TYPES OF VASCULITIS

Are you looking for information on your type of vasculitis? On this page, the individual vasculitis diseases are covered in detail. See the list of individual diseases, download the information relating to your type of vasculitis as a PDF and read about the basics of other types of vasculitis.

ANTI-GBM (GOODPASTURE’S DISEASE)

Anti-glomerular basement membrane (GBM) disease, also known as Goodpasture’s disease, is a rare condition that causes inflammation of the small blood vessels in the kidneys and lungs. Learn more, download card

AORTITIS

Aortitis refers to inflammation of the aorta, which carries oxygenated blood from the heart to the rest of the body. It is representative of a cluster of large-vessel diseases that have various or unknown causes. Learn more download card

BECHET’S SYNDROME

Behcet’s Syndrome is a form of Vasculitis characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. Behcet’s affects blood vessels of all sizes and types, and can potentially involve any organ, including the brain and spinal cord. Oral ulceration is a major feature. Learn more, download card

BUERGER’S DISEASE

Buerger’s Disease is vasculitis causing insufficient blood flow to the hands and feet, producing numbness, tingling and ultimately skin ulcers and gangrene. The classic Buerger’s patient is a male, smoker, (it is associated with exposure to nicotine) between the ages of 20 and 40, although there is a higher percentage of women and people over the age of 50 being diagnosed. Buerger’s disease is most common in Southeast Asia, India and the Middle East, but appears to be rare among African– Americans. Learn more, download card

CENTRAL NERVOUS SYSTEM VASCULITIS

Central nervous system (CNS) vasculitis is inflammation of blood vessel walls in the brain or spine. CNS vasculitis often occurs in the following situations:

Accompanied by other autoimmune diseases such as systemic lupus erythematosus, and, rarely, rheumatoid arthritis Infection, such as viral or bacterial Systemic (affecting the whole body) vasculitic disorders (granulomatosis with polyangiitis (GPA), microscopic polyangiitis, Behçet’s syndrome);
Infection, such as viral or bacterial
Systemic (affecting the whole body) vasculitic disorders (granulomatosis with polyangiitis (GPA), microscopic polyangiitis, Behçet’s syndrome);

It can also occur without any associated systemic disorder. In this case, the vasculitis is only confined to the brain or the spinal cord and it is referred to as primary angiitis of the CNS (PACNS). Learn more, download card

COGAN SYNDROME

Cogan syndrome is a rare disorder characterized by recurrent inflammation of the inner ears and eyes. It can lead to vision difficulties, hearing loss and dizziness. Cogan Syndrome primarily affest the large vessels. It affects othere organs such as the heart, nervous system and bowels. Learn more, download card

CRYOGLOBULINEMIC VASCULITIS

Cryoglobulins are abnormal proteins in the blood. If you have cryoglobulinemia (kry-o-glob-u-lih-NEE-me-uh), these proteins may clump together at temperatures below 98.6 F (37 C). These gelatinous protein clumps can impede your blood circulation, which can damage your skin, joints, nerves and organs — particularly your kidneys and liver. Learn more, download card

EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA)

Eosinophilic Granulomatosis and Polyangiitis (EGPA), previously called Churg-Strauss syndrome, is a disorder characterized by allergic rhinitis (inflammation of the nose), asthma, and an increase in certain blood cells known as eosinophils, which infiltrate and inflame different organ systems. The organs most commonly involved are the lungs, peripheral nerves, sinuses, and the skin, although any organ system can be affected including the cardiovascular (heart), gastrointestinal (stomach), kidneys and the central nervous system

Asthma is the most common sign of EGPA. The disorder can also cause a variety of other problems, such as hay fever, rash, gastrointestinal bleeding, and pain and numbness in your hands and feet. Skin involvement with rashes and nodules are also common. Manifestations of cardiac involvement in EGPA includes pericarditis, a condition characterized by inflammation of the external part of the heart, heart failure and myocardial infarction (heart attack).. Learn more, download card

GIANT CELL ARTERITIS (TEMPORAL ARTERITIS)

Giant Cell (temporal) Arteritis (GCA) is a vasculitis of large vessels. It may be generalized but vessel inflammation most frequently involves vessels in the scalp and head, especially the arteries over the temples. The disease is called temporal arteritis because the temporal arteries, along the sides of the head just in front of the ears (to the temples) often become inflamed. Women, Caucasians, and individuals over 50 years of age are most commonly affected by GCA.

GRANULOMATOSIS WITH POLYANGIITIS (GPA) (Formerly Wegener’s)

Granulomatosis with polyangiitis is an uncommon disorder that causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys. It was formerly called Wegener’s granulomatosis. It slows blood flow to some of your organs. The affected tissues can develop areas of inflammation called granulomas, which can affect how these organs work. Learn more, download card

HENOCH-SCHÖNLEIN PURPURA (IGA VASCULITIS)

Henoch-Schönlein purpura (HSP) is a systemic vasculitis that causes the blood vessels in the skin to become inflamed, causing red spots. When the blood vessels in the skin get inflamed, they can bleed, causing a rash that is called purpura. This rash is typically seen on the lower legs or arms. The specific skin lesion is characterized by the tissue deposition of an immune system product, called IgA immunoglobulin, which is also found in kidneys of patients with a renal disease, called IgA nephropathy. Learn more, download card

HYPERSENSITIVITY VASCULITIS

Hypersensitivity vasculitis (HV) is often used to describe different types of vasculitis related to drug reactions, skin disorders or allergic vasculitis; however this is not always the correct use of the term.

Given the wide range of symptoms, the varying definitions and frequent incorrect use of the term, the American College of Rheumatology made a list of criteria for the classification of HV. Three or more of these criteria are needed to determine that a patient with some form of vasculitis is defined as specifically having HV. The criteria are:

older than 16 years of age
use of a drug before the development of symptoms
skin rash
biopsy of the skin rash that shows neutrophils, a type of white blood cells, around a small vessel. Learn more, download card

KAWASAKI DISEASE

Kawasaki disease causes inflammation in the walls of medium-sized arteries throughout the body. It primarily affects children. The disorder affects the mucus membranes, lymph nodes, walls of the blood vessels, and the heart. The most important aspect of the disease is the heart’s involvement. The disease can cause inflammation of blood vessels in the coronary arteries, which can lead to aneurysms. Kawasaki disease is the leading cause of acquired heart disease in children. Learn more, download card

MICROSCOPIC POLYANGIITIS

Microscopic polyangiitis (MPA) is an uncommon disease. It is the result of blood vessel inflammation (vasculitis), which can damage organ systems. The areas most commonly affected by MPA include the kidneys, lung, nerves, skin, and joints. Over 90% of those with MPA have the disease in their kidney and it is indistinguishable from the kidney disease that patients with classic Granulomatosis with polyangiitis (GPA) (formerly Wegener’s granulomatosis) often have. The main difference between MPA and GPA is that MPA does not have a particular type of inflammation–granulomatous inflammation. Learn more, download card

POLYARTERITIS NODOSA

Polyarteritis nodosa (PAN) is a rare disease that results from blood vessel inflammation (“vasculitis”) causing injury to organ systems. The areas most commonly affected by PAN include the nerves, intestinal tract, heart, and joints. PAN can also affect the blood vessels to the kidney resulting in high blood pressure and reduced kidney function. Learn more, download card

POLYMYALGIA RHEUMATICA

Polymyalgia rheumatica (PMR) is a condition that is frequently linked to giant cell arteritis (GCA) (link). PMR occurs in about 50 % of patients who have GCA, while approximately 15% of patients with PMR develop GCA. There may be a common genetic component between the two disorders. PMR is almost exclusively a disease that affects older adults and is rarely diagnosed in people under the age of 50 years. Learn more, download card

RHEUMATOID VASCULITIS

Rheumatoid vasculitis (RV) refers to patients with rheumatoid arthritis, a chronic disease with painful inflammation of the joints, who also develop inflammatory disease in small and medium-sized blood vessels. RV most commonly occurs in the skin as venulitis or capillaritis, meaning the smallest blood vessels are affected by inflammation from the disease. RV occurs in approximately 2 to 5 % of patients who have active rheumatoid arthritis. Learn more, download card

TAKAYASU’S ARTERITIS

Takayasu’s arteritis (tah-kah-YAH-sooz ahr-tuh-RIE-tis) is a rare type of vasculitis, a group of disorders that cause blood vessel inflammation. In Takayasu’s arteritis, the inflammation damages the aorta — the large artery that carries blood from your heart to the rest of your body — and its main branches. The disease can lead to narrowed or blocked arteries, or to weakened artery walls that may bulge and tear (aneurysm). Learn more, download card

URTICARIAL VASCULITIS

Urticarial Vasculitis is a form of cutaneous vasculitis characterised by inflammation of the small blood vessels. It can be classified into three subtypes. All are defined by a measure of the “complement” levels in the blood. The complement system is a set of proteins that contribute to and amplify immune responses. They play a role in some, but not all, autoimmune disorders including some forms of Urticarial Vasculitis. learn more, download card