Vasculitis Clinica Mayo

7/25/2019 Vasculitis Clinica Mayo

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Vasculitis is an inflammation of your blood vessels. It causes changes in the walls of blood

vessels, including thickening, weakening, narrowing and scarring. These changes restrict

blood flow, resulting in organ and tissue damage.

There are many types of vasculitis, and most of them are rare. Vasculitis might affect just

one organ, such as your skin, or it may involve several. The condition can be short term

(acute) or long lasting (chronic).

Vasculitis can affect anyone, though some types are more common among certain groups.

epending on the type you have, you may improve without treatment. !r you will need

medications to control the inflammation and prevent flare"ups.

Vasculitis is also known as angiitis and arteritis.

The signs and symptoms of vasculitis vary greatly and are often related to decreased blood

flow throughout the body.

General signs and symptoms common to most vasculitis types

#eneral signs and symptoms of vasculitis include$

%ever

&eadache

%atigue

'eight loss

#eneral aches and pains

ight sweats

ash

erve problems, such as numbness or weakness

*oss of a pulse in a limb

Signs and symptoms for some types of vasculitis


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!ther signs and symptoms are related to specific types of vasculitis. The symptoms can

develop early and rapidly or in later stages of the disease.

Behcet's (beh-CHET'S syndrome!This condition causes inflammation of your

arteries and veins. It generally appears in your +s and -s. igns and symptoms

include mouth and genital ulcers, eye inflammation, and acne"like lesions on yourskin.

Buerger's disease!This condition causes inflammation and clots in the blood

vessels of your hands and feet. It can cause pain in your hands, arms, feet and legs,and ulcers on your fingers and toes. This disorder is linked with cigarette smoking.

It is also called thromboangiitis (throm"boe"an"jee"I"tis) obliterans.

Cryoglobulinemia!This condition results from abnormal proteins in the blood. It is

often associated with hepatitis / infections. igns and symptoms include rash, jointpain, weakness, and numbness or tingling.

Eosinophilic granulomatosis "ith polyangiitis (also called Churg-Strauss

syndrome!This condition is very rare. It mainly affects the kidneys, lungs and

nerves in your limbs. ymptoms vary greatly and include asthma, nerve pain andsinus changes.

Giant cell arteritis!This condition is an inflammation of the arteries in your head,

especially at the temples. It usually occurs in people over age 0. #iant cell arteritis

can cause headaches, scalp tenderness, jaw pain, blurred or double vision, and even

blindness. It is also called temporal arteritis.

#iant cell arteritis is linked to polymyalgia rheumatica, which causes pain andstiffness in muscles of the neck, shoulders, hips and thighs.

Granulomatosis "ith polyangiitis (also called #egener's granulomatosis!This

condition causes inflammation of the blood vessels in your nose, sinuses, throat,lungs and kidneys. igns and symptoms include nasal stuffiness, sinus infections

and nosebleeds. The affected tissues develop lumps called granulomas. If the lungs

are affected, you may cough up blood. The kidneys are often affected. 1ut most

people don2t have noticeable symptoms until the damage is more advanced.

Henoch-Schonlein purpura!This condition is an inflammation of the smallest

blood vessels (capillaries) of your skin, joints, bowel and kidneys. igns and

symptoms include abdominal pain, blood in the urine, joint pain, and a rash on your

buttocks or lower legs. &enoch"chonlein usually affects children, but it can occurat any age.


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Hypersensitivity vasculitis!The primary sign of this condition is red spots on your

skin, usually on your lower legs. It can be triggered by an infection or an adverse

reaction to medicine.

$a"asa%i disease!This condition most often affects children younger than age 0.

igns and symptoms include fever, rash and eye inflammation. It is also calledmucocutaneous lymph node syndrome.

&icroscopic polyangiitis!This form of vasculitis affects small blood vessels,

usually those in the kidneys and lungs. 3ou may develop abdominal pain and a rash.

If the lungs are affected, you may cough up blood.

olyarteritis nodosa!This form of vasculitis usually affects the kidneys, the

digestive tract, the nerves and the skin. It is often associated with hepatitis 1

infections. igns and symptoms include a rash, muscle and joint pain, abdominalpain, high blood pressure, and kidney problems.

Ta%ayasu's (tah"kah"34&"soo5 arteritis!This form of vasculitis affects the larger

arteries in the body, including the aorta. It typically occurs in young women. igns

and symptoms include a feeling of numbness or cold in the limbs, loss of pulse,

high blood pressure, headaches, and visual changes.

#hen to see a doctor

6ake an appointment with your doctor if you have any signs or symptoms that worry you.

ome types of vasculitis can get worse 7uickly, so early diagnosis is key to getting effectivetreatment.

The e8act cause of vasculitis isn2t fully understood. ome types are related to a person2s

genetic makeup. !thers result from the immune system attacking blood vessel cells by

mistake. 9ossible triggers for this immune system reaction include$

Infections, such as hepatitis 1 and hepatitis /

1lood cancers

Immune system diseases, such as rheumatoid arthritis, lupus and scleroderma

eactions to certain drugs

1lood vessels affected by vasculitis may bleed or become inflamed. Inflammation can

cause the layers of the blood vessel wall to thicken. This narrows the blood vessels,

reducing the amount of blood : and therefore o8ygen and vital nutrients : that reaches

your body2s tissues and organs.


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/omplications of vasculitis depend on the type and severity of your condition. !r they may

be related to side effects of the prescription medications you use to treat it. /omplicationsof vasculitis include$

rgan damage!ome types of vasculitis can be severe, causing damage to major

organs.

Blood clots and aneurysms!4 blood clot may form in a blood vessel, obstructing

blood flow. arely, vasculitis will cause a blood vessel to weaken and bulge,

forming an aneurysm (4"yoo"ri5"um). 3ou may need surgery to remove ananeurysm.

)ision loss or blindness!This is a possible complication if giant cell arteritis is left

untreated.

*nfections!These include serious and life"threatening conditions, such as

pneumonia and blood infection (sepsis).

6ake an appointment with your primary care doctor if you have signs or symptoms thatworry you. If your doctor suspects that you have vasculitis, he or she may refer you to a

joint and muscle specialist (rheumatologist) with e8perience in helping people with this

condition. 3ou may also benefit from a multidisciplinary approach. 'hat specialists yousee depends on the type and severity of your condition.

pecialists who treat vasculitis include$

;oint and connective tissue doctors (rheumatologists)

1rain and nervous system doctors (neurologists)

rinary and urogenital system doctors (urologists)

#hat you can do


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1ecause appointments can be brief, and because there2s often a lot of ground to cover, it2s a

good idea to be well"prepared. Try to$

Be a"are of any pre-appointment restrictions!4t the time you make the

appointment, ask if you need to do anything in advance, such as restrict your diet.

+ist any symptoms you're e,periencingincluding any that may seem unrelated to

the reason for which you scheduled the appointment.

+ist %ey personal medical informationincluding other recent health problems or

major stresses you2ve had and any medications, vitamins and supplements you2re

taking.

Consider ta%ing a family member or friend "ith you to the appointment!

omeone who accompanies you can help remember what the doctor says.

+ist .uestionsyou want to ask your doctor.

%or vasculitis, some basic 7uestions to ask include$

'hat type of vasculitis do I have?

'hat2s causing my vasculitis?

'ill I need more tests?

Is my vasculitis acute or chronic?

'ill my vasculitis go away on its own?

Is my vasculitis serious?

&as any part of my body been seriously damaged by vasculitis?

/an my vasculitis be cured?

'hat are my treatment options?

'hat are the benefits and risks of each treatment?

Is there one treatment you feel is best for me?

&ow long will treatment last?


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I have another medical condition. &ow can I best manage these conditions together?

hould I see a specialist?

o you have any brochures or other printed material that I can take with me? 'hat

websites do you recommend?

#hat to e,pect from your doctor

3our doctor may ask$

'hen did you first begin e8periencing symptoms?

&ave your symptoms been continuous or occasional?

&ow severe are your symptoms?

'hat, if anything, seems to improve your symptoms?

'hat, if anything, appears to worsen your symptoms?

T


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fills these arteries or veins. The outlines of your blood vessels are visible on the

resulting @"rays.

Biopsy!This is a surgical procedure in which your doctor removes a small sample

of tissue from the affected area of your body. 3our doctor then e8amines this tissue

for signs of vasculitis.

TREATMENT

3our treatment will be focused on controlling the inflammation with medications andresolving any underlying disease that triggered your vasculitis. %or your vasculitis, you may

go through two treatment phases : stopping the inflammation and preventing relapse

(maintenance therapy).

1oth phases involve prescription drugs. 'hich drugs and how long you need to take themdepend on the type of vasculitis, the organs involved and how serious your condition is.

ome people have initial success with treatment, then e8perience flare"ups later. !thers

may never see their vasculitis completely go away and need ongoing treatment.

6edications used to treat vasculitis include$

Corticosteroids to control inflammation!3our doctor may prescribe a

corticosteroid drug, such as prednisone or methylprednisolone (6edrol). These help

control inflammation in the affected blood vessels. ide effects of corticosteroids

can be severe, especially if you take them for a long time. 9ossible side effectsinclude weight gain, diabetes and bone thinning (osteoporosis). If a corticosteroid is

needed for long"term (maintenance) therapy, you2ll likely receive the lowest dosepossible.

&edications to affect the immune system!If you don2t respond to corticosteroids,

your doctor may prescribe cytoto8ic or immunosuppressant drugs. These types ofdrugs kill or decrease the function of immune system cells causing the

inflammation. They include a5athioprine (45asan, Imuran), methotre8ate (Tre8all,

heumatre8) and cyclophosphamide. 9ossible side effects of cyclophosphamideinclude increased risk of cancer, infertility and infection. o even though this drug is

effective in controlling inflammation, it isn2t always the first choice, especially for

long"term therapy.

itu8imab (itu8an) is a safe and effective option for treating some types ofvasculitis. itu8imab has proved to be a good option for maintenance therapy,

unless you have had hepatitis 1. 4 side effect of ritu8imab is an increased risk of

reactivating hepatitis 1.

COPING AND SUPPORT


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!ne of your greatest challenges of living with vasculitis may be coping with side effects of

your medication. The following suggestions may help$

/nderstand your condition!*earn everything you can about vasculitis and its

treatment. =now the possible side effects of the drugs you take, and tell your doctor

about any changes in your health.

1ollo" your treatment plan!3our treatment plan may include seeing your doctor

regularly, undergoing more tests and checking your blood pressure.

Choose a healthy diet!

Vasculitis Clinica Mayo

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