Talks

Presenting Author

Yong-Chan Kim

Presentation Type

Oral Presentation

Discipline Track

Patient Care

Abstract Type

Case Report

Abstract

Background: Diffuse alveolar hemorrhage (DAH) is defined as disruption of the alveolar-capillary basement membrane, causing bleeding into the alveolar spaces. It is one of the rarest complications in systemic lupus erythematosus (SLE) and life-threatening conditions. Symptoms include shortness of breath, cough, fluctuating fever, and rarely hemoptysis. Complications include acute respiratory distress syndrome. The test of choice for diagnosis is bronchoalveolar lavage (BAL). The hallmark is that BAL aliquots are progressively more hemorrhagic. CT-chest shows ground glass or consolidative opacities that are usually diffuse and bilateral nonspecific. There is no cornerstone therapy for DAH due to SLE. Considering this a fatal condition, patients receive supportive care and high doses of systemic glucocorticoids accompanied by another immunosuppressive agent. We report a case of DAH with severe acute respiratory distress syndrome due to SLE with a prompt diagnosis and management.

Case presentation: A 26-year-old female with a history of Systemic Lupus Erythematosus and lupus nephritis presented to ED after an episode of hemoptysis. She also complained of associated dyspnea and fever. Initial chest CT revealed extensive bilateral, right lung predominant parenchymal and surrounding ground-glass opacities. Due to worsening acute hypoxemic respiratory failure, she required orotracheal intubation and mechanical ventilation. Bronchoscopy with bronchoalveolar lavage was performed demonstrating scant thin bloody secretions with predominant neutrophils. Nebulized tranexamic acid, high-dose intravenous methylprednisolone, and antibiotics were initiated. The patient was successfully extubated and transitioned to a nasal cannula. Repeat chest x-rays showed improvement in bilateral pulmonary infiltrates; no further episodes of hemoptysis were reported. Oral prednisone and hydroxychloroquine were initiated, and the patient was able to tolerate breathing room air with no signs of acute distress.

Discussion: Alveolar hemorrhage is a rare and potentially life-threatening complication seen in patients with Lupus. It's prompt recognition and early intervention are crucial to prevent further lung damage and improve outcomes. The exact etiology is not clearly understood though it is thought that is the result of specific SLE autoantibodies causing damage in the small pulmonary vasculature. BAL is the preferred diagnostic method, evidencing progressive hemorrhagic fluid. A lung biopsy is rarely necessary. The optimal treatment has not been yet well established. Because of the high mortality risk, patients are treated initially with high-dose intravenous steroids plus a second immunosuppressive drug. There is a delicate balance in starting a second agent because of possible underlying infection that can worsen the patient’s status. This decision should be based on the disease severity. Survival outcomes range between 28-75% survival rate reported in small case series though, further studies are needed to better understand the pathophysiology of DAH improving treatment and survival outcomes.

Academic/Professional Position

Resident

Mentor/PI Department

Internal Medicine

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Managing Diffuse Alveolar Hemorrhage in a Critical Care Setting

Background: Diffuse alveolar hemorrhage (DAH) is defined as disruption of the alveolar-capillary basement membrane, causing bleeding into the alveolar spaces. It is one of the rarest complications in systemic lupus erythematosus (SLE) and life-threatening conditions. Symptoms include shortness of breath, cough, fluctuating fever, and rarely hemoptysis. Complications include acute respiratory distress syndrome. The test of choice for diagnosis is bronchoalveolar lavage (BAL). The hallmark is that BAL aliquots are progressively more hemorrhagic. CT-chest shows ground glass or consolidative opacities that are usually diffuse and bilateral nonspecific. There is no cornerstone therapy for DAH due to SLE. Considering this a fatal condition, patients receive supportive care and high doses of systemic glucocorticoids accompanied by another immunosuppressive agent. We report a case of DAH with severe acute respiratory distress syndrome due to SLE with a prompt diagnosis and management.

Case presentation: A 26-year-old female with a history of Systemic Lupus Erythematosus and lupus nephritis presented to ED after an episode of hemoptysis. She also complained of associated dyspnea and fever. Initial chest CT revealed extensive bilateral, right lung predominant parenchymal and surrounding ground-glass opacities. Due to worsening acute hypoxemic respiratory failure, she required orotracheal intubation and mechanical ventilation. Bronchoscopy with bronchoalveolar lavage was performed demonstrating scant thin bloody secretions with predominant neutrophils. Nebulized tranexamic acid, high-dose intravenous methylprednisolone, and antibiotics were initiated. The patient was successfully extubated and transitioned to a nasal cannula. Repeat chest x-rays showed improvement in bilateral pulmonary infiltrates; no further episodes of hemoptysis were reported. Oral prednisone and hydroxychloroquine were initiated, and the patient was able to tolerate breathing room air with no signs of acute distress.

Discussion: Alveolar hemorrhage is a rare and potentially life-threatening complication seen in patients with Lupus. It's prompt recognition and early intervention are crucial to prevent further lung damage and improve outcomes. The exact etiology is not clearly understood though it is thought that is the result of specific SLE autoantibodies causing damage in the small pulmonary vasculature. BAL is the preferred diagnostic method, evidencing progressive hemorrhagic fluid. A lung biopsy is rarely necessary. The optimal treatment has not been yet well established. Because of the high mortality risk, patients are treated initially with high-dose intravenous steroids plus a second immunosuppressive drug. There is a delicate balance in starting a second agent because of possible underlying infection that can worsen the patient’s status. This decision should be based on the disease severity. Survival outcomes range between 28-75% survival rate reported in small case series though, further studies are needed to better understand the pathophysiology of DAH improving treatment and survival outcomes.

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