Vasculitis

Blood vessels extend throughout the body, so inflammation in a vessel wall can affect the area supplied by that vessel. Inflammation can thicken the vessel wall and narrow the vessel, or, less commonly, weaken it and cause it to bulge. Symptoms therefore vary depending on the location and size of the vessels involved.

In medicine, the first consideration when classifying vasculitis is which size of blood vessel is primarily involved. The international consensus nomenclature revised in 2012 (the Chapel Hill Consensus Conference) also uses this criterion.

• Large-vessel vasculitis — The aorta and its major branches
• Medium-vessel vasculitis — The main visceral arteries and their initial branches
• Small-vessel vasculitis — Capillaries, small veins, and small arteries

Vessels of several sizes can be affected in the same person, so the classification system serves as a guide to understanding the broad categories.

Vasculitis often occurs with systemic symptoms such as fever, fatigue, weight loss, and loss of appetite. Symptoms in the areas through which the affected vessels pass may occur as well. Musculoskeletal features include:

• Headaches at the temples or along the sides of the head
• A feeling that the jaw tires easily when chewing
• Morning stiffness in both shoulders and hips
• A feeling of heaviness and tiring easily when using the arms or legs
• Muscle pain and joint pain

These symptoms are also common with posture or muscle problems, colds, and post-exercise soreness. It is enough to be aware that they can also occur with vasculitis. At a hospital, clinicians assess the overall picture using the pattern of symptoms along with blood tests, imaging, biopsies, and other examinations.

Giant cell arteritis (also called temporal arteritis) mainly occurs in people aged 50 and older. It can affect not only the arteries on the outside of the head but also the aorta and its branches. Typical symptoms include a new headache around the temples, jaw pain or fatigue when chewing (jaw claudication), and discomfort when using the arms or legs. If the vessels supplying the eyes are affected, vision changes can occur. If you experience a headache for the first time after age 50 along with unusual changes in your vision, seek prompt assessment at a hospital. It sometimes occurs alongside Polymyalgia rheumatica, so the two conditions are often discussed together. Stiffness around the shoulders and hips is a characteristic symptom.

Takayasu arteritis is vasculitis affecting the aorta and its major branches. It is known to often begin at a relatively young age, particularly in women. Narrowing or blockage of the vessels can cause a weak pulse in one arm or different blood pressure readings between the arms, which is why it was once nicknamed “pulseless disease.” Conversely, changes involving dilation of the vessel walls can also occur.

Polyarteritis nodosa is a rare condition that causes inflammation in the walls of medium-sized arteries. The “poly-” in its name refers to multiple arteries. Symptoms in several areas may occur together, including fever and weight loss, muscle and joint pain, palpable skin nodules or red patches, and nerve symptoms in the hands and feet. Because muscle and joint pain are also common in everyday life, assessment of this condition considers the overall picture along with other systemic symptoms.

Among the small-vessel vasculitides, the group associated with antineutrophil cytoplasmic antibodies (ANCA) in the blood is called “ANCA-associated vasculitis.” ANCA are antibodies that react to proteins inside neutrophils, a type of white blood cell. The following three conditions belong to this group.

Granulomatosis with polyangiitis (GPA) can affect several areas, including the nose and sinuses, lungs, and kidneys. It was previously called “Wegener's granulomatosis,” and was given its current name in the 2012 international consensus nomenclature. Both names refer to the same condition.

Microscopic polyangiitis (MPA) causes inflammation in very small blood vessels, most notably affecting the kidneys and lungs. It can also occur in the skin, peripheral nerves, and gastrointestinal tract. “Microscopic” in the name refers to the tiny size of the affected vessels, not to mild symptoms.

Eosinophilic granulomatosis with polyangiitis (EGPA) was previously called “Churg–Strauss syndrome.” It is characterized by the combination of late-onset asthma, increased Eosinophils in the blood, and inflammation of small- to medium-sized vessels. Eosinophils are a type of white blood cell involved in allergic reactions. The skin, peripheral nerves, heart, and gastrointestinal tract can also be affected.

ANCA test results can help inform the diagnosis of all three conditions, but how often ANCA are detected varies by condition and individual.

• Keep a record of your symptoms. Writing down which symptoms began when, how long they lasted, and whether you had a fever or weight changes can help during a consultation.
• If fever, fatigue, or weight loss persists for several weeks or longer, do not assume it is only a muscle problem; visit a hospital for an assessment of your overall condition.
• Seek prompt assessment in these situations in particular. These include a first-time severe headache after age 50 accompanied by vision changes, a sudden change in the pulse or blood pressure in one arm or leg, coughing up blood, or a change in urine color.
• Treatment usually centers on medication to reduce inflammation. The healthcare team determines which medication to use based on the type of vasculitis and the areas involved.
• After diagnosis, work together to decide how to care for your body. Appropriate exercise intensity and which areas can receive massage vary with the type and stage of the condition. Discussing these with your healthcare team can help you manage your body with greater peace of mind.