vasculitis

Vasculitis is an inflammation of the blood vessels. It happens when the body's immune system attacks the blood vessel by mistake. It can happen because of an infection, a medicine, or another disease. The cause is often unknown.

Vasculitis can affect arteries, veins and capillaries. Arteries are vessels that carry blood from the heart to the body's organs. Veins are the vessels that carry blood back to the heart. Capillaries are tiny blood vessels that connect the small arteries and veins.

When a blood vessel becomes inflamed, it can:

• Narrow, making it more difficult for blood to get through.
• Close off completely so that blood can't get through.
• Stretch and weaken so much that it bulges. The bulge is called an aneurysm. If it bursts, it can cause dangerous bleeding inside the body.

Symptoms of vasculitis can vary, but usually include fever, swelling and a general sense of feeling ill. The main goal of treatment is to stop the inflammation. Steroids and other medicines to stop inflammation are often helpful.

NIH: National Heart, Lung, and Blood Institute

Giant cell arteritis is a disorder that causes inflammation of your arteries, usually in the scalp, neck, and arms. It narrows the arteries, which keeps blood from flowing well. Giant cell arteritis often occurs with another disorder called polymyalgia rheumatica. Both are more common in women than in men. They almost always affect people over the age of 50.

Early symptoms of giant cell arteritis resemble the flu: fatigue, loss of appetite, and fever. Other symptoms include:

• Headaches
• Pain and tenderness over the temples
• Double vision or visual loss, dizziness
• Problems with coordination and balance
• Pain in your jaw and tongue

Your doctor will make the diagnosis based on your medical history, symptoms, and a physical exam. There is no specific test for giant cell arteritis, but you may have tests that measure inflammation.

Treatment is usually with corticosteroids. Early treatment is important; otherwise there is a risk of permanent vision loss or stroke. However, when properly treated, giant cell arteritis rarely comes back.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a rare disease. It is a type of vasculitis, or inflammation of the blood vessels. The inflammation limits the flow of blood to important organs, causing damage. It can affect any organ, but it mainly affects the sinuses, nose, trachea (windpipe), lungs, and kidneys.

The cause of GPA is unknown. It can affect people at any age. Men and women are equally affected. It is more common in whites. Symptoms may include joint pain, weakness, tiredness, and cold symptoms such as a runny nose that doesn't get better. Doctors use blood tests, chest X-rays, and biopsies to diagnose GPA and rule out other causes of the symptoms.

Early treatment is important. Most people improve with medicines to slow or stop the inflammation.

Kawasaki disease is a rare illness that usually affects small children. Other names for the disease are Kawasaki syndrome and mucocutaneous lymph node syndrome. It is a type of vasculitis, which is inflammation of the blood vessels. Kawasaki disease is serious, but most children can fully recover if they are treated right away.

Kawasaki disease happens when the immune system injures the blood vessels by mistake. Researchers do not fully know why this happens. But when it does, the blood vessels become inflamed and can narrow or close off.

Genetics may play a role in Kawasaki disease. There may also be environmental factors, such as infections. It does not seem to be contagious. This means that it cannot be passed from one child to another.

Kawasaki disease usually affects children under the age of 5. But older children and adults can sometimes get it. It is more common in boys than girls. It can affect children of any race, but those with Asian or Pacific Islander descent are more likely to get it.

The symptoms of Kawasaki disease may include:

• High fever lasting at least five days
• A rash, often on the back, chest, and groin
• Swollen hands and feet
• Redness of the lips, lining of the mouth, tongue, palms of the hand, and soles of the feet
• Pink eye (conjunctivitis)
• Swollen lymph nodes in the neck

Contact your child's health care provider if your child has a fever for 4 days, especially if they have any other symptoms of Kawasaki disease.

Sometimes Kawasaki disease can affect the walls of the coronary arteries. These arteries bring blood and oxygen to your heart. This can lead to:

• An aneurysm (bulging and thinning of the walls of the arteries). This can raise the risk of blood clots in the arteries. If the blood clots are not treated, they could lead to a heart attack or internal bleeding.
• Inflammation in the heart.
• Heart valve problems.

Kawasaki disease can also affect other parts of the body, including the brain and nervous system, the immune system, and the digestive system.

There is no specific test for Kawasaki disease. To find out if your child has Kawasaki disease, your child's provider:

• Will do a physical exam, which includes looking at the signs and symptoms
• Will likely order blood and urine tests to rule out other diseases and check for signs of inflammation
• May order tests to check for damage to the heart, such as an echocardiogram and electrocardiogram (EKG)

Kawasaki disease is usually treated in the hospital with an intravenous (IV) dose of immunoglobulin (IVIG) antibodies. Antibodies are proteins that your immune system makes to fight infections.

Aspirin may also be part of the treatment. But do not give your child aspirin unless the health care provider tells you to. Aspirin can cause Reye syndrome in children. This is a rare, serious illness that can affect the brain and liver.

Treatment usually works. But if it is not working well enough, the provider may also give your child other medicines to fight the inflammation. If the disease affects your child's heart, they might need additional medicines, surgery, or other medical procedures.

Lupus is a chronic (long-lasting) type of autoimmune disease.Autoimmune diseases happen when your immune system attacks healthy cells and tissues by mistake. This attack causes inflammation. It can also damage many parts of the body, including the joints, skin, kidneys, heart, lungs, and brain.

There are several types of lupus:

• Systemic lupus erythematosus (SLE) is the most common type. It can be mild or severe and can affect many parts of the body.
• Cutaneous lupus affects the skin. It causes a rash or sores, usually after your skin is exposed to sunlight. The two major types of cutaneous lupus are discoid lupus and subacute cutaneous lupus.
• Drug-induced lupus is caused by a reaction to some medicines. The symptoms may start 3 to 6 months after starting the medicine. The symptoms usually go away when you stop taking the medicine.
• Neonatal lupus, which is rare, affects newborns. It is caused by certain antibodies that are passed from the pregnant parent to the baby.

The cause of lupus is unknown. Researchers are studying what might cause or trigger the disease, such as:

• Genetics
• Environmental factors, such as viral infections, sunlight, certain medicines, and smoking
• Problems with the immune system

Anyone can get lupus, but women get it much more often than men.

Lupus is more common in African Americans than in White people. It is also more common in people of American Indian and Asian descent. African American and Hispanic women are more likely to have severe forms of lupus.

Lupus can have many symptoms, and they differ from person to person. Some of the more common ones are:

• Arthritis, causing painful and swollen joints and morning stiffness
• Fever
• Fatigue or feeling tired often
• Red rashes, most often on the face (also called the "butterfly rash")
• Chest pain when taking a deep breath
• Hair loss
• Pale or purple fingers or toes, from cold and stress (Raynaud phenomenon)
• Sensitivity to the sun
• Swelling in legs or around eyes
• Mouth ulcers
• Swollen glands
• Headache and dizziness
• Confusion and memory problems

Symptoms may come and go. When you are having symptoms, it is called a flare. Flares can range from mild to severe. New symptoms may appear at any time.

Lupus causes inflammation throughout your body. This can cause problems in your organs, including:

• Kidney damage (lupus nephritis).
• Heart problems, including inflammation in the heart (myocarditis), heart valves, or lining of the heart muscle (pericarditis).
• Inflammation of blood vessels (vasculitis).
• Blood clots
• Inflammation of the tissue that surrounds the lungs (pleurisy). This can make it painful to breathe.

Some people with lupus may be more likely to develop other conditions, such as coronary artery disease (CAD) and atherosclerosis.

There is no specific test for lupus, and it's often mistaken for other diseases that cause similar symptoms. So it may take a while to get a diagnosis. To find out if you have lupus, your health care provider:

• Will ask about your symptoms, medical history, and family health history
• Will do a complete physical exam
• May order blood tests, such as ANA (antinuclear antibody), antibodies, complete blood count, and complement tests
• May order other tests, such as urine tests
• May do biopsies:
  • Skin biopsy (looking at skin samples under a microscope)
  • Kidney biopsy (looking at tissue from your kidney under a microscope)

There is no cure for lupus, but medicines and lifestyle changes can help control it.

People with lupus often need to see different providers. You will most likely have a primary care provider and a rheumatologist (a doctor who specializes in diseases of the joints and muscles). Which other specialists you will depend on how lupus affects your body. For example, if lupus is damaging your heart or blood vessels, you would see a cardiologist (a doctor who specializes in heart diseases).

Your primary care provider should coordinate care between all of your other providers and treat other problems as they come up. You and your primary care provider will develop a treatment plan to fit your needs. You will both review the plan often to make sure that it is working. You should report new symptoms to your provider right away so that your treatment plan can be changed, if needed.

The goals of a treatment plan are to:

• Prevent flares
• Treat flares when they occur
• Reduce organ damage and other problems
• Improve your quality of life

Treatments may include drugs to:

• Reduce fever, swelling, and pain
• Reduce inflammation in your body
• Prevent or reduce flares
• Reduce or prevent damage to joints
• Suppress (lower) the activity of your immune system

Besides taking medicines for lupus, you may need to take medicines for problems that are related to lupus such as high cholesterol, high blood pressure, or infection.

Alternative and complementary therapies are therapies that are not part of standard treatments. Some people try alternative and complementary therapies to improve their lupus symptoms. But research has not clearly shown whether these treatments may help or treat lupus. Talk to your provider before trying any new treatments.

It is important to take an active role in your treatment. It helps to learn more about lupus - being able to spot the warning signs of a flare can help you prevent the flare or make the symptoms less severe.

It is also important to find ways to cope with the stress of having lupus. Exercising and finding ways to relax may make it easier for you to cope. A good support system can also help.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

An allergy is a reaction by your immune system to something that does not bother most other people. People who have allergies often are sensitive to more than one thing. Substances that often cause reactions are:

• Pollen
• Dust mites
• Mold spores
• Pet dander
• Food
• Insect stings
• Medicines

Normally, your immune system fights germs. It is your body's defense system. In most allergic reactions, however, it is responding to a false alarm. Genes and the environment probably both play a role.

Allergies can cause a variety of symptoms such as a runny nose, sneezing, itching, rashes, swelling, or asthma. Allergies can range from minor to severe. Anaphylaxis is a severe reaction that can be life-threatening. Doctors use skin and blood tests to diagnose allergies. Treatments include medicines, allergy shots, and avoiding the substances that cause the reactions.

Behcet's syndrome is a disease that involves vasculitis, which is inflammation of the blood vessels. It causes problems in many parts of the body. The most common symptoms are:

• Sores in the mouth
• Sores on the sex organs
• Other skin sores
• Swelling of parts of the eye
• Pain, swelling and stiffness of the joints

More serious problems can include meningitis, blood clots, inflammation of the digestive system and blindness.

Doctors aren't sure what causes Behcet's. It is rare in the United States, but is common in the Middle East and Asia. It mainly affects people in their 20s and 30s. Diagnosing Behcet's can take a long time, because symptoms may come and go, and it may take months or even years to have all of the symptoms. There is no cure. Treatment focuses on reducing pain and preventing serious problems. Most people can control symptoms with treatment.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Polymyalgia rheumatica is a disorder that causes muscle pain and stiffness in your neck, shoulders, and hips. It is most common in women and almost always occurs in people over 50. The main symptom is stiffness after resting. Other symptoms include fever, weakness and weight loss. In some cases, polymyalgia rheumatica develops overnight. In others, it is gradual.

The cause of polymyalgia rheumatica is unknown. There is no specific test for it. Your doctor will use your medical history, symptoms, and a physical exam to make the diagnosis. Lab tests for inflammation may help confirm the diagnosis.

Polymyalgia rheumatica sometimes occurs along with giant cell arteritis, a condition that causes swelling of the arteries in your head. Symptoms include headaches and blurred vision. Doctors often prescribe prednisone, a steroid medicine, for both conditions. With treatment, polymyalgia rheumatica usually disappears in a day or two. Without treatment, it usually goes away after a year or more.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Rheumatoid arthritis (RA) is a form of arthritis that causes pain, swelling, stiffness and loss of function in your joints. It can affect any joint but is common in the wrist and fingers.

More women than men get rheumatoid arthritis. It often starts in middle age and is most common in older people. You might have the disease for only a short time, or symptoms might come and go. The severe form can last a lifetime.

Rheumatoid arthritis is different from osteoarthritis, the common arthritis that often comes with older age. RA can affect body parts besides joints, such as your eyes, mouth and lungs. RA is an autoimmune disease, which means the arthritis results from your immune system attacking your body's own tissues.

No one knows what causes rheumatoid arthritis. Genes, environment, and hormones might contribute. Treatments include medicine, lifestyle changes, and surgery. These can slow or stop joint damage and reduce pain and swelling.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Scleroderma means "hard skin." It's the name of an autoimmune disease that causes inflammation and thickening in the skin and other areas of the body. This inflammation causes you to have areas of tight, hard skin. Scleroderma may affect just one area of your body, or it can affect many systems in your body.

There are two main types of scleroderma:

• Localized scleroderma only affects your skin and the muscles and tissues just under your skin.
• Systemic scleroderma, which is also called systemic sclerosis, is a more serious type. It affects many parts of your body and can damage your blood vessels and internal organs, such as your heart, lungs, and kidneys.

The exact cause of scleroderma is unknown. Researchers think that several factors may play a part in causing the disease:

• Your genetics. Certain genes can increase the chance that you will develop scleroderma. They may also play a role in which the type of scleroderma you have. Scleroderma is not passed from parent to child, but you are more likely to develop it if a close relative has it.
• Your environment. Exposure to certain things in the environment, such as viruses or chemicals, may trigger scleroderma.
• Immune system changes. When your immune system changes, it can trigger your cells to make too much collagen in the body. Too much collagen causes patches of tight, hard skin.
• Hormones. Hormonal or immune system differences between women and men might play a part in the disease.

Anyone can get scleroderma, but certain factors may make you more likely to develop it:

• Your sex. Scleroderma is more common in women than in men.
• Your age. The disease usually appears between the ages of 30 and 50.
• Your race. Scleroderma can affect people of all races and ethnic groups, but the disease can affect African Americans more severely.

The symptoms of scleroderma are different for each person, depending on the type of scleroderma you have:

• Localized scleroderma usually causes patches of thick, hard skin in one of two patterns:
  • Patches in firm, oval shapes that stay in one area or spread to other areas of skin. This is called morphea.
  • Lines of thickened or different colored skin that run down your arm, leg, and, rarely, on the forehead. This is called linear scleroderma.
• Systemic scleroderma can cause problems with your internal organs as well as your skin. It can cause symptoms such as:
  • Thick, tight skin on your fingers
  • Fatigue
  • Raynaud's phenomenon, a narrowing of blood vessels in the hands or feet
  • Damage to your internal organs, including your digestive system, lungs, kidneys, and heart

There is no single test for scleroderma. The symptoms can vary from person to person and can be similar to those of other diseases. This can make scleroderma hard to diagnose.

To find out if you have scleroderma, your health care provider:

• Will ask about your symptoms and medical history
• Will do a physical exam
• May order blood tests, including an ANA (antinuclear antibody) test
• May do a skin biopsy
• May do other tests, such as imaging tests, to check for organ damage

There is no cure for scleroderma, but treatments can help control your symptoms and limit damage. The treatments may include:

• Medicines to help decrease swelling, manage pain, control other symptoms, and prevent complications.
• Physical or occupational therapy to help with pain, improve muscle strength, and teach you ways to help with daily living.
• Regular dental care, because scleroderma can make your mouth dry and damage connective tissues in your mouth. These problems can speed up tooth decay and cause your teeth to become loose.

You may need to see specialists to help treat your disease. Many people with scleroderma will see a rheumatologist. This is a doctor who specializes in rheumatic diseases such as arthritis and other inflammatory or autoimmune disorders. Dermatologists, who specialize in conditions of the skin, hair, and nails, may also play an important role in treating the disease. And if you have organ damage, you may need to see other specialists.

You can also help manage some of your symptoms, for example by:

• Dressing warm and avoiding cold or wet environments
• Quitting smoking (if you smoke)
• Putting on sunscreen before you go outdoors
• Using moisturizers on your skin to help lessen stiffness
• Avoiding hot baths and showers, harsh soaps, and household cleaners
• Getting regular physical activity

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases