Introduction: Catastrophic antiphospholipid syndrome (CAPS) is a rare and serious phenomenon that requires prompt recognition and treatment. Case Presentation: The authors present the case of a puerperal woman with systemic lupus erythematosus (SLE) admitted to the emergency room with headache, blurred vision, thoracic pain, and purpuric lesions on both ears. Echocardiogram revealed global decrease in left ventricular function while cardiac and inflammatory markers were elevated. Three days after admission she developed cardiogenic shock due to rupture of mitral papillary muscle which required emergent cardiac surgery, with replacement of the mitral valve; treatment with anticoagulation, high-dose glucocorticoids, and intravenous immunoglobulins were initiated. Cardiac and brain MRI revealed signs of ischemic lesions in both organs. Histopathology analysis of the placenta and papillary muscle showed signs of ischemia secondary to microvascular thrombosis. Based on the clinical demonstration of thrombosis in three organs, and the presence of lupus anticoagulant antibodies, a diagnosis of probable CAPS was established. Conclusion: This case highlights the importance of a high level of suspicion of CAPS, particularly in patients with risk factors, and the value of immediate adequate treatment. Moreover, the rupture of a papillary muscle with histologically consistent signs of antiphospholipid syndrome expands the spectrum of involvement of this disease and should be considered as a rare but life-threatening possibility in patients with myocardial injury.
Takayasu arteritis (TA) is a rare type of vasculitis that affects mainly the aorta and its major branches. It is highly similar to giant cell arteritis (GCA), and differentiation between them may not be achieved even by histological examination. Arterial hypertension is typical of TA and is caused by stenosis of the renal arteries. Here we report the case of a 59-year-old woman, with a history of dyslipidemia and anemia, seen in the Internal Medicine department for resistant hypertension. Evaluation of secondary causes led to stenosis of the renal arteries. Assessment of target organ involvement was performed by computed tomography angiograph which revealed ectasia of the aortic arch and ascending aorta, tortuous course of the brachiocephalic trunk and the proximal portion of the right common carotid artery; positron-emission tomography which showed diffuse increased uptake in the ascending aorta, compatible with large vessels vasculitis. The patient was submitted to aortic valve replacement with a biological prosthesis combined with myocardial revascularization (Bentall-De Bono procedure). Aortic biopsy specimens showed anatomical and pathological features of GCA and TA. Due to persistently uncontrolled hypertension, prednisone 60 mg was initiated,with significant improvement in patient's condition.
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