P0460 - Diffuse Alveolar Hemorrhage. A Rare Initial Manifestation of Juvenile Onset Systemic Lupus Erythematosus with Novel Therapeutic Options: Case Report and Review of Literature

Introduction:
Juvenile onset systemic lupus erythematosus (JSLE) is a rare disease entity, characterized by a wide spectrum of organ involvement. Inclusive of these is a rare manifestation with high mortality known as diffuse alveolar hemorrhage (DAH). JSLE presenting with DAH presents a therapeutic challenge for both the rheumatologist and the intensivist, requiring careful attention to the underlying pathophysiology of widespread inflammation and capillaritis, as well as the acute symptomatic management of profound alveolar hemorrhage, airway obstruction and edema, and impaired gas exchange.

Case Description:
We present the case of a previously healthy 17-year-old African American male who presented with hemoptysis and severe anemia. He was subsequently transferred to the intensive care unit with DAH and acute respiratory failure requiring intubation and non-conventional mechanical ventilation. Following an extensive workup, serologies and renal biopsy confirmed a diagnosis of JSLE. He suffered persistent episodes of hemorrhage; ultimately symptom control required both systemic immunomodulatory and localized therapies. He required many traditional systemic therapies, including pulse dose steroids, intravenous immunoglobulin (IVIG), plasmapheresis and cyclophosphamide. As he continued to have life-threatening DAH, nebulized aminocaproic acid, an antifibrinolytic that is effective in enhancing hemostasis, was added. This combination of therapies was ultimately effective in managing his acute manifestation. He was extubated successfully and later discharged to an inpatient rehabilitation facility after 39 days in the ICU.

Discussion:
Diffuse alveolar hemorrhage is a rare and life-threatening complication of juvenile onset systemic lupus erythematosus. Given the rarity of this entity, it has only been studied in small case series. We present our own experience, highlighting the novel use of nebulized aminocaproic acid, with successful abatement of life-threatening alveolar hemorrhage and subsequent survival to hospital discharge.

Conclusion:
To our knowledge, there is no mention in the literature of the use of nebulized aminocaproic acid in the treatment of DAH, making this a novel therapy that proved beneficial in our patient.