Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder involving multiple organ systems. 1 With a prevalence of up to 241 per 100,000 people, the disease affects both sexes but has a clear female predominance across all age groups. 2 Cardiovascular manifestations like pericarditis, valvular heart disease, and endocarditis are common and are usually associated with significant morbidity and mortality. 3 Aortic aneurysm, however, is not a common feature of SLE, and remains asymptomatic in most patients until detected incidentally on imaging. 4 Further, aneurysm of the ascending aorta, which can have life-threatening consequences, is exceedingly rare with only a few cases reported so far. [5][6][7][8][9] In this report, we present the case of a 49-year-old woman with an aneurysm of the ascending aorta in the background of undiagnosed SLE. However, after an episode of sudden, refractory shock, likely due to the aneurysm rupture, she died while awaiting surgical intervention. With this unique case, we aim to alert clinicians to remain vigilant about the potential development of fatal
We report the case of a 62-year-old man who presented with shortness of breath, cough, bilateral lower limbs' swelling, and blackish discoloration of multiple fingertips over the past 2 months. Anti-Ribonucleoprotein antibodies were found to be present, and gadolinium-based cardiac MRI showed non-vascular subendocardial enhancement with diffuse symmetrical thickening of the left ventricular wall. A diagnosis of Mixed connective tissue disease with secondary cardiac amyloidosis was thus made, and the patient was successfully managed with intravenous cyclophosphamide, corticosteroids, and other supportive measures.Although extremely rare, this case shows that secondary cardiac amyloidosis should be considered while managing patients with MCTD.
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