Clinical Immunology focuses on the immune system’s health. Learn about the diagnosis and treatment of allergies, autoimmune diseases, and immunodeficiencies.

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Vasculitis: Overview and Definition

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Vasculitis refers to a group of rare conditions where blood vessels become inflamed because the immune system attacks them by mistake. This swelling can thicken the vessel walls and narrow the passage, which may limit blood flow and harm organs or tissues. Vasculitis can affect arteries, veins, or capillaries, and it may last for a short time or become a long-term issue.

Sometimes, vasculitis only affects the skin, but it can also cause serious problems in organs like the lungs, kidneys, or brain. Doctors classify vasculitis by the size of the blood vessels involved, from large to small. Knowing the exact type is important because treatment and outlook can be very different.

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The Mechanism of Vascular Inflammation

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Pathophysiology

Vasculitis happens when immune cells, mainly white blood cells, enter the vessel wall and damage its structure. This can start when immune complexes build up or when T-cells attack the lining of the vessels. As inflammation develops, the vessel may narrow and reduce blood flow, or it may weaken and form a bulge called an aneurysm, which can burst and cause dangerous bleeding. In small vessels, inflammation can make them break, leading to bleeding under the skin that often appears as purple spots.

The Role of Autoimmunity

Vasculitis is mainly an autoimmune disease. Normally, the immune system protects the body from germs like bacteria and viruses. In vasculitis, it cannot tell the difference between foreign invaders and the body’s own cells. Certain antibodies, such as ANCA, are important in some types of vasculitis. These antibodies attach to neutrophils, a kind of white blood cell, and make them release substances that harm blood vessel walls. This reaction can be set off by infections, medicines, or other immune problems, but often the exact cause is unknown.

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Classification by Vessel Size

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The most widely accepted classification system for vasculitides is the Chapel Hill Consensus Conference classification, which categorizes the diseases based on the predominant vessel size involved.

Large Vessel Vasculitis

This category primarily affects the aorta and its major branches.

  • Giant Cell Arteritis: This is the most common form of systemic vasculitis in adults over the age of 50. It typically affects the temporal arteries in the head but can involve the aorta. If untreated, it can lead to blindness and stroke.
  • Takayasu Arteritis: This rare form affects young women and involves the aorta and its main branches. It causes narrowing of the arteries, often leading to the loss of a pulse in the arms or legs, which is why it is sometimes called pulseless disease.

Medium Vessel Vasculitis

This group affects medium-sized arteries and is often associated with aneurysms and tissue death.

  • Polyarteritis Nodosa: This condition causes inflammation of medium-sized arteries, particularly those supplying the kidneys, gut, and nerves. It spares the lungs but can cause severe hypertension and abdominal pain.
  • Kawasaki Disease: Primarily affecting young children, this condition involves inflammation of the coronary arteries, which supply blood to the heart. It is a leading cause of acquired heart disease in children.

Small Vessel Vasculitis

This category affects the smallest blood vessels, including arterioles, capillaries, and venules.

  • Granulomatosis with Polyangiitis: Formerly known as Wegener’s granulomatosis, this affects the nose, lungs, and kidneys. It is strongly associated with ANCA antibodies.
  • Eosinophilic Granulomatosis with Polyangiitis: Formerly Churg-Strauss syndrome, this is associated with asthma and high levels of eosinophils in the blood.
  • Microscopic Polyangiitis: This affects the kidneys and lungs and causes inflammation of small vessels without the formation of granulomas.
  • IgA Vasculitis: Also known as Henoch Schonlein purpura, this is common in children and affects the skin, joints, intestines, and kidneys.

ANCA Associated Vasculitides

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A significant subset of small vessel vasculitides is termed ANCA-associated vasculitis.

  • Mechanism: These diseases are characterized by the presence of antibodies that target the cytoplasm of neutrophils.
  • Target Antigens: The two main targets are proteinase three and myeloperoxidase.
  • Clinical Relevance: The presence and type of ANCA are critical for diagnosis and monitoring disease activity. These conditions, which include GPA, MPA, and EGPA, often involve the kidneys and lungs and can be rapidly progressive if not treated aggressively.

Variable Vessel Vasculitis

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Some forms of vasculitides do not fit neatly into the size-based classification because they can affect vessels of any size.

  • Behcet’s Disease: This is a chronic condition that causes inflammation in blood vessels throughout the body. It is characterized by recurrent oral and genital ulcers and eye inflammation. It is more common in populations along the historic Silk Road.
  • Cogan’s Syndrome: This rare disorder is characterized by inflammation of the eyes and ears, leading to vision and hearing loss, and can also involve inflammation of the aorta.

Secondary Vasculitis

Vasculitis can also occur as a secondary condition to other diseases or exposures.

  • Infection-induced: Hepatitis B and C viruses are known triggers for certain types of vasculitides, such as polyarteritis nodosa and cryoglobulinemic vasculitis.
  • Drug-induced: Certain medications, including antibiotics like penicillin and sulfonamides, as well as some thyroid medications, can trigger an allergic type of vasculitis.
  • Connective Tissue Diseases: Vasculitis can appear as a complication of other autoimmune diseases like Rheumatoid Arthritis or Systemic Lupus Erythematosus.

Epidemiology and Demographics

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The distribution of vasculitides varies significantly by age, gender, and geography.

  • Age Factors: Kawasaki disease and IgA vasculitis are predominantly pediatric conditions. In contrast, Giant Cell Arteritis is almost exclusively a disease of the elderly.
  • Gender Differences: Takayasu arteritis and Giant Cell Arteritis are more common in women, whereas Polyarteritis Nodosa and Microscopic Polyangiitis affect men and women relatively equally.
  • Geographic Variance: Behcet’s disease is highly prevalent in Turkey and the Middle East, while Giant Cell Arteritis is most common in Northern European populations.

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FREQUENTLY ASKED QUESTIONS

What is vasculitis?

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