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Cholesterol crystal embolism (CCE) is a serious complication that occurs after cardiac and vascular procedures. CCE involves multiple organs, and the prognosis and renal function of patients is poor. Although the efficacy of steroid, statin, and low-density lipoprotein apheresis has been reported, no definitive treatment has been established. We herein report three consecutive cases treated with conventional steroid therapy with proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitor after catheterization. The renal function was preserved, steroid therapy was stopped, and wound healing of blue toes was achieved. PCSK9 inhibitor therapy was safe in the present patient and may be a potential treatment option for CCE.

Background: We report a unique case of renal cholesterol crystal embolism (CCE) induced by carotid artery stenting that was successfully treated with evolocumab, a fully human monoclonal antibody against proprotein convertase subtilisin kexin type 9 (PCSK9). Case presentation: A 77-year-old man with hypertension, hyperlipidemia, and chronic kidney disease was referred to our department for decreased estimated glomerular filtration rate (eGFR)-from 32.0 to 13.9 mL/min/1.73 m 2 -5 weeks after carotid artery stenting. Further examination revealed livedo reticularis in the bilateral toes and eosinophilia (723/μL). Skin biopsy from livedo reticularis tissue in the bilateral toes showed cholesterol clefts in the small arteries. The patient was therefore diagnosed with CCE. After 25 weeks' administration of evolocumab at a dose of 140 mg subcutaneously administered every 2 weeks, his eGFR had improved from 10.7 to 18.1 mL/min/1.73 m 2 . Conclusion: Evolocumab may have a beneficial effect on renal involvement in patients with CCE.

We report the case of a woman presenting with livedo reticularis of the breast who was found to have atheroembolism to the breast following upper extremity percutaneous access. Atheroembolism is the embolization of cholesterol crystals off an atherosclerotic plaque that can occur spontaneously or as a result of vascular intervention. This is a unique presentation of an otherwise well-described complication of vascular catheterization, and we propose that livedo reticularis of the breast can be interpreted as a sign of atheroembolism in the appropriate clinical context., (Copyright © 2019 Elsevier Inc. All rights reserved.)

An 80-year-old man presented at our hospital with renal failure. He had been treated with edoxaban, an oral direct factor Xa inhibitor, for deep vein thrombosis for 10 months prior to admission. Although the pulses in his bilateral pedal arteries were palpable, cyanosis was present in the bilateral toes. Laboratory data indicated azotemia and eosinophilia. A skin biopsy confirmed a diagnosis of cholesterol crystal embolism (CCE). Because no invasive vascular procedure was performed, we assumed that CCE was related to edoxaban. To the best of our knowledge, this is the first case report suggesting CCE induced by an Xa inhibitor.

Competing Interests: Competing interests: None declared.

There is a growing body of evidence to suggest that reactive oxidant species (ROS) including O2-, OH- or H2O2 act as second messengers to activate platelets via 1) calcium mobilisation, 2) nitric oxide (NO) inactivation, and 3) interaction with arachidonic to give formation of isoprostanes. Among the enzymes generating ROS formation NOX2, the catalytic core of NADPH oxidase (NOX), plays a prominent role as shown by the almost absent ROS production by platelets taken from patients with hereditary deficiency of NOX2. Experimental and clinical studies provided evidence that NOX2 is implicated in platelet activation. Thus, impaired platelet activation has been detected in patients with NOX2 hereditary deficiency. Similarly, normal platelets added with NOX2 specific inhibitors disclosed impaired platelet activation along with ROS down-regulation. Accordingly, animals prone to atherosclerosis treated with apocynin, a NOX inhibitor, showed reduced platelet adhesion and atherosclerotic plaque. Furthermore, a significant association between NOX2 up-regulation and platelet activation has been detected in patients at athero-thrombotic risk, but a cause-effect relationship needs to be established. These findings may represent a rationale to plan interventional trials with NOX inhibitors to establish if blocking NOX2 or other NOX isoforms may represent a novel anti-platelet approach.

Cholesterol crystal embolism (CCE) is a serious complication associated with invasive vascular procedures. The prognosis of the renal involvement type of CCE is very poor, and there is currently no established treatment, other than supportive therapy. We herein report four cases of CCE with severe atherosclerosis wherein the renal function progressively deteriorated after cardiac catheterization. In three of the four patients, low-dose corticosteroids (0.3 mg/kg/day) improved the renal function, whereas the fourth patient died from CCE of the digestive system. This report reviews the literature on CCE and discusses possible therapeutic options.

Purple toe syndrome is a rare complication of warfarin therapy. It occurs usually after 3 to 8 weeks of therapy and it is caused by cholesterol emboli from atheromatous plaque. Sudden onset of pain in affected area, typically in toes and feet, is the main characteristic of the syndrome. We describe a case of a 65-year-old female with purple toe syndrome after 6 weeks of warfarin. Indication of warfarin was a proximal deep venous thrombosis, which developed after prolonged immobilization. Factor V (FV) Leiden and persistent high FVIII activity were found as additional eliciting factors for venous thromboembolism. After warfarin withdrawal and enoxaparin treatment, symptoms disappeared promptly but a slight discoloration of the toe persists.

Acute atherothrombotic complications, as part of the accelerated atherosclerosis, contribute to cardiovascular morbibity and mortality in diabetic patients. Inhibition of platelet aggregation can reduce the risk for acute atherothrombosis. The present article represents the recommendations of the Austrian Diabetes Association for the use of antiplatelet drugs in diabetic patients according to current scientific evidence.

Cholesterol atheroembolism (CAE) is a systemic disorder whose incidence has increased in recent decades and that presents high morbidity and mortality. Although several therapeutic alternatives have been reported, there is no consensus about the best treatment for this disease. In this paper we report the case of a patient with CAE with skin, bowel and kidney involvement who presented a good response to combined therapy with steroids and prostaglandin analogues. Although there are no conclusive studies, we recommend this therapeutic alternative in the management of CAE with organic failure.

A 69-year-old man with a history of hypertension was admitted to our hospital because of proteinuria, renal dysfunction, and both purpura and edema in the lower extremities. Laboratory data on admission revealed proteinuria (3.4 g/day), microscopic hematuria (3+), and renal dysfunction (serum creatinine 1.47 mg/dL). In the renal biopsy, all glomeruli showed mild mesangial proliferation. A few glomeruli showed mild segmental endocapillary proliferation. Crescent was not found in any glomeruli. Immunofluorescent study revealed the deposition of IgA and C3 in the mesangial area. In addition, jagged-edged angular cholesterol clefts of atheromatous emboli were seen in a small artery with tubular atrophy and fibrosis. He was diagnosed as Henoch-Schonlein purpura nephritis accompanied by idiopathic cholesterol crystal embolism, because he previously had not undergone any cardiac procedures (e. g., percutaneous coronary intervention and coronary artery bypass grafting) and anticoagulating therapy. Oral prednisolone (40 mg/day) effectively decreased proteinuria and improved his renal dysfunction. In this case, renal dysfunction may be related to the ischemic interstitial damage caused by cholesterol crystal embolism, as well as purpura nephritis.

Purpose of Review: To describe cholesterol embolization syndrome (CES) and its risk factors, pathophysiology, clinical presentation, diagnosis and treatment., Recent Findings: To date, no specific diagnostic test (other than biopsy) for CES has been developed. Effective treatments for CES are yet to be developed., Summary: CES (also referred to as cholesterol crystal embolization, atheromatous embolization or atheroembolism) occurs when cholesterol crystals and other contents of an atherosclerotic plaque embolize from a large proximal artery to smaller distal arteries, causing ischemic end-organ damage. Clinical manifestations of CES include constitutional symptoms (fever, anorexia, weight loss, fatigue and myalgias), signs of systemic inflammation (anemia, thrombocytopenia leukocytosis, high erythrocyte sedimentation rate, elevated levels of C-reactive protein, hypocomplementemia), hypereosinophilia, eosinophiluria, acute onset of diffuse neurologic deficit, amaurosis fugax, acute renal failure, gut ischemia, livedo reticularis and blue-toe syndrome. CES may occur spontaneously or after an arterial procedure. There is no specific laboratory test for CES. Retinal exam demonstrating Hollenhorst plaques supports the diagnosis of CES. Biopsy of target organs (usually skin, skeletal muscles or kidneys) is the only means of confirming the diagnosis of CES. Treatment consists of supportive care and general management of atherosclerosis and arterial ischemia.

Cholesterol crystal embolization (CCE) is an important and often under-diagnosed cause of renal insufficiency in patients with atherosclerosis. So far, only statins are the mainstay of therapy and the role of corticosteroids is controversial. We describe a 57-year-old gentleman who presented with accelerated hypertension and renal failure three months after coronary angiogram. Renal biopsy showed cholesterol clefts in the arteriole. Initially, management with anti-hypertensives alone (already receiving statins since angiogram) was unsuccessful. A trial of high-dose corticosteroids resulted in an improvement of the general condition in the next two days, and the serum creatinine reduced gradually to 1.6 mg/dL over the next one month. In conclusion, high-dose corticosteroids are useful in the treatment of CCE associated renal failure, especially in cases with no spontaneous recovery of function.

Background: The effect of corticosteroids on renal cholesterol crystal embolism (CCE) remains uncertain. The aim of the present study was to elucidate the effect of steroid therapy on short- and long-term renal outcome in CCE patients., Methods: Fifty-one patients diagnosed with renal CCE were included in this retrospective study. The patients were divided into two groups according to whether or not they had received steroid therapy (steroid therapy (+), n = 32; (-), n = 19). Corticosteroids were administered at an initial dose of 10-20 mg/day after CCE diagnosis. The values of the estimated glomerular filtration rate (eGFR) in the two groups were examined at CCE diagnosis, 4 weeks after diagnosis and the last follow-up. Additionally, the % change in eGFR at 4 weeks after diagnosis and % change per year in eGFR at the last follow-up were calculated for each patient., Results: The median values of eGFR at diagnosis in patients with and without steroid therapy were 16.4 and 17.9 mL/min/1.73 m(2), respectively. The median % change in eGFR between diagnosis and 4 weeks after diagnosis was 24% in patients with steroid therapy and 5% in those without, and this difference was statistically significant. On the other hand, there was no significant difference between the two groups in the % change in eGFR per year between diagnosis and the last follow-up., Conclusions: During the short period after CCE diagnosis, steroid therapy showed a good renal outcome in CCE patients. However, this treatment did not have a favorable effect on long-term renal outcome.

A 65-year-old man with rheumatic combined valvular heart disease showed a persistent fever after cardiac catheterization. He was diagnosed with cholesterol embolism due to multiple mobile plaques in the descending thoracic aorta by transesophageal echocardiography (TEE) along with persistent eosinophilia, deteriorating renal function, and blue toe sign. He was treated with intensive cholesterol-lowering therapy for 3 years, resulting in marked regression of the aortic plaque on TEE.

A 72-year-old man was admitted to our hospital because of progressive renal dysfunction persisting for 1.5 months. Physical examination showed livedo reticularis of the toes of both feet, peripheral edema, and gait disturbance due to the toe pain. The levels of blood urea nitrogen (50.0 mg/dL) and creatinine (2.81 mg/dL) were elevated, and eosinophilia (10%, 870/µL) was noted. A biopsy of the area of livedo reticularis revealed cholesterin crystals. The patient had not undergone angiography, anticoagulation therapy, or antithrombotic treatment. Idiopathic cholesterol crystal embolization was diagnosed. Transesophageal echocardiography revealed intimal thickening of the aorta and plaque. Oral steroid therapy was started because of the progressive renal dysfunction. After steroid therapy, the symptoms improved. Early diagnosis and treatment are important. Renal dysfunction is a common symptom in elderly patients. Cholesterol crystal embolization should also be considered as a cause of unexplained renal dysfunction, especially in such patients.

A 73-year-old man was admitted to our hospital because of bilateral foot pain. He was treated with thrombolysis for cerebral infarction about 5 months ago. Anticoagulants had not been used because of hemorrhagic infarction. The pulses of bilateral pedal arteries were palpable, but cyanosis was present in the bilateral toes. Laboratory data indicated azotemia and eosinophilia. Magnetic resonance imaging revealed multiple plaques of the thoracic and abdominal aorta, one of which was ulcerated. Skin biopsy proved the diagnosis of cholesterol crystal embolism (CCE). Because no invasive vascular procedure was performed, we assumed that CCE in this patient was related to thrombolysis. We should be cautious for late onset of CCE after thrombolysis.

Instrumentation of the aorta during cardiac catheterization, resulting in peripheral embolization, is an underdiagnosed clinical entity. Such an atheromatous embolization can present in a subtle way or could be catastrophic. Isolated splenic infarction as a complication of the procedure is extreme rare. We report a 59-year-old man with risk factors for atherosclerotic vascular disease who underwent percutaneous coronary intervention and presented 3 days later with isolated splenic infarction. He was managed conservatively with heparin. Further evaluation revealed a concomitant mural thrombus in an abdominal aortic aneurysm, which could be a contributing factor along with atheroembolization from advanced atherosclerosis. Our case highlights the importance of using a right brachial or radial approach in an individual with significant atherosclerotic vascular disease and with an abdominal aortic aneurysm.

Atherothrombosis is the leading cause of death worldwide and has a large economic impact. It is a pathologic process related to atherosclerosis, which leads to adverse clinical manifestations, including acute coronary syndrome, cerebrovascular disease, and peripheral arterial disease. Patients with atherothrombosis are at heightened risk for recurrent ischemic events or death, and therefore, secondary prevention is an important goal in the treatment of these patients. Antiplatelet therapies available for long-term secondary prevention include aspirin (acetylsalicylic acid), extended-release dipyridamole plus aspirin, and clopidogrel. A number of clinical trials have demonstrated the benefit of combined antiplatelet therapy in secondary prevention, supporting the recommendations made in current published guidelines. Although the efficacy and safety of antiplatelet agents is well established and supported by clinical trials, their utilization rate in patients with atherothrombosis remains suboptimal. Quality improvement initiatives have demonstrated effectiveness in promoting the awareness and implementation of treatment guidelines. This article reviews the benefits and risks of antiplatelet therapy in patients with cardiovascular disease with the aim of spurring greater adherence to treatment recommendations and, thereby, better patient outcomes.

Atherothrombosis is responsible for most acute ischemic manifestations of atherosclerotic disease, including stroke. Individuals with evidence of atherothrombotic disease in one vascular bed have a significant risk of recurrence and show increased vulnerability over time for other manifestations elsewhere in the vasculature. Ischemic event rates for asymptomatic patients with multiple atherothrombotic risk factors appear to be similar to those in patients with documented cardiovascular disease. For example, diabetes mellitus and obesity are found at alarmingly high rates in patients with prior cardiovascular events, including stroke or transient ischemic attacks. Antiplatelet therapy is a key component of atherothrombotic event prevention. The results of the Clopidogrel for High Atherothrombotic Risk and Ischemic Stabilization, Management and Avoidance (CHARISMA) study showed that while dual antiplatelet therapy with aspirin and clopidogrel may not play a role in primary prevention, post hoc analysis alluded to the possibility of benefit for dual antiplatelet therapy in certain populations of stroke patients. We examined current recommendations for the prevention of atherothrombotic events, focusing on the role of oral antiplatelet agents in patients with ischemic stroke.

Atheroembolic renal disease is a part of a multisystem disease and can be defined as renal failure secondary to the occlusion of renal arterioles and glomerular capillaries with cholesterol crystal emboli deriving from the aorta and other major arteries. The kidney is usually involved because of the proximity of the renal arteries to abdominal aorta (where the erosion of atheromatous plaque is most likely to occur), and the high renal blood flow. Cholesterol crystal embolism can also occur in other visceral organs, as well as in the upper and lower extremities. Embolization may occur spontaneously or after angiographic and surgical procedures, and anticoagulation. Atheroembolic renal disease is an important yet underdiagnosed component of the spectrum of kidney diseases associated with atherosclerosis and remains an unexplored field of nephrology research.

We report an 81-year-old male with cholesterol crystal embolization (CCE). He had been treated with warfarin for atrial fibrillation and an old cerebral infarction but had recurrence of cerebral infarction after warfarin was discontinued as treatment of CCE. Anticoagulation therapy by heparin in addition to warfarin was restarted, with the result that symptoms of cerebral ischemic damage did not recur and skin manifestations of CCE rapidly improved. These results suggest the possibility that heparin may be effective in the treatment of CCE, because CCE is not the result of pure embolization by cholesterol crystals but is a result of a combination with simultaneous thrombi.

Background: Atherothrombosis is associated with significant mortality and morbidity in both men and women. Management of this disease, however, is generally guided by evidence from trials conducted predominantly in men, with few studies focused on women alone. Our objective was to review the characteristics and management of atherothrombotic disease in women, with particular emphasis on the therapeutic role of antiplatelet agents., Methods: Landmark clinical trials and other studies pertaining to atherothrombosis in women (including risk factors, clinical outcomes, and treatment patterns) were obtained through Pubmed and Medline literature research and reviewed., Results: Primary prevention studies indicate a clear benefit of antiplatelet therapy in the reduction of cardiovascular events and, in particular, the risk of stroke in women >or=65 years. In men, this benefit is attributable to a reduction in the risk of myocardial infarction (MI). Combination therapies, including aspirin plus dipyridamole or clopidogrel, effectively reduce the risk of recurrent ischemic events in women. However, the effects are not consistent between men and women across studies. Similarly, gender differences exist in the effect of intravenous glycoprotein IIb/IIIa inhibitors plus aspirin on the risk of death or cardiovascular events., Conclusions: The effects of antiplatelet therapy regimens differ between men and women. The mechanisms underlying these differences are still to be elucidated; this report highlights the need for more studies focused on women in order to optimize gender-specific therapy and, therefore, improve clinical outcomes in women with atherothrombosis.

Cardiovascular disease is prevalent among patients with stroke; thus, cardiologists frequently treat patients at high risk for stroke. Results from recent clinical trials of antiplatelet medications, given alone or in combination, may be of special interest to cardiologists. The MATCH study demonstrated no significant difference between clopidogrel alone and clopidogrel plus aspirin in reducing risk of vascular events after stroke or transient ischemic attack. A 1.3% increased risk of major bleeding was associated with clopidogrel plus aspirin. In CHARISMA, clopidogrel plus aspirin did not reach statistical significance vs. placebo plus aspirin in reducing incidence of myocardial infarction (MI), stroke, or death from cardiovascular causes in patients with stable atherothrombotic disease; clopidogrel was associated with an increase in moderate bleeding. These results suggest that clopidogrel plus aspirin may be inappropriate as first-line therapy for secondary stroke prevention. In patients with established cardiovascular disease at risk for MI or other vascular events, physicians must weigh the benefits and risks before choosing this therapy. Selection of an antiplatelet agent must be based on patient history, including previous MI and stroke, susceptibility to bleeding, and other high-risk factors (e.g. advanced age and diabetes). Aspirin plus extended-release dipyridamole may be more effective than clopidogrel for preventing stroke in high-risk patients. This article strives to put MATCH and CHARISMA results into context by providing an overview of antiplatelet therapy, including relevant clinical trial results, a review of current practice guidelines, and a summary of an ongoing study that will improve clinical decision making.

Cholesterol crystal embolism (CCE) is a systemic disease resulting from shedding of cholesterol crystals into the small vessels of multiple organs, including skin, kidney, gastrointestinal tract and others. Recently, neuroendovascular therapeutic procedures for athrosclerosis disease is increasing. We report a case of CCE after carotid stenting (CAS). A 73-year-old man with asymptomatic carotid stenosis was treated by percutanenous transluminal angioplasty with stenting. CAS was achieved in a short time without trouble. About 1 month after CAS, his renal function deteriorated and purpura appeared on both toe tips (blue toe syndrome) with muscle pain of the lower extremities. Under diagnosis of CCE, he was treated by Predonisolone 20 mg/day and Valsartan 160 mg/day, Pravastatin 10 mg/day. His symptom's dramatically improved, with partial recovery of renal function. CCE rarely occurs after angiographic or interventional procedures, but is difficult to diagnose clinically and there is no established therapy. For early diagnosis of CCE strict follow-up of a patients clinical presentation and laboratory data, especially in high risk patients, is needed.

Background: Cholesterol crystal embolism (CCE) is a complication of atherosclerosis. Vascular surgery, vascular angiography, and anticoagulation have been identified as inciting factors., Hypothesis: This paper sought to analyze the clinical characteristics of patients with CCE after percutaneous coronary intervention., Methods: Six patients with atherosclerosis presenting with simultaneous occurrence of acute renal failure and peripheral ischemic changes were diagnosed with CCE and their clinical data were analyzed., Results: The average age of the patients was 72 years. Most had risk factors of atherosclerosis such as hypertension, diabetes, and smoking. The levels of serum creatinine increased progressively after coronary angiography. All patients had concomitant skin lesions, including blue toes. Cholesterol crystal emboli were found in arterioles by cutaneous biopsy in one patient. All patients received statins and two of these received dialysis therapy. Three patients died and three remained in chronic renal failure., Conclusion: Since CCE is a severe complication of coronary intervention, special attention should be paid to this disease.

Aims: The prognosis of renal cholesterol crystal embolism (CCE) is poor. Although various treatments for CCE have been attempted, there is no optimal therapy. We tested the effect of low-dose prednisolone (PS) on CCE-related acute renal failure (ARF)., Patients and Methods: 7 patients (mean age 69 years) diagnosed with CCE-related ARF were treated with oral PS at 15-20 mg/day for 2-4 weeks, which was then tapered at 5 mg/day over 2-4 weeks, followed by 5 mg/day maintenance dose. Recurrent ARF during PS tapering was treated with a larger dose of PS., Results: Inciting factors were identified in four patients: coronary angiography (n=3) and cerebral angiography (n=1). On admission, serum creatinine (SCr) was 2.1 +/- 0.3 mg/dl (mean +/- SEM). SCr and eosinophil count before treatment were 4.2 +/- 0.4 mg/dl and 682 +/- 73/microl, respectively. PS therapy improved ARF in all cases at week 2 (SCr 3.8 +/- 0.5 mg/dl) parallel to a decrease in eosinophilia (116 +/- 30/microl), and at week 4 (3.1 +/- 0.4 mg/dl and 134 +/- 20/microl, respectively). At last follow-up, renal function was improved or maintained in 5 patients compared with that at week 4 post-treatment. One patient died of lung cancer. Another required LDL apheresis and hemodialysis but died due to CCE-related multi-organ failure. A third patient had recurrent ARF and was re-treated with a larger dose of PS, which resulted in an immediate decrease in SCr. However, the patient developed acute renal dysfunction due to congestive heart failure, and required hemodialysis., Conclusions: Low-dose PS improved CCE-related ARF, probably through amelioration of inflammatory reaction surrounding affected renal vessels.

Although cholesterol embolism syndrome was recognized as a clinicopathologic entity more than 50 years ago, it is attracting growing attention recently. It is a multisystemic disorder in which cholesterol crystals released from atherosclerotic plaques obstruct small arterioles, resulting in local ischemia and end-organ damage. There are no established treatments, and with the limited treatment options available, it is important to make the diagnosis as early as possible. We present the case of a 68-year-old man with cholesterol embolism who had a few fluttering atheromas in the aorta, as demonstrated by transesophageal ultrasonography. The diagnosis was confirmed by skin biopsy, and treatment with statins and steroids proved effective, as renal failure progressively improved. This case emphasizes the importance of early diagnosis and shows the possible therapeutic effects of statins and steroids for patients with this syndrome.

Cholesterol crystal embolization (CCE) is a dreaded complication of radiology, vascular surgery, and/or anticoagulation in patients with atherosclerosis and ulcerated aortic plaques. It also represents a cause of early graft failure and of poor results of renal artery surgery. Crystals lodge in small caliber renal arteries, where they induce early, transitory thrombosis followed by delayed, definitive obstruction by endarteritis, accompanied by evidence of inflammation and eosinophilia. Massive CCE leads to early oligoanuria. In subacute forms, renal insufficiency is often delayed by weeks or months following the triggering event. A third, chronic subset of CCE is easily mistaken for atherosclerotic renal ischemia and/or nephrosclerosis. The kidney is rarely the sole organ involved in acute/subacute forms, in which the central nervous system, the coronary arteries, the spinal cord, and the mesenteric and pancreatic blood supply compromise represent the main causes of death. Cutaneous, retinal, and muscle involvement allow diagnosis by inspection or scarcely invasive biopsies in about 80% of cases, whereas renal biopsy as the only diagnostic procedure is required in 20% of cases. Prevention is based on avoidance of endovascular radiology maneuvers, vascular surgery, and excess anticoagulation in atherosclerotic patients. Treatment of acute/subacute forms of renal insufficiency consisting of stopping anticoagulation and forbidding any new radiologic and/or vascular surgery procedure; treating hypertension with angiotensin 2 antagonists and vasodilators, strict volemic control by loop diuretics and ultrafiltration, along with parenteral nutrition and prednisone, has been credited with improved outcome. Iloprost may obtain favorable results. Statins definitely ameliorate the renal and patient's prognosis.

Background: Cholesterol crystal embolism syndrome (CCE) is an increasing end-stage renal disease cause. Few cases have been described on dialysis, despite the high prevalence of the predisposing factors., Methods: The diagnostic criteria of the present study were: skin lesions, myalgia, fatigue, fever and acute inflammatory serologic signs, in the presence of severe vasculopathy. The precipitating factors were: anticoagulation, endovascular intervention and ulcerated atherosclerotic plaque., Results: Between October 2003 and September 2005, CCE was diagnosed in 6 dialysis patients (of 200-210 on chronic treatment): 5 males, 1 female, median age 59.5 years (47-70) and end-stage renal disease follow-up 11.5 years (3-25). All had severe vasculopathy, 5 cardiopathy, and 4 were failed graft recipients. The treatment included: peritoneal dialysis, daily dialysis, 'conventional' hemodialysis (2 cases) and hemodiafiltration. The diagnosis was based on the clinical-laboratory picture in 1 patient. In the 5 others clues were present (dicumarol therapy, angioplasty, femoral artery thrombosis, CCE predialysis and ulcerated aortic plaque). The therapeutic approach consisted of corticosteroids (5 cases), statins (4 cases) and prostaglandin analogues (4 cases)., Conclusion: The differential diagnosis of CCE should also be considered in dialysis patients (necrotic lesions, limb pain and vasculitis-like signs).

Cholesterol crystal embolism (CCE) is a multivisceral disease caused by occlusion of small arteries with cholesterol crystal emboli deriving from eroded atherosclerotic plaques of the aorta and/or large feeder arteries. The factors precipitating CCE are manipulation of the aorta or other large arteries during arteriography or surgery, and anticoagulant or thrombolytic therapy. CCE has been reported to be a life threatening condition involving multiple vital organ dysfunction, including renal failure, cardiac failure, skin ischemic lesions such as livedo reticularis, patchy skin necrosis, and purple toes, gastrointestinal ischemia, and/or visual disturbance. We report a 63-year-old male patient of CCE after percutaneous transluminal coronary angioplasty, who contracted severe pneumonia of Pneumocystis carinii and Cytomegalo virus during steroid therapy (prednisolone 20 mg for 3 months). He was treated successfully with mechanical ventilation, hemodialysis, and appropriate antibiotic therapy. Although corticosteroid therapy has been reported to be effective in some CCE patients, the indications of steroid therapy, dosage of corticosteroids, duration of the treatment, or efficacy of prophylactic administration of antibiotics are not yet established. Further interventional studies are required in order to evaluate the benefit of corticosteroid therapy for CCE.

Cholesterol crystal embolism (CCE) is a systemic refractory disease especially prevalent amongst elderly patients suffering from atherosclerosis. Treatment of this condition remains controversial due to difficulties in diagnosis. Corticosteroid therapy may be an important treatment option despite its elusive mechanisms. To clarify the role of corticosteroid in CCE therapy, we collected the samples from six autopsied subjects with CCE, three of whom were clinically given various doses of corticosteroid to investigate stable atherosclerosis-related substances, advanced glycation end-products (AGE), and several AGE receptors such as scavenger receptor class B type 1 (SR-B1), receptor for AGE (RAGE), and galectin-3 in the liver tissues and atherosclerotic areas by immunostaining using a tissue macro-array technique. An intense expression of AGE and its receptors was identified in the enlarged Kupffer cells of CCE cases, which were given relatively high doses of corticosteroid. In addition, numerous mononuclear cells in the intimal atheromatous plaque presented strong expressions of AGE and SR-B1. In conclusion, we speculated that corticosteroid treatment for CCE may upregulate the activations, including phagocytic capacity of Kupffer cells mediated by overexpression of RAGE and scavenger receptors, resulting in efficient clearance of the lipid substances from the blood circulation released from atherosclerotic areas.

Cholesterol embolization (CE) is a potentially serious complication associated with invasive arterial maneuvers, in which standard therapy has not been established. We experienced two cases of CE in patients with severe atherosclerosis whose renal function deteriorated within a few months after invasive arterial maneuvers. CE was confirmed either by renal biopsy (case 1) or skin biopsy (case 2). Oral administration of prednisolone at a daily dose of 30 mg (0.4 mg/kg) was effective to improve their renal function. Our observation suggests that corticosteroid therapy may be beneficial in some patients with CE.

Cholesterol crystal embolization (CCE) is characterized by tissue ischemia secondary to occlusion of small arteries. It may occur spontaneously but more often follows radiological interventional procedures or vascular surgery. This systemic disease affects multiple organs, including skin, kidney, brain, eye, and gastrointestinal tract. We reported a Japanese male CCE patient with cutaneous manifestations of livedo reticularis, diarrhea, clouding of consciousness, and acute renal failure. Histopathological examination demonstrated multiple biconvex clefts in a vessel of the subcutis. Corticosteroid administration improved his consciousness, diarrhea and skin lesions. Awareness of the skin manifestations of CCE is essential for dermatologists to make an early diagnosis and prescribe appropriate treatment.

Cholesterol crystal embolization is a rare but recognized complication of cardiac catheterization. While management has traditionally been supportive only, we demonstrate the successful use of corticosteroids in treatment of a patient with cholesterol crystal embolization to the distal extremity.

A 65-year-old man developed acute renal failure with eosinophilia two weeks after a coronary bypass operation and angiography. Renal biopsy revealed cholesterol crystal embolism (CCE) in glomeruli and arterioles. Low-dose corticosteroid therapy failed to recover the renal function; further deterioration of renal function and peripheral ischemic symptoms such as livedo reticularis and blue toes occurred. However, steroid pulse therapy successfully attenuated CCE-induced renal failure and eosinophilia. It is suggested that steroid pulse therapy might be effective to treat CCE-induced renal failure and eosinophilia could be a useful marker for activity of CCE.

Cholesterol crystal embolization (CCE) is a complication of atherosclerosis. A 67-year-old Japanese man underwent coronary artery bypass grafting. After the surgery, he underwent coronary angiography via the right femoral artery. Twelve days later, he suddenly developed acalculous cholecystitis and was treated with antibiotics. Gradual deterioration in renal function, purplish discoloration of the distal portion of his toes, and eosinophilia were noted. We performed a skin biopsy and made a diagnosis of CCE. Cilostazol and intravenous heparin improved the symptoms and decreased the creatinine level. We retrospectively studied the clinical features of 36 cases registered with a diagnosis of CCE in the Japanese literature.

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.

A 70-year-old woman developed localized livedo racemosa on the right buttock 2 months after renal transplantation. The suspected diagnosis of cholesterol embolism was confirmed by histopathological demonstration of cholesterol crystals in the wall of a small artery. The gluteal region is supplied by blood vessels that branch off the internal iliac artery distal to the usual anastomosing site of the donor kidney. Cholesterol emboli dislocated in the course of a kidney transplantation can reach the gluteal region. Cholesterol embolism after renal transplantation as well as the observation of cholesterol crystals in the histological preparation are rare findings.

Renal atheroembolic disease presents several multisystemic signs and symptoms that may resemble the clinical signs of vasculitis. A few cases of renal atheroembolism associated with antineutrophil cytoplasmic antibodies (ANCA) have been described. We report the first case, to our knowledge, of a patient with cholesterol microemboli who also developed extracapillary glomerulonephritis in the presence of perinuclear ANCA (p-ANCA) with anti-myeloperoxidase ANCA. The patient had long-term follow-up of renal function after treatment with prednisone and cyclophosphamide., (Copyright 2002 by the National Kidney Foundation, Inc.)