Angiopathy

Angiopathy is a general term that describes diseases of blood vessels, particularly when small vessels become damaged and fragile, leading to bleeding and other complications throughout the body.

Table of contents

• What is angiopathy?
• Main types of angiopathy
• Symptoms
• Causes and risk factors
• Medical tests for diagnosis
• Complications
• Treatment approaches
• Prevention measures
• Medical specialists

What is angiopathy?

Angiopathy is a general medical term that means disease of the blood vessels. These blood vessels include arteries (which carry oxygen-rich blood from the heart), veins (which return blood to the heart), and capillaries (the smallest blood vessels that connect arteries and veins)^[1]. While the term can apply to any blood vessel disease, it most commonly refers to conditions where small blood vessels become damaged and burst open^[1].

Angiopathy can affect blood vessels in different parts of the body, leading to various health problems. The disease weakens vessel walls, making them prone to bleeding and reducing their ability to deliver blood effectively to organs and tissues^[5]. This can result in serious complications depending on which blood vessels are affected and where in the body they are located.

Main types of angiopathy

There are two main types of angiopathy based on how the disease affects blood vessels:

Congophilic angiopathy, also known as cerebral amyloid angiopathy (CAA), involves the buildup of amyloid proteins in the walls of blood vessels in the brain. These abnormal protein deposits accumulate in the walls of cerebral vessels over time, which weakens them and causes an increased risk of stroke^[1]. The disease appears when there is a rupture of the affected vessel, leading to bleeding in the brain^[1]. This type of angiopathy is strongly age-dependent and most commonly affects people over 60 years old^[2].

Diabetic angiopathy involves damage to blood vessels caused by high levels of glucose in the blood. This condition typically affects people with diabetes and can cause damage throughout the body^[1]. Diabetic angiopathy can affect both large vessels (macrovascular disease) and small vessels (microvascular disease)^[6]. It commonly presents as diabetic retinopathy (damage to the eyes) or diabetic nephropathy (damage to the kidneys)^[1].

In microangiopathy, the walls of small blood vessels become so thick and weak that they bleed, leak protein, and slow the flow of blood. For example, diabetics may develop microangiopathy with thickening of capillaries in many areas, including the eye^[5]. In macroangiopathy, fat and blood clots build up in large blood vessels, stick to the vessel walls, and block the flow of blood. This can affect the heart (coronary artery disease), the brain (cerebrovascular disease), or vessels in the legs (peripheral vascular disease)^[5].

Amyloidosis – cerebral, CAA, Congophilic angiopathy

Symptoms

The symptoms of angiopathy depend on which blood vessels are affected and how severe the damage is. Many people with angiopathy may have no symptoms at all, especially in the early stages^[2].

When cerebral amyloid angiopathy causes bleeding in the brain, symptoms usually occur because the brain tissue becomes damaged. Bleeding often occurs in the outer parts of the brain, called the cortex, and not the deep areas^[2]. If there is significant bleeding, immediate symptoms can resemble a stroke and may include^[1]:

• Sleepiness or drowsiness
• Headaches, usually in a certain part of the head
• Difficulties with vision
• Difficulty speaking
• Muscular weakness or sudden weakness
• Confusion or changes in mental function
• Seizures

Some people have gradual memory problems and may not realize they have had bleeding in the brain^[2]. In some cases, people learn they have cerebral amyloid angiopathy after having a brain scan for another issue^[2]. If bleeding is not severe or widespread, symptoms can include episodes of confusion, headaches that come and go, loss of mental function (dementia), and weakness or unusual sensations that come and go^[2].

Often, blood flow from damaged vessels is like a very slow leak. Most people won’t notice changes like memory loss or confusion right away. When they do notice symptoms, they may think these are a natural consequence of growing older. But symptoms get worse over time as amyloid proteins do more damage to blood vessel walls and larger amounts of blood flow into the brain^[2].

• Brain blood vessels
• Cerebral cortex
• Leptomeninges
• Retina (eyes)
• Kidneys
• Legs and feet

Causes and risk factors

The exact cause of congophilic angiopathy is unknown. Sometimes it can be transmitted from parents to children, making it hereditary^[1]. However, there have also been cases where it has developed at an advanced age, especially in people over 55 years old^[1]. Experts estimate that 23% to 29% of people in the general population above the age of 50 have moderate to severe cerebral amyloid angiopathy^[2].

For cerebral amyloid angiopathy, experts don’t know the exact cause, but research suggests the following conditions or situations may increase risk^[2]:

• Alzheimer’s disease
• Race – people who are white are more likely to have CAA than people in other ethnic groups
• Hypertension (high blood pressure)

Inherited cerebral amyloid angiopathy is very rare. It can develop quickly if you inherit certain genetic mutations (changes) that cause the condition. The most common type of inherited cerebral amyloid angiopathy causes life-threatening strokes. Most people with this type of cerebral amyloid angiopathy die within 10 years of developing symptoms^[2].

For diabetic angiopathy, the main risk factors include^[6]:

• Diabetes caused by poor diet
• High blood pressure or hypertension
• High cholesterol
• Tobacco smoking
• Family history of peripheral artery disease or coronary artery disease

Medical tests for diagnosis

To diagnose angiopathy, the doctor will carry out a physical examination, followed by diagnostic imaging techniques^[1]. For cerebral amyloid angiopathy, there is no conclusive way to diagnose this condition while a person is alive. The only way to do that with certainty is to examine samples of a person’s brain under a microscope, which is only possible during an autopsy after death^[2].

However, doctors can use imaging tests to support a diagnosis. Tests may include^[1]:

• Computed tomography scan (CT scan) to check for bleeding in the brain
• Magnetic resonance imaging (MRI) to look at brain structures and detect bleeding
• Magnetic resonance angiography (MRA) to check for large bleeds and rule out other causes of bleeding
• Positron-emission tomography (PET scan) to check for amyloid deposits in the brain

It is important for the doctor to ask detailed questions about the symptoms and medical history. The symptoms and results of the physical exam and any imaging tests may cause the doctor to suspect CAA^[2]. A physical exam can be normal if the bleed is small, but there may be some brain function changes^[2].

In some cases, the doctor may need to take a sample of brain tissue to diagnose congophilic angiopathy^[1].

Complications

Angiopathy can lead to serious complications if not properly managed. The complications vary depending on which type of angiopathy is present and which blood vessels are affected.

Diabetic angiopathy can cause progressive damage to the eyes and kidneys if blood glucose levels are not kept under control^[1]. Congophilic angiopathy is also progressive and may result in complications such as^[1]:

• Dementia
• Seizures
• Brain bleeding episodes
• Hydrocephalus (rarely)
• Repeated episodes of bleeding in the brain

Cerebral amyloid angiopathy is a type of hemorrhagic stroke. Over many years, blood vessel walls gradually weaken and develop microscopic cracks or fissures that let blood seep into the brain. When blood leaks from ruptured vessels, it damages brain tissue^[2]. In some cases, a large brain bleed may lead to coma^[2].

Cerebral amyloid angiopathy is the most common cause of cognitive decline, which is when you gradually lose your thinking abilities^[2]. People who survive a stroke from inherited cerebral amyloid angiopathy often have dementia, epilepsy, and recurring strokes that do further damage to their brains^[2].

Treatment approaches

There is no known effective treatment to cure angiopathy. At the moment, there is no cure for this condition, so angiopathy treatment is aimed at reducing the symptoms^[1]. The goal of treatment is to relieve symptoms and manage issues that may increase bleeding in the brain^[2].

Treatment options that can help relieve angiopathy symptoms include^[1]:

• Physiotherapy for cases where there is muscle weakness
• Occupational therapy to help with daily activities
• Speech therapy for difficulty speaking
• Medications for memory loss
• Anti-seizure medications to treat possible seizures (also called amyloid spells)

In some cases, rehabilitation is needed for weakness or clumsiness. This can include physical, occupational, or speech therapy^[2]. Sometimes, medicines that help improve memory, such as those for Alzheimer disease, are used^[2].

The disorder slowly gets worse over time^[2]. Because there are no disease-modifying treatments currently available, management focuses on controlling risk factors and preventing complications.

Prevention measures

You cannot prevent congophilic angiopathy, but you can help prevent diabetic angiopathy by taking certain measures^[1]:

• Follow a balanced and low-fat diet
• Stop smoking and drinking alcohol
• Exercise on a regular basis

For people with diabetes, keeping blood glucose levels under control is essential to prevent or slow the progression of diabetic angiopathy and avoid damage to the eyes and kidneys^[1].

While there are no specific prevention strategies for cerebral amyloid angiopathy, managing related health conditions like high blood pressure and avoiding certain risk factors may help slow the progression of the disease.

Medical specialists

The specialists that treat angiopathy are vascular surgeons^[1]. These doctors specialize in conditions affecting the blood vessels throughout the body. Depending on the type and location of angiopathy, other specialists may also be involved in care, including neurologists for cerebral amyloid angiopathy and endocrinologists for diabetic angiopathy.