Cholesterol embolism syndrome

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Classification according to ICD-10
I74 Arterial embolism and thrombosis
N28 Kidney ischemia and infarction
- renal artery embolism
K55.0 Acute vascular diseases of the intestine
- mesenteric artery embolism
H34 Retinal vascular occlusion
ICD-10 online (WHO version 2019)
Microfoto showing a cholesterol embolism of the kidney . Kidney biopsy. HE staining .

The cholesterol embolism syndrome (also cholesterol crystal embolism syndrome ) is caused by the occlusion of small arteries by the flooding (embolism) of cholesterol crystals from broken (ulcerated) arteriosclerotic plaques . Downstream (distal) of the occlusion, there is reduced blood flow ( ischemia ) and inflammation . In about 25% of the cases the release of cholesterol occurs spontaneously, in about 75% of the cases it is caused by medical measures such as angiography , vascular surgery , anticoagulant drugs ( anticoagulants ) or thrombolysis . The abdominal artery is usually the origin of the crystals, the deposits are mainly in the kidneys , intestinal tract , skin , legs and retina of the eye. The diagnosis is difficult because multiple organ systems can be affected simultaneously. The triad of triggering event, kidney failure and embolism symptoms is diagnostically indicative, especially if there is an increase in eosinophilic granulocytes (eosinophilia) in the blood count . The diagnosis is made by examining the fundus and taking tissue samples from the skin and kidneys. The prognosis is poor, the disease often turns into chronic kidney failure, and mortality is increased. The course is favorably influenced by fat-lowering drugs from the substance class of statins .

Pathogenesis

Cholesterol embolism syndrome comes in two forms:

  • The acute form is the result of a massive shower of cholesterol crystals or repeated embolisms due to sudden or repeated ruptures (ruptures) of unstable plaques.
  • The chronic form is the result of a continuous release of cholesterol crystals from superficially injured (eroded) plaques.

The most common cause is a cardiac catheterization through the femoral artery , followed by anticoagulant drugs for fibrinolysis and anticoagulation and surgery on the heart and vessels. Atherosclerotic plaques can be torn open by manipulation with catheters. The soft cholesterol-containing content can get into the bloodstream . Anticoagulant substances can lead to bleeding into plaques and to their rupture, or dissolve thrombi that cover ruptured plaque and release the cholesterol-containing contents. Spontaneous cholesterol embolism syndrome is rare, and 2/3 of the cases are chronic. The (iatrogenic) cholesterol embolism syndrome caused by medical measures , on the other hand, occurs acutely or subacute in over 90% of cases and has a worse prognosis than the spontaneously occurring.

Clinical manifestations

Cholesterol embolism syndrome primarily leads to acute or chronic kidney failure , as the kidney is closest to the abdominal aorta, the origin of the cholesterol crystals, and has a very high blood flow. In addition, there are mostly systemic symptoms in the area of ​​the skin, the gastrointestinal tract (gastrointestinal tract) and the nervous system . Characteristic changes in the area of ​​the skin are livedo reticularis , a purple reticular rash in the area of ​​the abdominal wall and legs, small hemorrhages in the nail bed and purple toes . Symptoms of the gastrointestinal tract include abdominal pain, nausea, vomiting, occult or visible blood in the stool. In the area of locomotor muscle and joint pain may occur. If the central nervous system is affected , it can lead to states of confusion, neurological failures and fleeting blindness (amaurosis fugax) . General symptoms such as feeling sick, fever, and weight loss are common.

Laboratory diagnostics

The laboratory diagnosis often shows a constellation of inflammation with increased erythrocyte sedimentation rate , increased CRP , eosinophilia and reduced complement . The urinalysis may be normal, but it can also red blood cells , protein or eosinophilic granulocytes be detectable in urine.

Risk factors

Risk factors for developing choesterine embolism syndrome are:

diagnosis

The diagnosis can be made without a histological examination if the following conditions are met: Triggering event (e.g. cardiac catheter examination),

  • Signs of peripheral embolism (e.g. typical skin changes) and
  • acute or subacute kidney failure.

The occurrence of gastric or intestinal bleeding (gastrointestinal bleeding) or neurological symptoms or increased eosinophilic granulocytes (eosinophilia) confirm the diagnosis.

In cases of doubt, the diagnosis can be confirmed by histological examination of a tissue sample from the kidney, skin or the gastrointestinal tract. In some patients, the diagnosis can be made by examining the fundus of the eye with evidence of cholesterol emboli in the vessels. This means that kidney puncture can often be dispensed with.

In the differential diagnosis , systemic diseases must be taken into account, such as B. vasculitis , subacute bacterial endocarditis, or polymyositis .

Histopathology

The histological examination of the kidney biopsy shows a blockage of arterioles and glomerular capillaries by biconvex needle-shaped fissures from which the cholesterol crystals were loosened as part of the preparation of the preparations for examination. In the further course, neutrophils and eosinophilic granulocytes migrate into the closed vessels, later monocytes and multinucleated giant cells . Finally it comes to the scarred closure of the vessel. Downstream, the reduced blood flow leads to scarring of kidney corpuscles (glomeruli) and kidney tubules (tubules).

forecast

The prognosis for cholesterol embolism syndrome is poor. Around a third of those affected remain dependent on dialysis treatment in the long term . The 1-year survival rate is 83%, after two years 75% of those affected are still alive. Unfavorable prognostic factors are:

  • older age
  • Side diseases , especially diabetes and heart failure
  • pre-existing impairment of kidney function
  • acute or subacute onset with rapid loss of renal function
  • Involvement of additional organs (extrarenal manifestation), especially the legs and gastrointestinal tract
  • iatrogenic cause (triggered by a medical measure)

The most common causes of death are diseases of the cardiovascular system (80%), followed by infections (10%) and circulatory disorders of the gastrointestinal tract, pancreatitis and cachexia .

therapy

The administration of statins improves the prognosis, even if the use was only started after the diagnosis. This may be due to the lipid-lowering and anti-inflammatory properties of statins. Corticosteroids had no effect. Randomized, controlled studies have not yet been carried out.

swell

  • Kulwant S. Modi et al: Atheroembolic Renal Disease . In: J Am Soc Nephrol . No. 12 , 2001, p. 1781–1787 ( Article ).
  • F. Scolari: The challenge of diagnosing atheroembolic renal disease clinical features and prognostic factors . In: Circulation . No. 116 , 2007, pp. 298-304 ( Article ).

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