Quick Fire Cases
Yeraz Khachatoorian, MD, MPH
Cardiology Fellow
Westchester Medical Center
Yeraz Khachatoorian, MD, MPH
Cardiology Fellow
Westchester Medical Center
Pragya Ranjan, MD
Cardiologist, Associate Program Director Cardiology Fellowship Program/MD FACC
Westchester Medical Center
Anthon Fuisz, MD
Cardiologist, Director Advanced Cardiac Imaging Department//MD
Westchester Medical Center
A 67-year-old female with a history of mixed connective tissue disease (MCTD), including systemic lupus erythematosus (SLE), Sjogren’s syndrome, and dermatomyositis, was being treated with belimumab, prednisone, and mycophenolate. She was initiated on intravenous immunoglobulin (IVIG) therapy by her primary rheumatologist due to therapy-resistant MCTD. At the onset of IVIG treatment, the patient exhibited no clinical signs of disease flare and had stable rheumatoid markers. Within 48 hours of receiving the first dose, she developed severe hypotension, myalgia, fatigue, fever and vasogenic shock. Work up was remarkable for neutropenia, elevated troponin and multi-organ failure. The patient required intensive care, CVVHD, and vasopressor support. Cardiology was consulted for newly observed reduced left ventricular (LV) systolic function.
Diagnostic Techniques and Their Most Important Findings:
Transthoracic echocardiography revealed an ejection fraction (EF) of 40%, with severe hypokinesis of the basal and mid inferolateral and inferior walls. Coronary artery disease was ruled out via CT angiography. Within 48 hours of ICU admission, the patient exhibited significant clinical recovery.
CMR imaging performed for further evaluation once she had improved clinically during the hospitalization. CMR demonstrated a mildly dilated LV with low-normal LV systolic function and focal hypokinesis of the mid-inferior wall. Post-contrast images revealed linear, mid-myocardial late gadolinium enhancement (LGE) in the basal to mid inferolateral, inferoseptal, and inferior walls. T2 mapping was not performed due to artifact (image 1).
Learning Points from this Case:
Given the absence of symptoms indicating a flare of MCTD, the stability of markers prior to the initiation of IVIG and the absence of infection, the patient's clinical deterioration was hypothesized to be due to a severe allergic reaction to the IVIG therapy. The acute onset of distributive shock and multiorgan failure within 48 hours of the first dose of IVIG in combination with rapid recovery of LV function and improvement across multiple organ systems in the absence of any other clear etiology suggests that this may have been a rare case of a profound allergic reaction to IVIG itself. Cardiac MRI findings are suggestive of myocarditis – this may reflect an acute inflammatory reaction to IVIG therapy. Subsequently, the patient was discharged and put back on the medications for MCTD she had been on prior to the hospitalization. Clinically, she has continued to do well even three months after hospital discharge.
