The University of Pittsburgh Center for Vasculitis
Center Mission:
- To provide the best possible care for patients with vasculitis
- To provide education and support for patients and their families
- To provide access to new therapies for patients with vasculitis
Center Goals:
- To help develop better therapies for the treatment of vasculitis
- To contribute to what is known about the causes and disease processes in vasculitis, by participating in local and multicenter clinical and translational research
- To educate the public and other health care professionals about the diagnosis and treatment of vasculitis
Definition
Vasculitis is a disease characterized by inflammation of the blood vessels. This inflammation can result in thickening, narrowing, weakening or scarring of the blood vessels. There are many types of vasculitis. Some types occur suddenly, while others may evolve over weeks or months. Vasculitis can occur in a single place, like the skin, or involve multiple body systems. It may be mild and self-limited, or can be life threatening.
Vasculitis that occurs without any known cause is known as primary vasculitis. The primary vasculitides include the following diseases:
- Wegener’s granulomatosis
- Microscopic polyangiitis
- Churg-Strauss syndrome
- Henoch-Schonlein purpura
- Polyarteritis nodosa
- Kawasaki disease
- Primary angiitis of the central nervous system (CNS vasculitis)
- Giant cell arteritis/temporal arteritis
- Takayasu’s arteritis
Vasculitis can occur as a result of other diseases. These forms of vasculitis are known as secondary vasculitis and can be associated with the following conditions:
- Autoimmune disorders: systemic lupus erythematosus, rheumatoid arthritis, Sjogren’s syndrome
- Infections: viral, bacterial, and fungal infections can all be associated with secondary vasculitis
- Cancers (often blood cancers, such as lymphoma or leukemia)
- Medications
Signs and symptoms of vasculitis
The signs and symptoms of vasculitis can be different in any person, and depend on the type and location of the blood vessels involved. With any type of vasculitis, people often note feeling generally unwell, and may have fever, chills, fatigue, loss of appetite, or weight loss.
Specific organ systems of the body affected by vasculitis may cause the following signs or symptoms:
- Brain: stroke, headache, vision changes, memory loss
- Eye: inflammation, vision loss
- Ear, Nose, Throat and Sinuses: inflammation and infection, nose or mouth sores, nose bleeding, nasal congestion, hearing loss, ear pain, change in voice, hoarseness
- Lungs: shortness of breath, cough, chest pain, coughing up blood, abnormal chest x-ray or CT scan
- Heart: heart attack, angina
- Stomach and Colon: bleeding from stomach or bowels, abdominal pain/cramping, perforation (hole) in intestines
- Kidneys: blood in urine, protein in urine, kidney failure
- Nerves: numbness, tingling, shooting pain, or weakness in arms or legs
- Blood: anemia (low red blood cell count), elevated white blood cell count, or elevated platelet count
It is important to remember that each of these signs and symptoms can be seen in many other conditions, and are not specific for vasculitis.
Diagnosis
Unfortunately, in many patients, vasculitis may not be diagnosed right away. The signs and symptoms of vasculitis can be nonspecific, or may mimic another more common condition.
Tests that are often used to help make the diagnosis of vasculitis include:
- Blood tests: a wide variety of blood tests are often used in screening for and monitoring vasculitis. These tests may include blood counts, liver function tests, kidney function tests (creatinine), c-reactive protein, sedimentation rate, anti-neutrophil antibody test, and hepatitis screening.
- Urinalysis: blood and/or protein in the urine can be seen in patients in whom vasculitis involves the kidney. In addition, cellular casts, or collections of red or white blood cells in the urine that can be seen under the microscope, can also be helpful in evaluating for kidney involvement in vasculitis.
- Radiologic testing: X-rays, CT scans, MRI imaging, ultrasound, and angiography are often used to look for organ or blood vessel involvement in vasculitis.
- Biopsy: in many forms of vasculitis, the best way to help establish the diagnosis is to take a small piece of tissue of an affected tissue or organ for pathologic study.
Treatment
The specific medications used to treat any type of vasculitis are individualized to each patient and depend on their particular disease, organs or tissues affected, and underlying health conditions. The following medications are often used in the treatment of vasculitis:
- Steroids (cortisone, prednisone, methylprednisolone)
- Methotrexate (Trexall)
- Azathioprine (Imuran)
- Mycophenolate mofetil (Cellcept)
- Cyclophosphamide (Cytoxan)
- Rituximab (Rituxan)
- Infliximab (Remicade)
In addition, patients receiving treatment with steroids are at risk for developing osteoporosis (decreased bone density), which increases the risk of broken bones. Patients are generally given supplements of calcium and vitamin D when started on steroids, and may also take other medications to help protect or improve bone density.
Patients who are treated with intensive immunosuppression, such as steroids in combination with cyclophosphamide, also require antibiotic therapy to protect them from a form of pneumonia that is seen primarily in patients whose immune system is very suppressed.
Living with vasculitis
Although vasculitis can be a life threatening condition, if it is diagnosed and treated early, the outcomes are often good. In many cases, vasculitis is a chronic condition, like diabetes or high blood pressure, and being an active participant in your treatment and management is important in maintaining overall good health and well-being.
Some suggestions that may be helpful in coping with living with a chronic illness:
- Good communication with your doctor(s)-ideally you will be partners in dealing with this disease. Don’t be afraid to ask questions about your disease and treatment. You should leave your office visits with a good understanding about your disease, what is going on with your body, what your treatment is and what side effects may happen.
- Be committed to your role in taking care of yourself. Keep your office visits, stay on your medications as prescribed, and be vigilant about keeping up with blood tests or other tests recommended to monitor your disease. If you have problems with this aspect of your care for any reason (transportation to your visits, side effects or costs of medications), discuss these with your physician right away. Most often, these problems can be addressed to make sure you get the care and treatment that you need.
Part of good self care also includes following a healthy diet and getting regular exercise. Dieticians and physical therapists can be helpful in these aspects of your care, so if you’re not certain how to handle this part of your self-care, your doctor can help get you to the right people to help get this on track.
- Don’t be afraid to get help or support from others. For some patients, their spouse or family members can be a great source of help and emotional support. For others, learning more about their disease, or even educating other people about their disease, is helpful. Patient support groups can also be a good source of fellowship and information.