Vasculitis refers to inflammation in the blood vessel wall (artery, vein, or capillary) that can cause narrowing (stenosis), dilation (aneurysm), and in some cases blood clots. It results when the immune system attacks the body's own blood vessels by mistake. In most cases, there is a genetic predisposition combined with environment exposure that leads to the disease.
The vascular specialists at Mount Sinai are highly skilled in the diagnosis and management of patients with vasculitis.
Vasculitis Causes and Risk Factors
In most cases, the cause of vasculitis is not known. Because it is likely a combination of genes that predispose to the disease and environment exposure it is impossible to prevent – each person is different. However, principles for overall vascular health, including avoiding tobacco products, blood pressure and cholesterol control will help all patients regardless of vasculitis.
Vasculitis presents with general symptoms (including fevers, night sweats, and weight loss) as well as those that are specific to the affected blood vessels and type of disease:
- Behcet's disease typically affects men and women ages 20-40 with origins from the old Silk Road (e.g., Turkey, Iran, China, Japan). Symptoms include recurrent oral and genital sores, artery and/or vein blood clots, artery aneurysms, skin rashes, and eye inflammation.
- Giant cell arteritis (GCA) affects the aorta and its branches like TA, but older patients (older than age 60) of European descent are usually affected. It is associated with temporal arteritis (inflammation of the temporal artery above the ear), which can cause headaches and result in blindness. There is overlap with polymyalgia rheumatic, a condition that causes weakness in the shoulder and hip muscles. Like TA, patients are at risk for artery stenosis and aneurysm.
- Scleroderma results in hardening or "sclerosis" of the skin, blood vessels, and other organs (e.g., stomach, lungs, musculoskeletal system). Most people with scleroderma are affected in their early to mid-adulthood. Symptoms may be limited to the skin, or throughout the body. Vascular symptoms include Raynaud's phenomenon (blue and white discoloration of the fingers/toes in cold), telangiectasias (vascular malformations visible on the skin or organs), and ulcers from lack of blood flow to the hands or feet. High blood pressure in the lungs (pulmonary hypertension), kidney failure with high blood pressure (scleroderma renal crisis), are more serious consequences.
- Takayasu's arteritis (TA) involves the aorta and its branches, resulting in aortic dilation (aneurysm) and/or artery narrowing (stenosis) that may progress to total blockage if not treated. Most often, arteries to the arms, legs, brain, heart ,and kidneys are affected can result in arm/leg pain with activity, stroke, heart attack, high blood pressure or kidney damage, respectively. Usually women ages 40 years or younger are affected, often with origins from Southeast Asia or South America. A difference in blood pressures between the arms may be the first sign of disease.
- Thromboangiitis obliterans (Buerger's disease) causes inflammation in the small arteries and veins related to tobacco use (e.g., cigarette, chewing tobacco). Most often young men and women are affected. Patients develop inflamed, painful veins (superficial thrombophlebitis). Hand/foot ulcers and gangrene due to inflammatory cell plugs that block blood flow can also occur; claudication (pain, fatigue with activity that goes away with rest) may develop. In its most severe form, amputation may be necessary. Patients who discontinue tobacco use completely rarely require an amputation.
Vasculitis is diagnosed with a combination of clinical signs, laboratory testing, and imaging. General markers of inflammation in the blood (e.g., C-reactive protein, erythrocyte sedimentation rate, interleukin-6) may be elevated in all diseases; however, there are specific one that help the vascular specialist to identify a specific type of vasculitis.
All patients with a suspected vasculitis should have a rheumatologic work-up to search out antibody markers for their disease. In addition, vascular imaging with ultrasound, computer tomography (CT), or magnetic resonance angiography (MRA) [three dimensional (3D) images that combine intravenous contrast to enhance blood vessels with CT or magnetic waves] should be performed on the entire body to evaluate for blood vessel involvement and complications (aneurysms or stenosis). Patients should be under the care of a vascular and rheumatology specialist to create a comprehensive and personalized diagnosis, plan for treatment, and long-term follow-up.
For most types of vasculitis, treatment includes suppression of the immune system with steroids or other related medications. The exception is Buerger's disease, which is best treated with tobacco cessation – the only definitive treatment to prevent progressive gangrene and amputation in these cases.
Other treatments include symptom management with medications or procedures to improve blood flow if needed. Regular imaging with ultrasound, CT or MR angiography, and echocardiography is crucial in some cases to monitor for disease activity and prevent complications. Blood work can also provide your physician with clues about your progress or disease activity. Each patient is unique, and your care plan should be tailored to meet your needs.
Long-Term Effects of Vasculitis
The long-term outlook depends on time of diagnosis and associated complications. If identified early, patients do much better than those that are diagnosed later. Regular follow-up with a vascular physician and rheumatologic specialist, that includes a monitoring and prevention program with imaging and blood work, can help prevent complications before they occur.
Division of Vascular and Endovascular Surgery
Vascular Interventions – Cardiac Cath Lab
1190 Fifth Avenue, 1st Floor
New York, NY 10029