Vasculitis

Vasculitis is not one illness. It is a group of rare conditions in which the immune system attacks the walls of blood vessels, and because blood vessels run everywhere, almost any part of the body can be caught up in it. Two people given the same broad diagnosis can describe completely different problems: one a rash on the shins, another a blocked nose that never clears, another a sudden change in vision.

What vasculitis is, and what it is not

There is no single description that fits all of it. Vasculitis Foundation Canada describes roughly 26 separate but related diseases, sorted by the size of the blood vessels involved and where in the body those vessels sit. The large vessel types include giant cell arteritis and Takayasu arteritis. The ANCA-associated small vessel types include granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Medium vessel types include polyarteritis nodosa and Kawasaki disease, which affects young children. Behcet syndrome, cryoglobulinemic vasculitis, skin-only vasculitis and vasculitis of the brain and spinal cord sit outside those groups again.

So vasculitis does not follow one course and does not have one outcome. Some forms stay in the skin. Others can threaten the kidneys, the lungs or eyesight quickly. Vasculitis is also not something you catch, and Vasculitis Foundation Canada states that researchers currently believe these conditions are not passed directly from parent to child.

Signs and symptoms

• Ongoing tiredness, fever, night sweats, aching muscles or unexplained weight loss
• A new or changed headache, tenderness of the scalp, or pain in the jaw when chewing
• A change in vision in one eye, including blurring, double vision or loss of sight
• A rash of small red or purple spots, most often on the lower legs, or skin ulcers
• A blocked or crusting nose, sinus pain, nosebleeds, ear problems or a change in hearing
• Cough, shortness of breath or coughing up blood
• Numbness, tingling or weakness in a hand or foot, or blood or protein found in the urine

Which of these appear, and how quickly, depends on the type and on which vessels are inflamed, so this list looks very different from one person to the next.

What raises the risk

Some things cannot be changed:

• Age, which differs by type. Giant cell arteritis is diagnosed almost only after 50 and is most common after 70, the ANCA-associated types peak between 60 and 79, and Kawasaki disease occurs mainly in children under 5
• Being female, since giant cell arteritis was about twice as common in women as in men in the Ontario data
• Genetic and geographic background, since rates of individual types vary widely between populations
• Living with another autoimmune or inflammatory condition, which can occur alongside some forms of vasculitis

Others can often be worked on, usually with support from a health care team:

• Workplace exposure to silica dust, in jobs such as construction drilling, agriculture and textiles, which a published review links to higher risk of ANCA-associated vasculitis
• Long-term hepatitis B or hepatitis C infection, which is linked to particular forms of vasculitis and which can be treated
• Certain prescription medicines and recreational drugs, which are linked to a drug-induced form of vasculitis. A prescriber or pharmacist can review a medication list

How vasculitis is diagnosed in Canada

There is no single test for vasculitis. Most people are seen first by a family doctor, a nurse practitioner or an emergency department, then referred to a rheumatologist, and often also to a kidney, lung, ear nose and throat or eye specialist depending on which organs are involved. Blood tests look for inflammation and for signs of kidney trouble, including CRP, ESR, complete blood count and creatinine, and urine is checked under the microscope and tested for protein. ANCA blood testing helps identify the small vessel group, and a negative result does not on its own rule vasculitis out. Imaging is common, including chest X-ray or CT, and CT of the sinuses when the nose and sinuses are involved. A tissue biopsy is often taken to confirm the diagnosis and rule out look-alike conditions, most often from the kidney, or from the temporal artery when giant cell arteritis is suspected. Doppler ultrasound of the temporal arteries is also used in Canada, and a rheumatologist-led fast-track ultrasound clinic has been evaluated here for that purpose.

Treatment and day-to-day management

Clinical research for Vasculitis is enrolling. See the current studies ↓

Care is usually described in two stages: bringing active inflammation under control, then keeping it quiet. What that involves depends on the type and on which organs are affected, so a specialist team may discuss:

• Glucocorticoid medicines to settle active inflammation, now often on faster tapering schedules following the PEXIVAS trial
• Medicines to bring the disease under control in the ANCA-associated types, including rituximab and cyclophosphamide, chosen with the pattern of organ involvement in mind
• Maintenance medicines aimed at reducing the chance of relapse, including repeat-dose rituximab, azathioprine and methotrexate
• Newer targeted medicines, including avacopan for severe ANCA-associated vasculitis, which CanVasc has issued Canadian consensus recommendations on, tocilizumab in giant cell arteritis, and mepolizumab in eosinophilic granulomatosis with polyangiitis
• Plasma exchange for some people with severe kidney involvement, alongside supportive care such as kidney follow-up, infection prevention, bone health and cardiovascular risk
• In Kawasaki disease, intravenous immune globulin and aspirin during the acute illness with cardiac follow-up afterwards, as set out in Canadian Paediatric Society guidance

None of that is a template. Which medicines fit your situation, and for how long, is a decision for you and your own specialist team, and this page is not a recommendation about any of them.

When to talk to a doctor

• Sudden loss or blurring of vision in one eye, double vision, or a new severe headache with scalp tenderness or jaw pain when chewing, in anyone over 50, needs assessment the same day. Go to an emergency department, because sight loss in giant cell arteritis can be permanent.
• Coughing up blood, new shortness of breath, or blood in the urine needs urgent assessment. Call 911 or go to an emergency department if breathing is difficult.
• A new rash of red or purple spots that does not fade when pressed, especially with fever, joint pain, numbness or feeling generally unwell, should be assessed promptly rather than watched at home.

Why clinical research matters for vasculitis

Vasculitis Foundation Canada states there is no known cause, and no cure, for the primary types of vasculitis. Relapse shapes a lot of what follows: the organisation reports that in ANCA-associated vasculitis relapses and flares occur in over half of people as time goes on, and a Montreal clinic found nearly half of its giant cell arteritis patients relapsed at least once. Long courses of glucocorticoids carry their own harms, so much of the current work is aimed at reducing them. CanVasc, the Canadian network for education and research on vasculitis, notes that only a few epidemiological studies on vasculitides in adults exist in Canada, so even the number of Canadians affected is unsettled for most types. Studies under way include complement-blocking and cytokine-blocking medicines, B-cell targeted approaches, glucocorticoid-sparing strategies, blood and imaging markers to predict relapse, and work on how quickly vasculitis is recognised and referred.

Clinical studies are how any of these questions get answered. Taking part is voluntary, you give informed consent before anything begins, and you can stop at any time without affecting the care you get from your own doctor. Joining a study does not mean giving up your current specialist, and no one can tell in advance whether a study will help you personally.

Learn more from Canadian sources

• Vasculitis Foundation Canada
• CanVasc: the Canadian network for education and research on vasculitis
• Kawasaki Disease Canada: about Kawasaki disease
• Canadian Paediatric Society: Kawasaki disease guidance