Cholesterol Embolization Syndrome With an Atypical Proximal Presentation Simulating Calciphylaxis (original) (raw)
Related papers
Cholesterol crystal embolization following plaque rupture: a systemic disease with unusual features
The Journal of Biomedical Research, 2017
Cholesterol crystal embolic (CCE) syndrome is often a clinically challenging condition that has a poor prognostic implication. It is a result of plaque rupture with release of cholesterol crystals into the circulation that embolize into various tissue organs. Plaque rupture seems to be triggered by an expanding necrotic core during cholesterol crystallization forming sharp tipped crystals that perforate and tear the fibrous cap. Embolizing cholesterol crystals then initiate both local and systemic inflammation that eventually lead to vascular fibrosis and obstruction causing symptoms that can mimic other vasculitic conditions. In fact, animal studies have demonstrated that cholesterol crystals can trigger an inflammatory response via NLRP3 inflammasome similar to that seen with gout. The diagnosis of CCE syndrome often requires a high suspicion of the condition. Serum inflammation biomarkers including elevated sedimentation rate, abnormal renal function tests and eosinophilia are useful but non-specific. Common target organ involvement includes the skin, kidney, and brain. Various testing including fundoscopic eye examination and other non-invasive procedures such as trans-esophageal echocardiography and magnetic resonance imaging may be helpful in identifying the embolic source. Treatment includes aspirin and clopidogrel, high dose statin and possibly steroids. In rare cases, mechanical intervention using covered stents may help isolate the ruptured plaque. Anticoagulation with warfarin is not recommended and might even be harmful. Overall, CCE syndrome is usually a harbinger of extensive and unstable atherosclerotic disease that is often associated with acute cardiovascular events.
Cholesterol embolization syndrome: report of two cases
Turk Kardiyoloji Dernegi Arsivi-Archives of the Turkish Society of Cardiology, 2016
Özet-Kolesterol embolizasyon sendromu (KES), immünolojik manifestasyonları olan çoklu-sistemi ilgilendiren bir hastalıktır. İnvazif anjiyografi sonrası akut böbrek hasarına (ABH) neden olabilir. İleri derecede aterosklerozu olan erkek hastalarda daha sık görülür. Kolesterol embolizasyon sendromu, anjiyografi sonrası gelişen ABH'nın ayırıcı tanısında mutlaka düşünülmelidir; çünkü prognozu ve tedavisi kontrast nefropatisinden çok farklıdır. Burada anjiyografi sonrası gelişen iki KES'li olguyu sunmayı amaçladık. İlk olguda KES, böbrek biyopsisindeki karakteristik bulgularla ispatlandı. İkinci olguda "mavi ayak sendromu" ve kalıcı böbrek yetersizliği gelişen bir hasta sunuldu. Summary-Cholesterol embolization syndrome (CES) is a multisystemic disease with immunological features, and a rare but an important cause of acute kidney injury (AKI) following invasive angiography. It frequently occurs in the elderly male population with extensive atherosclerosis. CES should be considered in the differential diagnosis of AKI following angiography, as prognosis and treatment are completely different from contrast-induced nephropathy. Two cases of CES that developed after invasive angiography are described in the present report. In the first case, renal biopsy was performed, and CES was diagnosed by presence of characteristic renal lesions. The second patient had blue toe syndrome and persistent renal dysfunction.
Vascular Calcifications in Homozygote Familial Hypercholesterolemia
Arteriosclerosis, Thrombosis, and Vascular Biology, 2008
Background-Patients with homozygous familial hypercholesterolemia (hmzFH) attributable to LDL receptor gene mutations have shown a remarkable increase in survival over the last 20 years. Early onset coronary heart disease (CHD) and calcific aortic valve stenosis are the major complications of this disorder. We now report extensive premature calcification of the aorta in patients with hmzFH. Methods and Results-We examined 25 hmzFH patients from Canada; mean age was 32 years (range 5 to 54), and mean baseline cholesterol before treatment was 19Ϯ5 mmol/L (737Ϯ206 mg/dL). Aortic calcification was quantified using computed tomography (CT). An elevated mean calcium score was found in patients by age 20 and correlated with age (r 2 ϭ0.53, Pϭ0.001). One quarter (24%) of patients underwent aortic valve surgery. Conclusions-We document premature severe aortic calcifications in all adult hmzFH patients studied. These presented considerable surgical management challenges. Strategies to identify and monitor aortic calcification in hmzFH by noninvasive techniques are required, as are clinical trials to determine whether additional or more intensive therapies will prevent the progression of such calcifications. Whether vascular calcifications in hmzFH subjects are related to sustained increases in LDL-C levels or to other mechanisms, such as abnormal osteoblast activity, remains to be determined. (Arterioscler Thromb Vasc Biol. 2008;28:777-785)
The Cholesterol Emboli Syndrome in Atherosclerosis
Current Atherosclerosis Reports, 2013
Cholesterol emboli syndrome is a relatively rare, but potentially devastating, manifestation of atherosclerotic disease. Cholesterol emboli syndrome is characterized by waves of arterio-arterial embolization of cholesterol crystals and atheroma debris from atherosclerotic plaques in the aorta or its large branches to small or medium caliber arteries (100-200 μm in diameter) that frequently occur after invasive arterial procedures. End-organ damage is due to mechanical occlusion and inflammatory response in the destination arteries. Clinical manifestations may include renal failure, blue toe syndrome, global neurologic deficits and a variety of gastrointestinal, ocular and constitutional signs and symptoms. There is no specific therapy for cholesterol emboli syndrome. Supportive measures include modifications of risk factors, use of statins and antiplatelet agents, avoidance of anticoagulation and thrombolytic agents, and utilization of surgical and endovascular techniques to exclude sources of cholesterol emboli.
Journal of the European Academy of Dermatology and Venereology, 2003
In this paper the basic pathogenesis of cholesterol crystal embolization (CCE) is described, its clinical characteristics are presented and diagnosis and therapy are discussed. The main focus will be on the cutaneous manifestations; however, considering that CCE is a systemic illness, findings in other organs will also be highlighted, particularly the commonly involved renal and gastrointestinal systems.
Cholesterol crystal embolism (atheroembolism)
Heart International, 2006
Cholesterol crystal embolism, known as atheroembolic disease, is caused by showers of cholesterol crystals from an atherosclerotic plaque that occludes small arteries. Embolization can occur spontaneously or as an iatrogenic complication from an invasive vascular procedure (angiography or vascular surgery) and after anticoagulant therapy. The atheroembolism can give rise to different degrees of renal impairment. Some patients show a moderate loss of renal function, others severe renal failure requiring dialysis. Renal outcome can be variable: some patients deteriorate or remain on dialysis, some improve and some remain with chronic renal impairment. Clinically, three types of atheroembolic renal disease have been described: acute, subacute or chronic. More frequently a progressive loss of renal function occurs over weeks. Atheroembolization can involve the skin, gastrointestinal system and central nervous system. The diagnosis is difficult and controversial for the protean extrarenal manifestations. In the past, the diagnosis was often made post-mortem. In the last 10 yrs, awareness of atheroembolic renal disease has improved. The correct diagnosis requires the clinician to be alert. The typical patient is a white male aged >60 yrs with a history of hypertension, smoking and arterial disease. The presence of a classic triad (precipitating event, renal failure and peripheral cholesterol crystal embolization) suggests the diagnosis. This can be confirmed by a biopsy of the target organs. A specific treatment is lacking; however, it is an important diagnosis to make because an aggressive therapeutic approach can be associated with a more favorable clinical outcome. (Heart International 2006; 3-4: 155-60)