Case Report: Systemic Lupus Erythematous Flare with Lupus Nephritis Complicated by Acquired Angioedema Possibly Linked with C1 Esterase Inhibitor Deficiency

This case report describes the hospitalization of a 21-year-old female patient with a history of systemic lupus erythematosus (SLE) and lupus nephritis, presenting with myalgia, low-grade fever, and pricking chest pain. The patient developed angioedema of the face, neck, and tongue, leading to a critical airway compromise. Further investigation revealed significant abnormalities, including severe anemia, complement deficiencies and there may be possible acquired C1 esterase inhibitor deficiency. Treatment involved fresh frozen plasma transfusions, C1 esterase inhibitor Concentrate for angioedema, hemodialysis and immunosuppressive therapy. Administration of C1 esterase inhibitor concentrate was a pivotal in restoring complement system function and alleviating excessive bradykinin effects and also other supportive care measures taken to stabilize the patient condition. End of the day patient's condition improved, and she was discharged with a plan for ongoing hemodialysis, discharge medications and follow-up recommendations.