Vascular inflammation

Overview: What is vasculitis?

Inflammation can occur in various parts of the body. The blood vessels, which transport oxygen, nutrients and metabolic waste products to almost every tissue in the body, can also become inflamed. Medical professionals refer to vasculitis or vasculitides in the plural when referring to vascular inflammation. In principle, the disease can affect all types of vessels: Arteries (arteritis), veins (phlebitis) or lymph vessels (lymphangitis). However, arterial vessels are most frequently affected. A distinction is made between primary and secondary vasculitis – depending on the cause. Primary vasculitis is an autoimmune disease in its own right. The immune system attacks the body’s own structures and causes inflammation there – in this case the blood vessels. Secondary vasculitis, on the other hand, occurs as a result of other diseases, infections or the intake of certain medications.

According to the Chapel Hill Consensus Conference of 1994 and 2012, vasculitis is categorized as follows depending on the vascular involvement:

• Large vessels: giant cell arteritis (formerly temporal arteritis or arteritis temporalis), Takayasu arteritis
• Medium-sized vessels: Kawasaki syndrome, panarteritis nodosa
• Small vessels:
  • ANCA-associated vasculitides: microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) (formerly Wegener’s disease), eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome)
  • Immune complex-associated small vessel vasculitis: Cryoglobulinemic vasculitis (special proteins occur that damage vessels, especially at low temperatures), Schoenlein-Henoch purpura (or so-called IgA vasculitis), hypocomplementemic urticaria vasculitis
• Mixed arterial/venous vessels: Behcet’s syndrome, Cogan’s syndrome
• Vasculitis associated with systemic diseases in: Systemic Lupus Erythematosus, Rheumatoid Arthritis

A distinction is made between the primary vasculitides mentioned above, whose cause is unknown, and secondary vasculitides, where a trigger is often known. Some examples are:

• Autoimmune diseases such as rheumatoid arthritis, Crohn’s disease, systemic lupus erythematosus (SLE) or sarcoidosis
• Infectious diseases such as Lyme borreliosis, HIV disease (AIDS), liver inflammation (hepatitis) or infection with bacteria (e.g. streptococci)
• Cancer, for example lymphoma, blood cancer (leukemia) or multiple myeloma
• Medication: Some cause vascular inflammation as a side effect, such as antibiotics, antihypertensive drugs (ACE inhibitors) or cytotoxins (cytostatics in cancer = chemotherapy).

In the later course of these diseases, the function of the organs that are supplied by the inflamed vessels usually also suffers.

Vascular inflammation is rare

Vasculitides are diseases that occur rarely overall. They are difficult to diagnose because vascular inflammation can have many facets. Giant cell arteritis is most common in adults and often affects vessels in the head or chest area. Secondary vasculitis occurs more frequently than primary vasculitis.

Vascular inflammation: causes in the immune system

Vascular inflammations are autoimmune diseases. In some forms, this can be seen in the form of so-called autoantibodies (antibodies, i.e. proteins that are directed against the patient’s own body). Such autoantibodies are, for example, antineutrophil cytoplasmic antibodies, or ANCA for short. They are involved in the inflammation of small blood vessels, for example in granulomatosis with polyangiitis.

However, antibodies are also involved in the normal response of the immune system against pathogens (antigens), such as hepatitis or HIV viruses. If the immune system is particularly active, the antibody-antigen complexes can also damage blood vessels and trigger vascular inflammation. These are immune complexes that are deposited on the vessel walls and cause inflammation.

Symptoms: Vascular inflammation often causes general discomfort

Vascular inflammation is difficult to recognize, both for those affected and for medical staff, because it initially only causes very general symptoms. And these symptoms can occur in the context of many diseases, so they are not specific to vasculitis. The following symptoms are possible:

• (Severe) feeling of illness
• Fever
• Night sweats
• (Unintentional) weight loss
• Decrease in physical performance, loss of performance
• Sometimes joint pain

In addition, the symptoms depend on the type of vascular inflammation, i.e. whether large, medium-sized, small or all vessels are affected. For example, giant cell arteritis manifests itself differently from Behçet’s disease. In addition, the symptoms of vasculitis can vary in severity and affect a wide range of organs: Skin, eyes, nervous system, lungs, throat, nose and ears, kidneys, heart, muscles and joints or the gastrointestinal tract. An overview of some examples of the symptoms of the different types of vasculitis.

Large vessel vasculitis

Vascular inflammation that affects the large arteries of the body is called large vessel vasculitis. The two most important diseases in adults are Takayasu’s arteritis, which mainly occurs in younger patients, and giant cell arteritis, which mostly occurs in older patients (> 50 years). Inflammation of the large vessels can lead to occlusion of vessels, e.g. in the arm, in which case the hand is no longer supplied with blood as well. However, the inflammation can also lead to thinning of the vessel wall with bulges, which is called an aneurysm, and there is a risk of the blood vessel tearing at this point.

Giant cell arteritis

Giant cell arteritis is the most common form of vasculitis and affects the large blood vessels. Vascular inflammation usually occurs in the aortic arch and the vessels branching off from it. It particularly often affects older people. Symptoms of giant cell arteritis can include

• Headache in the temple area
• Pain when chewing
• General feeling of illness
• Loss of appetite, weight loss
• Increased body temperature
• Visual disturbances
• Pain in the limbs

Sometimes the vascular inflammation leads to vascular occlusion of the central retinal artery, which can result in severe eye damage and even blindness. A stroke is also possible due to vascular occlusion. The majority of patients also experience muscle and joint pain in the shoulder and pelvic girdle area at the same time or beforehand. This is called polymyalgia rheumatica.

Polymyalgia rheumatica

This clinical picture does not describe an actual vascular inflammation, but a pain syndrome of the shoulder and pelvic girdle with joint and muscle pain. There can also be a real swelling of the joints and you often feel very stiff in the muscles and joints, especially in the morning. It also leads to increased inflammation levels in the blood. Up to 50% of patients with PMR also develop giant cell arteritis.

Takayasu arteritis

Takayasu’s arteritis is also an inflammation of the large blood vessels, for example the aorta and its branches. This form of vasculitis often develops in younger women before the age of 40. Possible symptoms are

• Tiredness, fatigue, exhaustion
• Reduced physical performance
• night sweats
• Weight loss
• Occlusion of individual vessels (depending on the region in which the vessel is located): Pain in the arms, reduced pulse in one arm, different blood pressure in both halves of the body, symptoms of a stroke, visual disturbances, dizziness or chest tightness(angina pectoris)

• Further information on Takayasu arteritis

ANCA-associated vasculitis (AAV)

This term covers three diseases in which specific autoantibodies (the antineutrophil cytoplasmic autoantibodies, ANCAs) occur: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA).

Granulomatosis with polyangiitis

Granulomatosis with polyangiitis used to be called Wegener’s disease. Coarse nodular inflammations form on the small or medium-sized blood vessels. Vasculitis initially manifests itself particularly in the airways, e.g. the nose, paranasal sinuses, trachea and lungs. Later, the vascular inflammation can also affect other organs, such as the kidneys. The following symptoms are possible in granulomatosis with polyangiitis:

• Stuffy nose, runny nose with blood in the secretions, nosebleeds
• Sinusitis (inflammation of the paranasal sinuses)
• Irritating cough and shortness of breath if the vasculitis affects the lungs
• Other symptoms if it spreads to other organs, for example symptoms of kidney inflammation (e.g. water retention in the legs/feet)
• Nerve inflammation with tingling/numbness or paralysis
• General symptoms such as fever, tiredness, fatigue, back pain and weight loss

Microscopic polyangiitis

The clinical picture of microscopic polyangiitis (MPA) is similar to that of granulomatosis with polyangiitis. Microscopic polyangiitis most frequently leads to kidney damage, but can also affect the lungs. Involvement of the nervous system is also often possible.

Eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis used to be called Churg-Strauss vasculitis. Vascular inflammation usually affects the airways. Special white blood cells, the so-called eosinophil granulocytes, can be detected in the blood and inflamed tissue. Eosinophilic granulomatosis with polyangiitis can lead to the following symptoms:

• Sinusitis with polyp formation (sinusitis), bronchial asthma
• Joint pain

Later, other organs are often also affected by the vascular inflammation, for example the heart and kidneys. But the nerves can also be severely damaged if the disease is not treated.

Diagnosis of vascular inflammation

Vascular inflammation can manifest itself through a wide variety of symptoms, which are often of a very general nature and can occur in the context of many diseases. The symptoms are therefore often very difficult to classify. In addition, vasculitis is a rare disease. These factors also make the diagnosis so difficult.

The examination always begins with a discussion between you and your doctor to take your medical history (anamnesis). You will be asked about the nature of the symptoms, when they first occurred and how severe they are. Existing illnesses, such as hepatitis, and the intake of medication can also be important indicators. In addition to the medical history and physical examination, the following methods play a role in the diagnosis of vasculitis:

• Blood test and blood values: Various inflammatory markers are elevated in the blood in the case of inflammation. These include the C-reactive protein (CRP), the erythrocyte sedimentation rate (ESR) or the white blood cell count. However, elevated inflammation levels can be caused by a variety of different diseases. They alone are not meaningful enough. In certain forms of vasculitis, special antibodies, known as ANCA, can be detected. The abbreviation stands for antineutrophil cytoplasmic antibodies.
• Imaging examinations: Ultrasound (sonography), magnetic resonance imaging (MRI or magnetic resonance imaging), X-ray examination, X-ray imaging of the vessels using a contrast medium(angiography), PET-CT, or bronchoscopy of the lungs.
• Tissue sampling (biopsy): We take tissue samples from the suspicious areas of organs or vessels. Pathology then analyzes them under the microscope in the laboratory. The biopsy allows conclusions to be drawn about the form of vascular inflammation.

Vascular inflammation: prevention, early detection, prognosis

They cannot prevent vasculitis because it is often unknown why the immune system is directed against the body’s own structures. There is also no targeted early diagnosis for vasculitis. In fact, it usually takes a long time for us to detect the vascular inflammation due to the diverse, often general symptoms. It is always advisable to seek medical advice if symptoms persist or worsen over time.

Course and prognosis of vasculitis

The course and prognosis of vascular inflammation often vary greatly. Primary (independent) vasculitis cannot be cured, but its progression can often be slowed down and the inflammation controlled by early treatment. Then the forecast is also more favorable. In many cases, this makes normal everyday life possible.

However, the vasculitis can flare up again after a while. It is therefore important that you take the first warning signs seriously and seek medical advice promptly. Relapses can thus be detected and treated in good time. You should also stay in regular contact with a doctor who has a lot of experience with the multifaceted clinical picture of vascular inflammation.

Vascular inflammation: Treatment with medication

The treatment of vascular inflammation depends on the form and extent of the disease (e.g. the organs involved). Although it cannot be cured, it can be treated.

The most important pillar of therapy is medication that dampens the activity of the immune system – so-called immunosuppressants. Frequently used active substances are cortisone, methotrexate and azathioprine or mycophenolate mofetil or cyclophosphamide. The active substances Rituximab and Actemra – both artificially produced antibodies that influence the immune system – can also help with certain forms of vascular inflammation.