Systemic Lupus Erythematosus–associated Diffuse Alveolar Hemorrhage: A Case Report and Review of the Literature

Abstract

Systemic lupus erythematosus is associated with numerous pleuropulmonary complications. Although uncommon, diffuse alveolar hemorrhage represents a life-threatening cause of acute respiratory failure among patients with lupus. Here, we present a 24-year-old woman with a history of lupus who developed hemoptysis and respiratory failure associated with diffuse radiographic infiltrates and anemia. Bronchoscopy confirmed diffuse alveolar hemorrhage. She was managed with supportive care, plasmapheresis, and immunosuppressive pharmacotherapy leading to sustained resolution of her pulmonary hemorrhage and respiratory failure. We then review the available literature on the pathophysiology and management of lupus-associated diffuse alveolar hemorrhage, which centers on supportive care, reversal of coagulopathy, and immunosuppressive measures. SUMMARY FOR TABLE OF CONTENTS A 24-year-old woman with a history of systemic lupus erythematosus presented with hemoptysis, diffuse radiographic infiltrates, anemia, and respiratory failure; bronchoscopy confirmed diffuse alveolar hemorrhage. Her condition was managed with supportive care, plasmapheresis, and immunosuppression with glucocorticoids, cyclophosphamide, and mycophenolate mofetil. Diffuse alveolar hemorrhage represents an uncommon but life-threatening complication of lupus with a growing evidence base to support acute and chronic management strategies centered on immunosuppressive pharmacotherapy.

Maintenance DNA methylation is essential for regulatory T cell development and stability of suppressive function

“…Humans with FOXP3 mutations develop similar endocrine and enteral inflammation as part of immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome (6). Treg dysfunction contributes to the pathogenesis of numerous autoimmune conditions, including systemic lupus erythematosus (7)(8)(9)(10)(11) and systemic sclerosis (12). In contrast, modern cancer immunotherapy blocks Treg-suppressive function to disinhibit effector T cell-mediated killing of malignant cells (13)(14)(15).…”

Experience of therapeutic plasma exchange in rheumatic diseases: Albumin may be a suitable substitute for plasma

“…However, for the treatment of severe SLE, particularly for the patients with DAH or neuropsychiatric lupus, the application of TPE achieved substantial clinical responses, compared to conventional therapies, based on anecdotal cases or non-controlled studies. 23,25,26 According to the latest guidelines recommended by the ASFA, SLE accompanied with severe complications was classified as a Category II indication for TPE, 27 corresponding to the reasons subject to TPE of SLE patients in our center. In our series, up to 62.9% SLE patients achieved PR and patients with CR accounted for 8.4%, which is an encouraging outcome, particularly in severe SLE or patients with involvement of kidney and or central nervous system.…”

Respiratory Manifestations in Systemic Lupus Erythematosus

“…Some patients can present chest pain. Hemoptysis can be of variable severity, dramatic in some cases, or initially absent in up to 33% of cases [8,10,33,36].…”