Types of Vasculitis

Doctors categorize the different types of vasculitis based on the size of blood vessels they impact – large, medium, and small.

Large Arteries

Giant cell arteritis (GCA): One of the more common forms of large vessel vasculitis occurring in adults older than age 50. It can present with headaches and/or jaw pain, sometimes with flu-like symptoms, fatigue and fever. GCA can impact blood flow to the eye, resulting in visual disturbance or loss. GCA can co-occur with polymyalgia rheumatica (PMR) which causes stiffness and pain in the shoulders and hips. Aortitis, or inflammation of the aorta and its large branches, is common in GCA.

Takayasu’s arteritis (TAK): An inflammation of the walls of the aorta and its main branches, TAK occurs predominantly in younger women. Reduced blood flow to arms, legs and major organs may cause symptoms of  arm or leg pain, elevated blood pressure, discoloration in hands or feet, fatigue, headache, vision problems, chest pain, heart attack or stroke. TAK symptoms may develop slowly over time, making it difficult to diagnose.

Aortitis: Inflammation of the aorta which can have no identifiable underlying cause (“isolated aortitis”) or can occur secondary to another autoimmune disease (such as Cogan’s syndrome, Behcet’s syndrome or spondyloarthropathy). Aortitis can cause fever, weight loss, or pain in the chest, limbs, or abdomen, or can be asymptomatic. If untreated, it can lead to thinning and an outpouching of the aortic wall called an aneurysm.

Medium Arteries

Kawasaki disease (KD): Inflammation to the blood vessels that supply the heart occurring in childhood, usually under the age of 5. KD can cause fever, rash, red eyes, cracked lips and tongue, and swollen lymph nodes. Symptoms of KD might resolve on their own, but if not diagnosed and treated, KD can damage the heart. Pediatric rheumatologists diagnose and treat KD.

Polyarteritis nodosa (PAN): Inflammation to the blood vessels in the skin, nervous system, kidneys, gastrointestinal tract, heart, and other organs, more commonly in middle aged men. Because it can involve multiple systems in the body, it can present with a wide range of symptoms including fatigue, numbness, high blood pressure, abdominal pain, leg pain and skin sores.

Small Arteries

Granulomatosis with polyangiitis (GPA): Formally known as Wegener’s Granulomatosis, is an inflammation to the blood small vessels of the lungs, kidneys, upper airway, skin, nerves and sinuses. Symptoms can also be caused by small areas of inflammation called granulomas in the sinuses, lungs, ear canals and orbits of the eyes. Depending on which system is involved it can present with chronic nasal congestion, shortness of breath, cough and nosebleeds, joint pain, eye pain and redness, trouble hearing, skin rashes, fatigue, fever, weight loss, weakness and numbness. 

Microscopic polyangiitis (MPA): Inflammation of small blood vessels in most commonly in the kidneys, lungs and nerves but also can involve the skin and joints. The most common symptoms are blood in the urine and high blood pressure from kidney inflammation, and shortness of breath  and coughing up blood from lung inflammation. Weight loss, fever, skin lesions and nerve damage can also occur.

Eosinophilic granulomatosis with polyangiitis (EGPA), formally known as Churg-Strauss syndrome: Nearly all EGPA patients have asthma and/or sinus polyps, but additional symptoms depend on which body parts are involved and can include the lungs, sinuses, nerves, heart, skin, and kidneys.Patients with EGPA have elevated levels of eosinophils, a kind of white blood cell, which drive the inflammation.

GPA, MPA and EGPA arise from inflammation caused by antineutrophil cytoplasmic antibodies (ANCA). ANCAs are antibodies that damage blood vessels by attacking white blood cells called neutrophils.

Primary angiitis of the central nervous system (PACNS): This form of vasculitis is limited to the blood vessels in the brain and usually occurs in adults after age 50, and is twice as likely to occur in men. The most common symptoms are headache, brain dysfunction (encephalopathy) and/or recurrent strokes. 

IgA vasculitis (Henoch-Schonlein Purpura - HSP): Usually diagnosed in children as a self-limited illness, however can also impact adults in a more serious form of the disease, The most common symptom of HSP is purpura, a red or dark purple rash usually seen on the legs and buttocks. HSP may also cause joint swelling, abdominal pain, and kidney inflammation. While children usually make a full recovery, some cases of HSP (particularly in adults) can result in kidney disease, making early detection and treatment important. 

Systemic lupus erythematosus (SLE or lupus) or Sjögren’s syndrome (small Vessel Vasculitis related to rheumatoid arthritis): Inflammation of small arteries can occur in conjunction with one of these three autoimmune conditions.

• Cryoglobulinemic vasculitis: Can be  associated with the hepatitis C virus (HCV), Sjogren’s, lupus, blood cancer, or idiopathic (no cause identified). Symptoms include purplish patches of skin (purpura), nerve damage, weakness, joint pain, and kidney problems.

• Anti-GBM disease / Goodpasture’s syndrome): Can cause inflammation of the the kidneys and/or lungs.The inflammation causes the organs to bleed, which can eventually lead to organ failure, making early diagnosis and treatment very important. Symptoms of advanced disease include bloody cough, bloody urine, chest pain and leg swelling.

• Drug-induced vasculitis: Vasculitis has been associated with use of a variety of medications, including (and not limited to) various antibiotics, hydralazine, minocycline, levamisole (a contaminant found in cocaine), and anti-thyroid drugs, to name a few. Symptoms frequently include rash but can include serious organ damage including the kidneys.

Blood Vessels of Various Sizes

Behcet’s disease/ Behcet’s syndrome: This chronic form of vasculitis, which is seen most commonly in people of Middle Eastern or East Asian descent, can cause sores in the mouth, skin and genitals, joint and muscle pain, headache, eye inflammation, and blood clots.

Relapsing polychondritis (RP): Results from inflammation of cartilage, typically causing pain and swelling to the ear, nose, upper airway and joints. Symptoms usually begin between ages 20 and 60, but children may be diagnosed with RP as well.

Causes and Risk Factors

Researchers continue to investigate what causes most forms of vasculitis. It is classified as an autoimmune disease, because the body’s immune system attacks blood vessels. While genetics seem to play a role in vasculitis, in most cases scientists have yet to isolate the specific gene or genes that drive the development of the disease.

Some risk factors have been identified for a few types of vasculitis. Reactions to some medications, past infections with certain bacteria or viruses (hepatitis B or hepatitis C virus), exposure to environmental  factors and smoking have all been linked to one or more types of vasculitis.

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Online resources from the following organizations were cited for information on vasculitis types, symptoms, risk factors, diagnosis and treatment: