Vasculitis

Vasculitis is a group of rare diseases that cause inflammation of small, medium and large blood vessels. There are many types of vasculitis, such as giant cell arteritis (GCA), Kawasaki disease, microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA or Churg-Strauss), Behçet's, as well as others. Vasculitis causes poor blood flow to organ tissues such as the lungs, kidneys, skin, eyes, or nerves. Patients may have one or repeated episodes throughout their life. It may be an autoimmune disease, but some types of vasculitis are reactions to medications, cancer or viral infections like hepatitis B or C.

What Are the Signs/Symptoms?

Vasculitis symptoms include rashes that look like red spots (purpura), lumps (nodules) or sores (ulcers) on the skin, headaches with visual changes, shortness of breath, cough, and numbness or weakness in the hand or foot. Some patients may have joint pain, fatigue, or nose and sinus problems. Vasculitis may seriously affect kidneys but can have little to no symptoms initially. Diagnosis can be made by a rheumatologist and is based on physical exam findings, lab tests for specific autoantibodies and organ function, tissue biopsy, and sometimes imaging to look for blood vessel abnormalities.

What Are Common Treatments?

Glucocorticoids (Deltasone) are used to treat inflammation in many types of vasculitis. For milder vasculitis, methotrexate (Rheumatrex), azathioprine (Imuran) and other immunosuppressants may be used. Newer biologic drugs can treat certain types of vasculitis, such as rituximab (Rituxan) for GPA, MPA; tocilizumab (Actemra) for GCA; and mepolizumab (Nucala) for EGPA. Cyclophosphamide (Cytoxan) is an immunosuppressant used for life threatening disease. Some patients with severe disease receive treatments like plasma exchange (plasmapharesis) or intravenous immunoglobulin (IVIG). If severe vasculitis damages blood vessels or organs, surgery may be needed to repair them. This may include vascular bypass grafting, sinus surgery or kidney transplant.

Living with Vasculitis

Doctors may focus care on preventing permanent damage to organs like the lungs, kidneys, brain, or the nerves. Patients with vasculitis may have other troubling issues such as fatigue, arthritis, or sinus problems. Side effects from medications, such as glucocorticoids, may also need careful monitoring and management from a rheumatologist. Patients on immunosuppressants need to take precautionary measures to reduce infection risk. Depending on their vasculitis type, patients may also need care from an ophthalmologist, dermatologist, otolaryngologist, neurologist, nephrologist, or pulmonologist at times. With treatments, the outlook for patients with vasculitis is good.

Updated February 2023 by Kristen Lee, MD, and reviewed by the American College of Rheumatology Committee on Communications and Marketing.

This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.