Abstract
Diffuse alveolar hemorrhage (DAH) is a serious pulmonary complication in patients with autoimmune diseases who are undergoing chemotherapy or have had hematopoietic stem cell transplantation. The use of recombinant factor VIIa (rFVIIa) to treat the acute phase of DAH by endobronchial bronchoscopy has been shown to have a significant clinical impact on the survival and evolution of these patients. We report a clinical case of a patient with DAH secondary to systemic lupus erythematosus (SLE) who was treated with rFVIIa administered using a jet nebulizer, obtaining an adequate hemostatic effect with immediate control of DAH and a significant improvement in gas exchange.
Highlights
Diffuse alveolar hemorrhage (DAH) is a serious pulmonary complication secondary to autoimmune diseases, chemotherapy, and stem cell transplantation
Interstitial alveolar inflammation influences the expression of the intra-alveolar tissue factor (TF), causing an increase in the markers of thrombin generation in bronchoalveolar lavage (BAL) fluid [1,2,3,4,5]
The aim of this paper is to report the case of a female patient with systemic lupus erythematosus (SLE) and DAH who was treated during the active phase of bleeding with inhaled recombinant factor VIIa (rFVIIa) using a jet micronebulizer
Summary
Diffuse alveolar hemorrhage (DAH) is a serious pulmonary complication secondary to autoimmune diseases, chemotherapy, and stem cell transplantation. Its immunopathogenesis has been linked to the release of inflammatory cytokines, which are involved in alveolar capillary endothelial injury and inflammation. Pulmonary complications in systemic lupus erythematosus (SLE) may occur in 50%–70% of patients. Their clinical spectrum is characterized by pleuritis, interstitial pneumonitis, thromboembolism (mainly associated with antiphospholipid syndrome), nodules, bronchiolitis obliterans, infections, diaphragmatic weakness, and DAH. DAH is a rare manifestation that occurs in 2% to 5.4% of SLE patient, and it is associated with high mortality, reaching 50% to 80% [6]. DAH can even occur in patients treated with steroids and with good disease control.
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