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 (purpure), lumps (nodules) or sores (ulcers) an 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 (plasmapheresis) 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.