FVIIa Key to Fast Response in Deadly SLE Lung Hemorrhage

Sep 29, 2014

A case study reviews appropriate diagnostic and treatment steps for diffuse alveolar hemorrhage, a rare but devastating comorbidity of lupus and vasculitis.

Alabed IB. Treatment of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus Patient With Local Pulmonary Administration of Factor VIIa (rFVIIa): A Case ReportMedicine (2014) 93:e72 doi: 10.1097/MD.0000000000000072

This review and case study discusses a rare but life-threatening complication whose causes include systemic lupus erythematosus (SLE), systemic vasculitides, Goodpasture syndrome, coagulopathies, and other disorders. Diffuse alveolar hemorrhage (DAH) should be considered in any SLE patient presenting with any pulmonary complaint, and bronchoscopy performed quickly to establish the diagnosis.

Mortality is >50% in patients with diffuse alveloar hemorrhage (DAH) who need ventilator support. A major risk factor is active lupus nephritis with hypoalbuminemia.

DAH is characterized by disruption of the alveolar-capillary basement membrane, which allows red blood cells and inflammatory cytokines to enter the alveolar spaces.

The clinical syndrome is hemoptysis, falling hematocrit, hypoxemic respiratory failure, and diffuse pulmonary infiltrates.

The traditional strategy was to treat the underlying disease and symptoms, but usually DAH patients are already being treated with corticosteroids and immunosuppressives.

A few case reports and series in the last decade showed good results with local use of recombinant activated factor VII (rFVIIa), which promotes local formation of thrombin when it combines with tissue factor exposed at the endothelium.

In this case, a 37-year-old African woman with SLE, lupus nephritis class IV, was being treated with mycophenolate mofetil and prednisolone. She presented with shortness of breath, fever, pleuritic chest pain, no history of hemoptysis, and oxygen saturation that got progressively worse. She was admitted to the intensive care unit with hypoxemic respiratory failure. Hemoglobin decreased to 8 g/dL, platelet count was 213 x 109/L, activated thromboplastin time 40 seconds. Despite intravenous methylprednisolone and cyclophosphamide, pulmonary bleeding persisted.

Diffuse alveloar hemorrhage in a patient with SLE.

Praveen Rudraraju MD

et al.

The clinical deterioration led to the decision to administer 75 μg/kg of rFVIIa in saline via the bronchoscopy channel. The bleeding stopped immediately, and she improved slowly.

Pulmonary hemostasis can be induced more effectively via the alveoli side than via the endothelium, so bronchoscopy is preferably to intravenous administration. A complex of FVIIa and receptor tissue factor (TF), reached directly by the airway route, activates coagulation factors IX and X, resulting in homeostasis.

Alveolar rFVIIa also counters TF pathway inhibitors produced by alveolar macrophages, with the effect of reversing this immune-mediated anticoagulating effect.

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