Vasculitis: when the blood vessels are inflamed

Vasculitis is a rare but serious disease characterized by inflammation of the blood vessels. This inflammation can affect the blood supply to various organs and tissues and lead to a variety of symptoms. Overall, vasculitis is a complex and multifaceted disease that requires a multidisciplinary approach, with different medical disciplines working together to provide comprehensive care and treatment. In this article you will learn more about the different forms of vasculitis, their symptoms, causes, diagnosis and treatment options.

Content of this article

• What is vasculitis?
• Classification of vasculitis
• Symptoms of vasculitis
• Causes of vasculitis
• Diagnosis of vasculitis
• Therapy of vasculitis
• Forecast

What is vasculitis?

Vasculitis occurs when the immune system mistakenly attacks and inflames healthy blood vessels. This inflammation can lead to thickening, narrowing or even blockage of the blood vessels, which impairs normal blood circulation. There are different types of vasculitis, depending on the blood vessels and organs affected.

Classification of vasculitis according to the Chapel Hill Consensus Conference

Vasculitis of the small vessels

1. ANCA-associated vasculitis
   1. Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis)
   2. Microscopic polyangiitis (MPA)
   3. Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome)
2. Immune complex vasculitis
   1. IgA vasculitis (Henoch-Schönlein purpura)
   2. Anti-GBM disease
   3. Cryoglobulinemic vasculitis: This form of vasculitis often affects small blood vessels and is associated with abnormal proteins in the blood called cryoglobulins. Symptoms can include skin rashes, joint pain, abdominal pain and kidney problems.
3. Vasculitis associated with systemic diseases of the connective tissue, for example:
   1. Rheumatoid Vasculitis
   2. Dermatomyositis

Vasculitis of the large vessels

This form of vasculitis often affects the large arteries, particularly the temporal arteries in the head and aorta. Symptoms include headaches, visual disturbances, jaw pain and fatigue.

1. Large vessel vasculitis
   1. Giant cell arteritis (temporal arteritis)
   2. Takayasu arteritis

Vasculitis of the medium-sized vessels

• Polyarteritis nodosa
• Kawasaki syndrome

Vasculitides of variable vessel size

• Behcet's disease (BC)
• Cogan syndrome (CS)

Single organ vasculitis

• Cutaneous leukocytoclastic vasculitis
• Primary CNS vasculitis

Vasculitides with associated systemic diseases

• Vasculitis in malignancies
• Vasculitis in gastrointestinal diseases
• Vasculitis in other autoimmune or systemic diseases

 

Symptoms of vasculitis

The symptoms of vasculitis can vary depending on the type of blood vessels and organs affected. The most common symptoms include

• Fever
• Tiredness
• Weight loss
• Joint and muscle pain
• Skin rashes
• Numbness or tingling in the extremities
• Shortness of breath
• Abdominal pain or bleeding
• Visual disturbances
• Cough or breathing difficulties
• The "red eye" (usually in the form of dermatitis)
• Ear, nose and throat complaints (persistent nasal congestion, bloody cold with crusts, sudden loss of hearing)
• Severe temporal headache with swelling of the temporal artery and visual disturbances

 

Causes of vasculitis

The exact cause of vasculitis is often unknown. It is thought that a combination of genetic, environmental and immune factors contribute to the development of the disease. Infections, certain medications and autoimmune diseases can also increase the risk of vasculitis.

Diagnosis of vasculitis

The diagnosis of vasculitis can be challenging as symptoms can vary and many other conditions can have similar signs. Diagnosis is usually based on a combination of clinical examinations, laboratory tests, imaging techniques and, if necessary, a tissue biopsy. Important steps in the diagnosis include:

1. Medical history and clinical examination: The doctor will take a detailed medical history and examine the patient thoroughly to identify symptoms of vasculitis or signs of underlying disease.
2. Laboratory tests: Various laboratory values can be helpful in the diagnosis of vasculitis. These include:
   1. Inflammatory markers such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), which can indicate increased inflammatory activity in the body.
   2. Complete blood count (including differential blood count) to identify signs of anemia, leukocytosis (increased white blood cell count) or thrombocytosis (increased platelet count).
   3. Autoantibody tests: Certain antibodies, such as ANCA (antineutrophil cytoplasmic antibodies), can be elevated in certain forms of vasculitis such as granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis.
   4. Electrolyte levels and renal function tests: To assess renal involvement, which can occur in some forms of vasculitis.
   5. Further specific tests depending on suspected organ involvement, e.g. liver function tests, thyroid values or specific antibody tests.
3. Imaging procedures: Imaging techniques such as X-ray, ultrasound, CT (computed tomography) and MRI (magnetic resonance imaging) can be used to identify abnormalities in the blood vessels or affected organs and to assess the severity of the disease.
4. Tissue biopsy (histology): In some cases, a tissue biopsy may be necessary to make a definitive diagnosis. This can be done through a skin biopsy, kidney biopsy, muscle biopsy or biopsy of other affected organs. Analyzing the tissue under a microscope can help identify signs of vasculitis and rule out other possible causes for the symptoms.

The diagnosis of vasculitis usually requires a thorough examination by an experienced physician or rheumatologist and a careful evaluation of all available clinical information and test results. Early diagnosis and treatment are critical to minimize damage to the affected organs and improve prognosis.

Therapy of vasculitis

The treatment of vasculitis aims to control the inflammation, prevent damage to the affected organs and alleviate symptoms. Therapy depends on the type and severity of the vasculitis as well as individual factors such as the patient's age and general health. Common treatment methods to induce remission include:

 

1. Glucocorticoids (corticosteroids): These drugs are often used as the first line of treatment for vasculitis to rapidly suppress inflammation. They are usually prescribed in higher doses to quickly control the inflammatory response and then gradually reduced to minimize side effects.
2. Immunomodulators: Drugs such as methotrexate, azathioprine and cyclophosphamide can be used together with glucocorticoids to treat vasculitis. They work by suppressing the immune system and controlling inflammation.
3. Biologics: Biologics are drugs that specifically target certain components of the immune system. In some forms of vasculitis, such as giant cell arteritis and granulomatosis with polyangiitis, biologics such as tocilizumab and rituximab can be effective.
4. Plasmapheresis: This procedure is used in the most severe cases of organ involvement to remove harmful antibodies from the blood, especially when other treatment methods are not sufficiently effective.
5. Long-term therapy (remission-maintaining therapy): As vasculitis is often a chronic disease, long-term drug therapy may be required to prevent relapses and maintain remission.
6. Newer treatment options: In recent years, new therapies have been developed for certain forms of vasculitis, particularly ANCA-associated vasculitides such as granulomatosis with polyangiitis and microscopic polyangiitis. These therapies include drugs such as rituximab and avacopan, which can target the immune system and control inflammation.

It is important that the treatment of vasculitis is individualized for each patient and is carried out under the supervision of an experienced rheumatologist. Regular medical examinations and laboratory tests are often necessary to monitor the course of the disease and adjust treatment. 

Forecast

The prognosis for people with vasculitis depends on several factors, including the type and severity of the disease, the response to treatment, the presence of complications and adherence to therapy and follow-up. Early diagnosis and appropriate treatment can improve quality of life and reduce the risk of long-term complications.