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Successful Use of Bronchoscopic Recombinant Activated Factor VII in the Treatment of Systemic Lupus Erythematosus-Related Diffuse Alveolar Hemorrhage FREE TO VIEW

Joseph Zeman, MD; Brett Sadowski, MD; John Sherner, MD
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Walter Reed National Medical Center, Bethesda, MD

Chest. 2015;148(4_MeetingAbstracts):873A. doi:10.1378/chest.2275329
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SESSION TITLE: Pulmonary Manifestations of Systemic Disease Student/Resident Case Report Posters I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a life-threatening complications characterized by damage to the microcirculatory system of alveoli leading to accumulations of intraalveolar red blood cells. DAH is a rare (2-5.4%) but dangerous (50-80% mortality) complication in systemic lupus erythematosus (SLE). We present a case of a patient with a prolonged history of SLE who presented with DAH refractory to standard therapy who was successfully treated with recombinant activated Factor VIIa (rFVIIa) administered locally through bronchoscopy.

CASE PRESENTATION: The patient is a 28 year old African American woman with a past medical history of SLE complicated by biopsy proven lupus nephritis who presented to the emergency room with acute onset of right-sided chest pain and shortness of breath. She developed hemoptysis and hypoxic respiratory failure requiring intubation. Bronchoscopy performed was consistent with diffuse alveolar hemorrhage through serial aliquots on bronchoalveolar lavage (BAL) . She initially treated with pulse dose intravenous methylprednisolone and received 5 treatments of therapeutic plasma exchange. She was extubated on hospital day 6, but had severe hemoptysis prompting same day reintubation, with bronchoscopy demonstrating persistent diffuse alveolar hemorrhage persistent on serial BAL aliquots on hospital day 6/7. On hospital day 7, family was consented for an attempt to control the hemorrhage with local instillation or rFVIIa (75µg/kg) divided into six aliquots and delivered at each secondary bronchi. After repeat dosing with local rFVIIa targeted at bronchi with persistent infiltrates on CT scan, repeat bronchoscopy revealed resolution of DAH and patient was successfully extubated. Coagulation studies during dosing of local rFVIIa remained unchanged.

DISCUSSION: DAH is a rare complication of SLE and carries a high mortality rate (50-80%) if left untreated. It is often refractory to the conventional therapy of high dose steroids, cyclophosphamide and 3-6 cycles of plasma exchange as in our patient. Off-label use of rFVIIa by local instillation or aerosolization into lungs has been utilized in treatment of DAH with some reported success and a excellent safety profile as it does not cross the alveolar-blood barrier.

CONCLUSIONS: We describe a case of successful treatment of SLE-associated DAH refractory to conventional therapy by means of locally administered rFVIIa.

Reference #1: Esper RC, et al. Treatment of diffuse alveolar hemorrhage secondary to lupus erythematousus with recombinant activated factor VII administered with a jet nebulizer. Journal of Intensive Care 2014,2:47.

Reference #2: Heslet L, et al. Successful pulmonary administration of activated recombinant factor VII in diffuse alveolar hemorrhage. Crit Care 2006;10(6):R177.

Reference #3: Estella A, Jareno A, Fontaina LP. Intrapulmonary administration of recombinant activated factor VII in diffuse alveolar hemorrhage: a report of two case stories. Cases Journal 2008,1:150.

DISCLOSURE: The following authors have nothing to disclose: Joseph Zeman, Brett Sadowski, John Sherner

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