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Necrotizing Autoimmune Myositis - A Rare Etiology ...
Necrotizing Autoimmune Myositis - A Rare Etiology of Advanced Cardiomyopathy
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Pdf Summary
A 40-year-old man with biopsy-proven necrotizing autoimmune myositis (NAM) on abatacept presented with two days of worsening shortness of breath. On arrival, he was tachycardic with a heart rate of 170/min. Labs showed elevated troponin, creatine phosphokinase, and aldolase, suggesting active muscle and possible cardiac injury. ECG demonstrated new atrial flutter with variable conduction and an underlying right bundle branch block. CTA of the chest also revealed multiple right-sided pulmonary emboli.<br /><br />He was treated with amiodarone and anticoagulation with low-molecular-weight heparin. Although he initially improved, he became hypotensive within 24 hours and required pressor support. TTE then showed severe new cardiomyopathy with an LVEF of 10%. After attempted transesophageal echocardiography and cardioversion, he suffered cardiac arrest and required escalating inotropic support and ECMO. Coronary angiography showed non-obstructive coronary arteries. Given the rapidly progressive course, cardiac involvement from NAM was suspected. Cardiac MRI showed diffuse late gadolinium enhancement, supporting myocardial involvement.<br /><br />He was treated with pulse-dose steroids and cyclophosphamide. Over the next three weeks, his cardiac function improved dramatically, with LVEF recovering to 55%, and he was successfully weaned from ECMO. He was discharged four weeks later on steroids and cyclophosphamide to a long-term acute care facility.<br /><br />This case highlights that NAM, like other idiopathic inflammatory myopathies, can cause serious cardiac complications including arrhythmias and severe heart failure. Early recognition, telemetry monitoring, and prompt immunosuppressive therapy may be lifesaving and improve prognosis.
Asset Subtitle
Aleesha Kainat
Meta Tag
Author List
Aleesha Kainat, Anjana Chandrasekhara Pillai, Corey Rearick, Rahul Chaudhary
Category
Clinical Vignettes
Concept
Statin-induced necrotizing autoimmune myopathy
Concept
Cardiac Involvement
Concept
Cardiac Arrhythmia
Concept
Acute Heart Failure
Concept
Cardiogenic Shock
Distinguished
Non-Finalist
Presenter Organization
Department of General Internal Medicine, University of Pittsburgh Medical Center, McKeesport
Presenting Author
Aleesha Kainat
Track
Adult
Keywords
necrotizing autoimmune myositis
cardiac involvement
atrial flutter
pulmonary embolism
cardiomyopathy
cardiac arrest
ECMO
cardiac MRI
immunosuppressive therapy
heart failure
Statin-induced necrotizing autoimmune myopathy
Cardiac Involvement
Cardiac Arrhythmia
Acute Heart Failure
Cardiogenic Shock
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