What is vascular inflammation?

Inflammation is your body's response to irritation or injury. The signs of inflammation are pain, redness, heat, swelling, and loss of function. During the inflammatory process, an injured area becomes reddened and warm as blood flow increases to heal the affected tissue. This increased blood flow allows white blood cells to travel more quickly through the body. Vasculitis means inflammation of blood vessels. 

When your body's immune system attacks blood vessels in any part of your body, you have vasculitis. More than 1 million Americans have vasculitis at some point in their lives. Some types are more common than others. Some types may affect only one organ or part of the body (localized) while other types can affect many organs (multifocal).


vascular inflammation
vascular inflammation


The inflammation can be mild, with a slow onset and recovery, or might come on more quickly and severely. 

The most common form of vasculitis in the United States is Wegener's granulomatosis, which is a rare autoimmune disorder that affects the lungs. It becomes evident in 30% to 40% of people with systemic lupus erythematosus (SLE). 


Statistics

Here are some important statistics about vasculitis: 

  •  In 2010 there were an estimated 516,000 cases in the U.S., with women affected twice as often as men. 

  • Vasculitis occurs at all ages but mostly affects young adults between 15 and 35 years of age. 

  • The yearly incidence of all types of vasculitis in the United States is approximately 23 cases per 100,000 people.
  • Approximately 4% of new rheumatology clinic patients have vasculitis (120,000 new cases yearly). 

  • Vasculitis is responsible for 5% to 10% of lupus flares; however, this number may be higher due to a lack of awareness among clinicians about how to recognize and manage vascular inflammation. 

  • There are seven categories of systemic vasculitides: large vessel (polyarteritis nodosa), medium vessel (Giant cell arteritis), small vessel (granulomatosis with polyangiitis, microscopic polyangiitis), cutaneous vasculitis, connective tissue disease-associated vasculitis, hypersensitivity vasculitis (also known as Drug Reaction with Eosinophilia and Systemic Symptoms [DRESS]), and cryoglobulinemic vasculitis.

  • The most serious types of systemic vasculitides are the small vessel (granulomatosis with polyangiitis, microscopic polyangiitis), giant cell arteritis, lymphomatoid granulomatosis (a form of angiotropic large cell lymphoma), Churg-Strauss syndrome (allergic granulomatosis), Takayasu's arteritis (aortic arch syndrome) and Wegener's granulomatosis.

  • Vasculitides can affect any size of blood vessels and any organ in the body. 


Types of vasculitis 

There are several different types of vasculitis, including: 

  • Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis: This is a systemic necrotizing vasculitis that affects the respiratory tract, kidneys, and other organs. It is also associated with an underlying condition such as rheumatoid arthritis or lupus. It accounts for 10% of all cases of systemic vasculitis. 

  • Microscopic polyangiitis: Also known as microscopic polyarteritis, this form most commonly occurs in middle-aged men and can affect blood vessels throughout the body. However, it most often strikes the kidneys and lungs. The damage caused by microscopic polyangiitis can be permanent and leads to scarring of blood vessels known as crescentic glomerulonephritis, a common cause of kidney failure.

  • Churg-Strauss syndrome (CSS): This disorder is characterized by asthma or sinus problems, eosinophilia, and vasculitis caused by the release of chemicals from white blood cells called mast cells. It can also affect other parts of the body such as the intestines, nervous system, or skin. 

  • Wegener's granulomatosis: A rare autoimmune disorder that affects the lungs. It becomes evident in 30% to 40% of people with systemic lupus erythematosus (SLE). One study found that in people with SLE, Wegener's granulomatosis was the most likely vasculitis to cause death.

  • Cryoglobulinemic vasculitis: Blood vessels are directly affected by deposits of immune complexes containing antibodies and antigens. It can lead to skin lesions or kidney problems. 

  • Takayasu arteritis: Also known as transient ischemic attack syndrome, this is an inflammatory disorder affecting arteries servicing the brain, heart, and arms. Inflammation in these large blood vessels decreases blood flow to the organs they supply. Symptoms include numbness or weakness in limbs, blurred vision, headaches, and chest pain when breathing deeply. 

  • Giant cell arteritis (GCA): This condition affects medium-sized arteries, including those supplying blood to the head and neck. Headache is a common symptom for many patients, as well as eye pain and jaw pain when chewing. If untreated, GCA can cause blindness. 


What causes vascular inflammation? 

Vasculitis can be caused by infection or it may be an autoimmune disorder. 

  • Infection: Many people with vasculitis have what is called ANCA (anti-neutrophil cytoplasmic antibodies). This means that their immune system mistakes parts of its own body as foreign, attacking them and causing inflammation in various organs or parts of organs. However, in vasculitis, we often do not know exactly why the immune system begins to attack cells in a particular area. We think that certain bacteria and viruses might trigger this reaction in some individuals, but it's not known for sure. 

  • Autoimmune: In many other cases, we think that the person's own immune system causes inflammation in blood vessels and organs by mistake, as if it were mistaking cells and tissues as foreign and attacking them. This is called an autoimmune disorder. We also do not know why the immune system goes after parts of its own body in this way. 

  • Vasculitis can be caused by infection or can be autoimmune (in which case it would fall into one of 7 categories). 

The following are potential causes of vasculitis: 

  • Drugs/toxins (could be a side effect of medication or drug abuse)

  • Infection (bacterial, fungal, parasitic infections; also see angiostrongyliasis)

  • Graft vs. Host Disease (GVHD) after stem cell transplants

  • Cryoglobulinemia (mechanism unknown, but may occur secondary to viral infection,
  •  autoimmune diseases such as SLE and mixed connective tissue disease, 
  • lymphoproliferative disorders such as Waldenstrom's macroglobulinemia and MGUS [monoclonal gammopathy of undetermined significance], 
  • neoplastic conditions such as cancer - especially B-cell lymphomas, 
  • B cell chronic lymphocytic leukemia, 
  • multiple myeloma and plasmacytoma - and autoimmune diseases such as Sjögren's syndrome) 

  • Neoplasms (grade 1: benign blood vessel tumor; grade 2: malignant blood vessel tumor); other neoplasms include Trousseau’s syndrome and portal hypertension due to neoplasia (tumor of the liver or pancreas), hemangiomas)


Symptoms of vasculitis 

 can be nonspecific, but they may include: 

  • Fever/chills

  • Fatigue and weakness

  • Skin rashes can appear in a variety of colors depending on the type. 
  • Nodular vasculitis will present with purplish-red lesions that are flat or slightly raised and have a central depression


What is Cutaneous Vasculitis?

Vasculitis can also affect the skin. In some instances, it is referred to as cutaneous vasculitis or angio cutaneous syndrome. It usually affects small blood vessels in the skin which limits its ability to carry oxygen and nutrients. Symptoms may include inflammation of the skin subsequent redness, blisters, bruises, ulcers, small blood vessel hemorrhage, joint pain, and fever. Though the symptoms will depend on the type of vasculitis affecting the skin, they tend to include some or all of these symptoms. 

  • Photosensitivity
  • Intense itching in affected areas


Risk factors

Causative agents or risk factors are not always identified when patients are diagnosed with vasculitis; however, known causes include infections (bacterial, fungal, parasitic), environmental exposures (drugs/toxins, radiation), autoimmune diseases (especially systemic lupus erythematosus, rheumatoid arthritis, and Sjögren syndrome), hematologic disorders (polycythemia vera, essential thrombocytosis, myeloproliferative disorders such as chronic myelogenous leukemia or post-polycythemia vera phase), malignancies (especially B cell lymphomas) and connective tissue disease. 


How is it diagnosed? 

  • The diagnosis may be confirmed by biopsy of the affected tissue. Findings must rule out other inflammatory syndromes such as necrotizing vasculitis (polyarteritis nodosa), eosinophilic granulomatosis with polyangiitis, and Churg-Strauss syndrome.

  • Blood tests to detect ANCA elevated in nearly all types of vasculitis. 

  • A small number of patients with vasculitis may not have detectable levels of antibodies, and some individuals without vasculitis can have normal or even high ANCA titers. It is therefore important to perform a biopsy as well as laboratory testing for antineutrophil cytoplasmic antibodies (ANCA) to establish the correct diagnosis.


Treatment of vasculitis 

Treatments for vasculitis vary depending on the type of vasculitis. Treatments focus on decreasing inflammation, preventing organ damage, and preserving organ function. Treatment may include:

  • Corticosteroids (prednisone) to decrease inflammation.

  • In some cases, treatment with chemotherapy drugs is required. 

  • The reason why Corticosteroids are given as the first line of defense is that these drugs have been shown to be very effective at inhibiting the activation and chemotaxis of neutrophils which is one of the primary drivers of inflammation in Vasculitides. This inhibition results in decreased generation of oxygen free radicals which had been thought to play a role in tissue damage associated with Vasculitides.

  • Treatment goals are to minimize inflammation, reduce the risk of infection, and control pain or other symptoms. 

  • For patients with ANCA vasculitis, corticosteroids are typically initially given at high doses but should be slowly tapered down because their use is associated with many side effects. The dose can be decreased after several weeks if remission occurs. Resumption of treatment is often required if clinical relapses occur during tapering. Some people may need long-term treatment with immunosuppressive medications such as methotrexate or cyclophosphamide (an antineoplastic agent). 

  • Other treatment options for vasculitis include: - Plasma exchange to remove pathogenic antibodies from the blood (plasmapheresis). - Thrombolytic drugs such as alteplase and anistreplase dissolve blood clots.

  • Therapies aimed at blocking tumor necrosis factor (TNF) or other cytokines, such as infliximab and etanercept. 

  • Chemotherapy with either classic cytotoxic drugs (e.g., methotrexate) or newer biologic agents that target specific aspects of inflammation. 

  • The use of eculizumab in ANCA-associated vasculitis was shown to be useful in some cases, but this drug is not as effective as rituximab as a first-line treatment and is more commonly used as second-line therapy. 

  • People with Wegener's granulomatosis usually require plasma exchange and/or intravenous cyclophosphamide or methotrexate for remission. 

  • A small number of people with Churg-Strauss syndrome—typically those who have asthma, eosinophilia, and significant peripheral blood eosinophilia—may be treated successfully with corticosteroids alone. If not, then therapy with cyclophosphamide combined with corticosteroids may be required to achieve remission. More recently, mycophenolate mofetil (CellCept) has been used instead of cyclophosphamide in some cases. 

  • Tumor necrosis factor inhibitors (infliximab, etanercept) should be considered in the conclusion of first or second-line therapy. 

  • Most patients with microscopic polyangiitis are treated first with high doses of corticosteroids for one to three months. If remission is achieved, low doses of prednisone may be continued long term to control the disease and decrease the risk that it will flare up again at some time in the future. 

  • Methotrexate may also be used as an alternative to steroids if the person cannot take corticosteroids because of side effects or other health problems. Colchicine is sometimes helpful for controlling flares reducing acute symptoms during remissions. Vasculitis that does not respond to corticosteroids may be treated with other immunosuppressive medications. These include azathioprine, rituximab, cyclophosphamide, mycophenolate mofetil (CellCept), and methotrexate. 


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Is arthritis common in people with systemic vasculitides? 

Yes, 60% of people with large vessel vasculitides and 40%-50% of those with small vessel disease have joint symptoms including: 

  • Monoarticular arthritis 

  • Arthritis involving larger joints such as knees and elbows 

  • Joint swelling that comes and goes over time 

  • Affected joints may be hot or painful at times but usually not very red or warm 

  • Arthritis is more likely to occur with systemic vasculitides that are due to autoimmune causes rather than infections. 


Prevention of vasculitis. 

In general, vasculitis is difficult to prevent. 

People with an increased risk of certain types of vasculitis should take extra precautions when they are in contact with certain triggers or during periods when the disease flares. For example:

  • Some people with Wegener's granulomatosis may benefit from taking penicillin (an antibiotic) to suppress their immune systems and decrease the risk that the condition will flare up in response to streptococcal bacteria in the future.

  • People who have anti-neutrophil cytoplasmic antibodies (ANCA—which can cause small vessel vasculitides including Churg–Strauss syndrome and microscopic polyangiitis) should avoid taking nonsteroidal anti-inflammatory drugs (NSAIDs—which can act as ANCA inducers) and may wish to avoid other medications that could increase their risk of vasculitis. They should also avoid contact with certain triggers such as latex gloves, cigarette smoke, animal dander, and foods that contain nitrates (e.g., lunch meats, hotdogs).


Prognosis

The prognosis for patients with systemic vasculitides varies depending on the condition. About 1/3 of people recover completely, about 1/3 have permanent organ damage or require ongoing treatment to control disease activity, and about 1/3 die from the disease at some point. The prognosis is generally better for small vessel diseases than large vessel diseases.


How long does it take for systemic vasculitides to get better? 

For some people, remission is achieved within weeks after starting treatment. However, many people require multiple treatments with corticosteroids and/or other medications over several months or even years before the condition is fully controlled. If remission is not reached in one year, then long-term therapy to control recurring symptoms may be required.


What are the common side effects of systemic vasculitides? 

Side effects associated with systemic vasculitis include: 

  • Nausea 
  • Loss of appetite 
  • Weight loss 
  • Muscle aches and pains (myalgia) 
  • Headache 
  • Fever 
  • Fatigue (tiredness) 


How serious is vasculitis? 

Vasculitis can be a serious disease. Even corticosteroids, which are extremely effective at controlling the condition in most people, may cause side effects such as weakness or infections. The long-term prognosis depends on the type of vasculitides and how well you respond to treatment. 


Is vasculitis an autoimmune disease? 

Yes, vasculitis is an autoimmune disease that affects blood vessels. 

  • Vasculitides are believed to be caused by immune system cells attacking the endothelial cells of the vasculature. 

  • This leads to damage or destruction of blood vessels and may involve any organ or body part. 

  • The types of vasculitides are defined by the cell type involved (e.g., neutrophils, eosinophils, etc.). They are further defined by whether they cause inflammation in small or large blood vessels or affect specific organs/tissues. 

  • While some forms of the condition do have infectious triggers, most systemic vasculitides cannot be spread from person to person and can only rarely be linked to an infection. 

  • The condition is generally considered autoimmune because the immune system cells involved in the disease may escape from their normal control and attack healthy tissue (similar to what happens during inflammation of joints, kidneys, lungs, or intestines).

  • With systemic vasculitides, organ damage can occur if large amounts of blood vessels are affected. For example: 

  •  When large numbers of capillaries (small blood vessels) get damaged with Wegener's granulomatosis, this may cause extensive scarring within the nose and/or sinuses; lead to bleeding inside both air passages, and impair kidney function and circulation. 


Is vasculitis curable? 

Yes, with corticosteroids and/or other treatments most types of systemic vasculitis can be brought under control and symptoms may resolve. How long it takes for symptoms to get better varies from person to person and depending on the type of vasculitis. While many people experience only mild symptoms when the condition is first diagnosed, some people have more severe disease that requires treatment in a hospital or intensive care unit (ICU). 


What foods should I avoid with vasculitis? 

It is important to avoid foods that you are allergic to. You should also eat a healthy diet that includes lots of vegetables, fruits, and whole grains, as well as lean proteins like fish, chicken, or turkey (without the skin), beans, and lentils. 


Can you live a long life with vasculitis? 

Most people with vasculitis can lead active, fulfilling lives. The condition may require ongoing treatment with medications and sometimes multiple hospitalizations. 


What are systemic vasculitides? 

Systemic vasculitides are a group of rare disorders that cause inflammation in small or large blood vessels or affect specific organs/tissues. This can damage the affected organs/tissues and interfere with their normal function over time.  There are many types of systemic vasculitis conditions, which vary in severity and tend to have varying long-term prognoses. Systemic vasculitis is believed by most experts to be autoimmune in nature.


How do you reduce vascular inflammation?

 There are many medical therapies that can potentially reduce vascular inflammation. These include Corticosteroids, TNF-α blockers (e.g., etanercept), Methotrexate, Cyclosporine, and other medications. 


Is vasculitis dangerous? 

While vasculitis can be serious, it is rarely life-threatening for most people who develop the condition if it is diagnosed early enough and treated with medication(s). Most people with "classic" systemic vasculitides like polyarteritis nodosa or granulomatosis with polyangiitis (formerly known as Wegener's) may recover completely without residual damage to their organs/tissues. However, other types of systemic vasculitis, such as microscopic polyangiitis, do have a higher mortality rate.