 DAH has a broad differential including ANCA-associated vasculitides, anti-GBM disease, lupus and rarely systemic sclerosis.4,6,7 Treatment depends on the underlying cause, but in the setting of life-threatening DAH, intravenous aminocaproic acid and intrabronchial rFVIIa have been shown to temporize bleeding. Data on mortality benefit is lacking.1–3