Vasculitis: Types, Symptoms,Causes, Comlications, Diagnosis, Treatment and Living With Vasculitis


Vasculitis also known as angiitis and arteritis is a group of uncommon diseases which result in inflammation of the blood vessels.

April 5, 2018

Vasculitis also known as angiitis and arteritis is a group of uncommon diseases which result in inflammation of the blood vessels. It causes changes in the blood vessel walls, including thickening, weakening, narrowing or scarring which can restrict blood flow, resulting in organ and tissue damage.
Vasculitis are of many types and most of them are rare. It might affect just one organ, or several. So depending upon the organs affected and the severity of the disease, the symptoms of vasculitis vary greatly. The condition can be acute or chronic. A biopsy of involved tissue or angiography can confirm the diagnosis of vasculities. Decreasing the inflammation of the blood vessels and improving the function of affected organs is the goal of treatment. Depending on the type you have, you may not require any treatment or require medications to control the inflammation and prevent flare-ups.

Types of Vasculitis:

The vascular system of the body consist of blood vessels are comprised of arterie and veins. The function of arteries is to pass oxygen-rich blood to the tissues of the body and veins return oxygen-depleted blood from the tissues to the lungs for oxygen. The inflammation and damage to the walls of various blood vessels are known as vasculities.
When arteries are the inflamed blood vessels, the condition is also referred to as arteritis and when the veins are inflamed, it is referred to as venulitis. Although some types of vasculities are more common among certain groups, it can affect anyone. Depending on certain patterns of distribution of blood vessel involvement and particular organ involvement, vasculities can be catagorized in to

  • Behcet's disease
  • Buerger's disease
  • Churg-Strauss syndrome
  • Cryoglobulinemia
  • Giant cell arteritis
  • Granulomatosis with polyangiitis
  • Henoch-Schonlein purpura
  • Kawasaki disease
  • Takayasu's arteritis

As a group, these diseases are referred to as vasculitides.

Symptoms of Vasculitis:

The signs and symptoms of vasculitis are due to decreased blood flow throughout the body. These can vary greatly.
General signs and symptoms common to most types of vasculitis:

  • These may include:
  • Fever
  • Headache
  • Fatigue
  • Weight loss
  • General aches and pains
  • Rash
  • Night sweats
  • Nerve problems, such as numbness or weakness

Signs and symptoms for specific types of vasculitis:

The symptoms of a particular type of vasculities can develop early and rapidly or in later stages of the disease. These may include:

Behcet's disease:

This condition causes inflammation of your arteries and veins. Mouth and genital ulcers, eye inflammation, and acne-like skin lesions are the signs and symptoms of this disease.

Buerger's disease:

This condition causes inflammation and clots in the blood vessels of your hands and feet, resulting in pain and ulcers in these areas. This disease can also affect blood vessels in the abdomen, brain and heart which is very rare. It is also called thromboangiitis obliterans.

Churg-Strauss syndrome:

This is also known as Eosinophilic granulomatosis with polyangiitis. This condition is very rare. It mainly affects the lungs, skin, kidneys, heart and nerves in your limbs. Asthma, skin changes, nerve pain and nasal allergies are the signs and symptoms of this condition which can vary greatly.

Cryoglobulinemia:

Abnormal proteins in the blood are the cause of Cryoglobulinemia. Signs and symptoms include rash, joint pain, weakness, and numbness or tingling.

Giant cell arteritis:

An inflammation of the arteries in your head, especially at the temples is known as Giant cell arteritis. This condition can cause headaches, scalp tenderness, jaw pain, blurred or double vision, and even blindness. It is also called temporal arteritis.

Granulomatosis with polyangiitis:

Inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys is known as Granulomatosis with polyangiitis. Signs and symptoms include nasal stuffiness, sinus infections, nosebleeds and possibly coughing up blood. But most of the time symptoms are not noticeable until the damage is in advanced stage.

Henoch-Schonlein purpura:

This is also called as IgA vasculitis and is more common in children than in adults. It causes inflammation of the capillaries of your skin, joints, bowel and kidneys. Signs and symptoms include abdominal pain, blood in the urine, joint pain, and a rash on your buttocks or lower legs.

Hypersensitivity vasculitis:

This is also called as allergic vasculitis. The primary sign of this condition is red spots on your skin, usually on your lower legs. An infection or an adverse reaction to medicine can trigger the condition.

Kawasaki disease:

It is also called mucocutaneous lymph node syndrome and most often affects children younger than age 5. Signs and symptoms include fever, rash and redness of the eyes.

Microscopic polyangiitis:

Small blood vessels, usually those in the kidneys, lungs or nerves are mostly affected by this form of vasculitis. You may develop abdominal pain, rash, fever, muscle pain and weight loss. You may cough up blood if the lungs are affected.

Polyarteritis nodosa:

The kidneys, digestive tract, nerves and the skin are mostly affected by this form of vasculitis. Signs and symptoms include a rash, general malaise, weight loss, muscle and joint pain, abdominal pain after eating, high blood pressure, muscle pain, weakness, and kidney problems.

Takayasu's arteritis:

The larger arteries in the body, including the aorta are affected by this form of vasculitis. Signs and symptoms include joint pain, loss of pulse, high blood pressure, night sweats, fever, general malaise, appetite loss, headaches and visual changes.
If you experience any of these signs or symptoms, consult your doctor immediately as early diagnosis is key to get effective treatment. Some types of vasculitis can worsen quickly while others can develop very slowly.

Causes of Vasculitis:

The actual cause of these vasculitis diseases is unknown. However, immune system abnormality and inflammation of blood vessels are common cause in development of disease. When the immune system attacks blood vessel cells by mistake, vasculities occurs. Possible triggers for this immune system abnormalities include:

  • Infections such as hepatitis B or hepatitis C
  • Exposure to chemicals such as amphetamines and cocaine
  • Reactions to certain medications,
  • Cancers such as lymphomas and multiple myeloma
  • Immune system diseases, such as rheumatoid arthritis, lupus and scleroderma
  • Smoking

Blood vessels may bleed or become inflamed when gets affected by vasculitis. The layers of the blood vessel wall thicken as a result of inflammation. This narrows the blood vessels, reducing the amount of blood and therefore oxygen and vital nutrients to your body's tissues and organs.

Complications of Vasculitis:

The complications of vasculitis arises depending on the type and severity of your condition. They may be related to side effects of the prescription medications you use to treat the condition. Complications of vasculitis include:

Organ damage:

Some types of vasculitis can be severe, causing damage to major organs such as kidney,lungs and heart.

Blood clots and aneurysms:

A blood clot may form in a blood vessel, obstructing blood flow. Aneurysm is formed when a blood vessel weaken and bulge due to vasculitis.

Vision loss or blindness:

This is a possible complication of untreated giant cell arteritis.

Infections:

These include serious and life-threatening conditions, such as pneumonia and sepsis.

Diagnosis of Vasculitis:

After taking your medical history and performing a physical exam, your doctor may order one or more diagnostic tests and procedures to either rule out other conditions that mimic vasculitis or diagnose vasculitis.
Tests and procedures might include:

Blood tests:

Several blood test can be done to help diagnose vasculitis. A high level of C-reactive protein indicates inflammation in the body. A complete blood cell count can tell whether you have enough red blood cells. Blood tests done to determine the presence of certain antibodies, such as the anti-neutrophil cytoplasmic antibodies test can help diagnose vasculitis.

Urine tests:

These tests may reveal whether your urine contains red blood cells or has too much protein, which indicates the possibility of vasculities.

Imaging tests:

Imaging tests such as X-rays, ultrasound, computerized tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) can help determine what blood vessels and organs are affected. They are also used to monitor whether and how much you are responding to treatment.

X-rays of your blood vessels (angiography):

A flexible catheter is inserted into a large artery or vein in this procedure. A special dye is then injected into the catheter. When the dye fills the artery or vein, X-rays are taken. The outlines of your blood vessels will be visible on the resulting X-rays.

Biopsy:

This is a surgical procedure in which a small sample of tissue from the affected area of your body will be removed and then examination of this tissue will be done for signs of vasculitis.This can help demonstrate the pattern of blood vessel inflammation. Tissues used for biopsy include skin, sinuses, lung, nerve, and kidney.

Treatment of Vasculitis:

The treatment type for various forms of vasculitis depends on the severity of the illness and the organs involved. The goal of treatment is to stop or reduce the inflammation and suppress the immune system. Controlling the inflammation with medications and resolving any underlying disease that triggered your vasculitis is the first step in treating vasculities.
The treatment of vasculitis has two phases.  These include first stopping the inflammation and then preventing relapse which include maintenance therapy. Both phases require prescription medicines. It depends on the type of vasculitis, the organs involved and how serious your condition is to decide which drugs and how long you need to take them. Some people may have initial success with treatment, but then experience flare-ups later. In some cases there is no cure to vasculitis completely and require ongoing treatment.

Medications:

Corticosteroid medicines, such as prednisone or methylprednisolone are usually recommended to help control inflammation. Side effects of corticosteroids can be severe, especially if you take them for a prolonged time period. Possible side effects include weight gain, diabetes and osteoporosis. The possible lowest dose will be prescribed, if a corticosteroid is needed for long-term  or maintenance therapy.
Some steroid-sparing medications such as methotrexate, azathioprine, mycophenolate or cyclophosphamide can be used along with corticosteroids to control the inflammation so that the dosage of corticosteroids can be tapered more quickly.
The specific medication that will be presribe for you will depend up on the type and severity of vasculitis you have, which organs are involved, and your overall health. Biologic therapies such as rituximab or tocilizumab may be recommended, depending on the type of vasculitis you have.

Surgery:

Sometimes, vasculitis causes a balloon like bulge called aneurysm to form in the wall of a blood vessel. Surgery may need to remove this bulge. Blocked arteries also may require surgical treatment.

Living With Vasculitis:

Coping with side effects of your medication is one of your greatest challenges of living with vasculitis. To cope with this situation you need to understand your condition, follow your treatment plan, choose a healthy diet, get routine vaccinations, exercise most days of the week and maintain a strong support system.

  • You should be aware of the possible side effects of the drugs you take, to keep an observation about any changes in your health. Visit your doctor, undergo some more tests if required and check your blood pressure regularly.
  • Choose a diet that emphasizes fresh fruits and vegetables, whole grains, low-fat dairy products, and lean meats and fish which can help prevent potential problems that can result from your medications, such as thinning bones, high blood pressure and diabetes. You may need to take a vitamin D or calcium supplement if you are on corticosteroid drug.
  • Keeping up to date on vaccinations, such as for the flu and pneumonia, can help prevent problems that can result from your medications, such as infection.
  • Regular aerobic exercise, such as walking, cycling or swimming can help prevent bone loss, high blood pressure and diabetes that can be associated with taking corticosteroids. It also benefits your heart and lungs. In addition to these health benefits, many people find that exercise improves their mood and overall sense of well-being.
  • As talking with other people who are living with vasculitis can make you feel better, you can ask a member of your health care team about connecting with a support group. Family and friends can also help you as you cope with this condition.