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.
Chest x-ray showing bilateral multifocal patchy airspace opacities
Fig. 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
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
Acknowledgments
We would like to thank Michael Ansfield, M.D., the attending pulmonologist, for his teaching, leadership and guidance in reviewing this case, and Timothy Poore, M.D., former chief resident at San Francisco General Hospital, for his assistance with obtaining images.
Compliance with Ethical Standards
Conflict of Interest
The authors declare that they do not have a conflict of interest.
Funding
None
References
- 1.Rathi NK, Tanner AR, Dinh A, et al. Low-, medium- and high-dose steroids with or without aminocaproic acid in adult hematopoietic SCT patients with diffuse alveolar hemorrhage. Bone Marrow Transplant. 2015;50:420–6. doi: 10.1038/bmt.2014.287. [DOI] [PubMed] [Google Scholar]
- 2.Pathak V, Kuhn J, Gabriel D, Barrow J, Jennette JC, Henke DC. Use of activated factor VII in patients with diffuse alveolar hemorrhage: a 10 years institutional experience. Lung. 2015;193:375–9. doi: 10.1007/s00408-015-9720-z. [DOI] [PubMed] [Google Scholar]
- 3.Heslet L, Nielsen JD, Nepper-Christensen S. Local pulmonary administration of factor VIIa (rFVIIa) in diffuse alveolar hemorrhage (DAH)—a review of a new treatment paradigm. Biologics. 2012;6:37–46. doi: 10.2147/BTT.S25507. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 4.Lichtenberger JP, Digumarthy SR, Abbott GF, Shepard J-AO, Sharma A. Diffuse pulmonary hemorrhage: clues to the diagnosis. Curr Probl Diagn Radiol. 2014;43:128–39. doi: 10.1067/j.cpradiol.2014.01.002. [DOI] [PubMed] [Google Scholar]
- 5.Zamora MR, Warner ML, Tuder R, Schwarz MI. Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome. Medicine (Baltimore) 1997;76:192–202. doi: 10.1097/00005792-199705000-00005. [DOI] [PubMed] [Google Scholar]
- 6.Ioachimescu OC, Stoller JK. Diffuse alveolar hemorrhage: diagnosing it and finding the cause. Cleve Clin J Med. 2008;75:258. doi: 10.3949/ccjm.75.4.258. [DOI] [PubMed] [Google Scholar]
- 7.Phillips D, Phillips B, Mannino D. A case study and national database report of progressive systemic sclerosis and associated conditions. J Women’s Health. 1998;7:1099–104. doi: 10.1089/jwh.1998.7.1099. [DOI] [PubMed] [Google Scholar]