Talks
Presenting Author Academic/Professional Position
Resident
Academic Level (Author 1)
Resident
Discipline/Specialty (Author 1)
Internal Medicine
Academic Level (Author 2)
Faculty
Discipline/Specialty (Author 2)
Internal Medicine
Presentation Type
Oral Presentation
Discipline Track
Clinical Science
Abstract Type
Case Report
Abstract
Background: Acute decompensated dilated cardiomyopathy (DCM) in a middle-aged patient without traditional cardiovascular risk factors presents a diagnostic and therapeutic challenge. While acute coronary syndrome (ACS) and pulmonary embolism must be rapidly excluded, a systematic evaluation is required to identify less common etiologies. Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease commonly presenting as pericarditis. Lupus myocarditis is a life-threatening complication with high morbidity and mortality that can present as DCM. Presentation of previously undiagnosed SLE as heart failure with reduced ejection fraction due to DCM is rare and occasionally reported. We describe a case of new-onset SLE initially manifesting as DCM secondary to lupus perimyocarditis.
Case Presentation: A 44-year-old Hispanic male with no significant medical history presented with four weeks of progressive dyspnea, lower extremity edema, orthopnea, and pleuritic chest pain. He denied joint pain, skin rash, or mucositis. Examination revealed bilateral basal crackles and 3+ pedal edema extending to the lower abdomen. Laboratory studies showed severe anemia and thrombocytopenia, along with elevated ESR, CRP, and BNP. Baseline EKG was unremarkable, and high-sensitivity troponin was elevated but did not demonstrate a dynamic rise on repeat testing, making ACS less likely. Pulmonary embolism and deep vein thrombosis were ruled out by CT pulmonary angiography and lower extremity venous Doppler ultrasound. Chest radiography demonstrated marked cardiomegaly with bilateral pleural effusions. Transthoracic echocardiography revealed a large circumferential pericardial effusion and globally reduced left ventricular ejection fraction of 25%, suggestive of perimyocarditis.
Autoimmune testing showed positive ANA, elevated anti-dsDNA and anti-Smith antibodies, hypocomplementemia, and elevated anti-SS-A antibodies. The patient met diagnostic criteria for SLE, with a SLE Disease Activity Index 2000 (SLEDAI-2K) score of 9, consistent with an active disease. The presence of anti-Ro/SSA autoantibodies, as seen in this case, is strongly associated with lupus-related myocardial injury, which manifested as DCM and served as the first clinical presentation of SLE in this patient. Treatment with high-dose prednisone (1 mg/kg/day), IV diuretics, and guideline-directed medical therapy for heart failure resulted in clinical improvement. Due to concern for evolving tamponade, he underwent a pericardial window procedure and subsequently recovered uneventfully, with discharge and planned cardiology and rheumatology follow-up.
Conclusion: SLE-associated myocarditis is challenging to diagnose in patients without previously established lupus, as its presentation can mimic other causes of acute heart failure. Endomyocardial biopsy remains the gold standard but is often unnecessary in self-limited cases with predominant pericarditis. Diagnosis is typically made by integrating clinical features, biomarker elevation, autoantibody profile, and advanced imaging, including speckle-tracking echocardiography and cardiac MRI. Coronary angiography to assess myocardial infarction is reserved for patients with intermediate to high risk for ACS. Early recognition is essential, as untreated lupus myocarditis carries mortality exceeding 10%. Evidence supports using high-dose corticosteroid therapy, with additional immunosuppressive agents reserved for severe or refractory cases. This case highlights lupus pericarditis as an important cause of DCM in patients with new-onset heart failure without traditional cardiovascular risk factors. Early recognition, timely immunosuppression, and guideline-directed heart failure management can lead to rapid recovery and improved outcomes.
Figure
Recommended Citation
Singh, Ripudaman and Gutierrez, Cesar, "When Lupus Begins in the Heart: New-Onset Lupus Perimyocarditis Presenting as Acute Dilated Cardiomyopathy" (2026). Research Symposium. 3.
https://scholarworks.utrgv.edu/somrs/2026/talks/3
Included in
When Lupus Begins in the Heart: New-Onset Lupus Perimyocarditis Presenting as Acute Dilated Cardiomyopathy
Background: Acute decompensated dilated cardiomyopathy (DCM) in a middle-aged patient without traditional cardiovascular risk factors presents a diagnostic and therapeutic challenge. While acute coronary syndrome (ACS) and pulmonary embolism must be rapidly excluded, a systematic evaluation is required to identify less common etiologies. Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease commonly presenting as pericarditis. Lupus myocarditis is a life-threatening complication with high morbidity and mortality that can present as DCM. Presentation of previously undiagnosed SLE as heart failure with reduced ejection fraction due to DCM is rare and occasionally reported. We describe a case of new-onset SLE initially manifesting as DCM secondary to lupus perimyocarditis.
Case Presentation: A 44-year-old Hispanic male with no significant medical history presented with four weeks of progressive dyspnea, lower extremity edema, orthopnea, and pleuritic chest pain. He denied joint pain, skin rash, or mucositis. Examination revealed bilateral basal crackles and 3+ pedal edema extending to the lower abdomen. Laboratory studies showed severe anemia and thrombocytopenia, along with elevated ESR, CRP, and BNP. Baseline EKG was unremarkable, and high-sensitivity troponin was elevated but did not demonstrate a dynamic rise on repeat testing, making ACS less likely. Pulmonary embolism and deep vein thrombosis were ruled out by CT pulmonary angiography and lower extremity venous Doppler ultrasound. Chest radiography demonstrated marked cardiomegaly with bilateral pleural effusions. Transthoracic echocardiography revealed a large circumferential pericardial effusion and globally reduced left ventricular ejection fraction of 25%, suggestive of perimyocarditis.
Autoimmune testing showed positive ANA, elevated anti-dsDNA and anti-Smith antibodies, hypocomplementemia, and elevated anti-SS-A antibodies. The patient met diagnostic criteria for SLE, with a SLE Disease Activity Index 2000 (SLEDAI-2K) score of 9, consistent with an active disease. The presence of anti-Ro/SSA autoantibodies, as seen in this case, is strongly associated with lupus-related myocardial injury, which manifested as DCM and served as the first clinical presentation of SLE in this patient. Treatment with high-dose prednisone (1 mg/kg/day), IV diuretics, and guideline-directed medical therapy for heart failure resulted in clinical improvement. Due to concern for evolving tamponade, he underwent a pericardial window procedure and subsequently recovered uneventfully, with discharge and planned cardiology and rheumatology follow-up.
Conclusion: SLE-associated myocarditis is challenging to diagnose in patients without previously established lupus, as its presentation can mimic other causes of acute heart failure. Endomyocardial biopsy remains the gold standard but is often unnecessary in self-limited cases with predominant pericarditis. Diagnosis is typically made by integrating clinical features, biomarker elevation, autoantibody profile, and advanced imaging, including speckle-tracking echocardiography and cardiac MRI. Coronary angiography to assess myocardial infarction is reserved for patients with intermediate to high risk for ACS. Early recognition is essential, as untreated lupus myocarditis carries mortality exceeding 10%. Evidence supports using high-dose corticosteroid therapy, with additional immunosuppressive agents reserved for severe or refractory cases. This case highlights lupus pericarditis as an important cause of DCM in patients with new-onset heart failure without traditional cardiovascular risk factors. Early recognition, timely immunosuppression, and guideline-directed heart failure management can lead to rapid recovery and improved outcomes.
