Vasculitis: Three Types to Know
A rare but serious inflammation of the blood vessels. A look at GPA (Wegener's), Behçet's disease, and Kawasaki disease in children.
Vasculitis is a rare but often serious autoimmune blood vessel disorder in which inflammation of the vessels can lead to serious complications. It can affect both sexes and all ages, and there are types involving large arteries, medium arteries, small and medium arteries, small arteries, and vessels of various sizes. Here are three types worth knowing.
Granulomatosis with polyangiitis (GPA, formerly Wegener's) is a rare vasculitis affecting small and medium blood vessels, with tissue injury that forms granulomas on biopsy. It commonly affects the lungs, kidneys, sinuses, eyes, ears, skin, and joints. Many people present with nosebleeds, shortness of breath, cough with bloody sputum, skin rashes, eye redness, weight loss, and numbness. A blood test called ANCA is often positive. Microscopic polyangiitis (MPA) shares many features.
Behçet's disease is a rare syndrome affecting young adults of both sexes. Features include painful mouth sores, acne-like or tender skin nodules (often on the legs), painful genital ulcers, eye inflammation (uveitis), joint pain and swelling, and brain inflammation (headache, stroke). Complications may include blood clots, aneurysms, and vessel blockage.
Kawasaki disease is a medium-vessel vasculitis in children, notable for a possible association with COVID-19. Features include fever lasting five or more days, a diffuse rash, red eyes without discharge, red or swollen palms and soles that may peel, 'strawberry tongue,' swollen neck lymph nodes, and irritability. Complications include coronary aneurysms, heart attacks, heart valve problems, and blood clots.
Steroids and other immunosuppressant medications are used to treat these blood vessel disorders. Because vasculitis can progress quickly, early recognition and specialist care are essential.
Dr. Maggie Cadet, MD