Vasculitis

In vasculitis the immune system attacks the wall of a blood vessel. The wall swells, the channel narrows or closes, and the tissue beyond is starved of blood; a weakened wall can also bulge into an aneurysm and, at worst, burst. Why the attack begins is seldom found. Some forms follow an infection, notably hepatitis, some are set off by a medicine, and some accompany another autoimmune illness, lupus, rheumatoid arthritis or Sjögren's among them; the rest arise on their own and are thought to be autoimmune. Vasculitis is not inherited directly, though genes contribute and it occasionally runs in families, and it is not infectious.

It is uncommon: in the UK about ten to fifteen people in every 100,000 develop a vasculitis each year; the exception is giant cell arteritis, common enough in the over-50s to be familiar in later life. Each type has its own age and pattern: giant cell arteritis almost always after 50, Takayasu arteritis in young women, IgA vasculitis mostly in children, Kawasaki disease mostly in the under-fives. Polymyalgia rheumatica and Behçet's disease are close relations, each with a guide of its own.

Many people feel generally ill first: tired, feverish, sweating, off their food, losing weight. What follows depends on the vessels involved:

• Skin: a rash of small purple or red spots that do not fade when pressed, or spots that break down into ulcers
• Joints and muscles: aching, and sometimes swollen joints
• Nose, sinuses and ears: crusting and bleeding inside the nose, sinus pain, a hoarse voice, hearing loss
• Lungs: a cough that does not clear, breathlessness, coughing up blood
• Kidneys: often silent at first, so that the first sign is blood or protein on a urine test; later, red or brown urine, swollen legs or a rising blood pressure, which is why urine is tested at reviews
• Nerves: pins and needles, numbness, burning pain or weakness in a hand or a foot
• Eyes: redness, pain or altered sight
• Gut: abdominal pain, especially after eating, and bleeding
• Brain: occasionally a stroke

In older adults the form to know is giant cell arteritis; its signs are a new headache, tenderness over the temples, jaw pain on chewing, or altered sight, and they need care the same day, from an urgent GP appointment or NHS 111, or, for altered sight with none of the other signs of a stroke, from an eye casualty or urgent eye clinic; sight lost suddenly and completely is a 999 or A&E emergency, and so is a headache that comes on suddenly and severely, as the first list under When to seek help says.

The members of the vasculitis family are sorted by the size of the vessels each one favours. The main ones are:

Large-vessel vasculitis (LVV)

Large-vessel vasculitis, LVV for short, attacks the aorta and the big arteries that branch from it. Its two forms start at different ages:

• Giant cell arteritis, among the commonest forms of vasculitis, which as a rule starts after 50 and shows itself most at the arteries of the temples, though it often reaches the aorta as well; it endangers sight and is treated the day it is suspected
• Takayasu arteritis, rare in the UK, which affects the aorta and the vessels leaving it, in younger people, most of them women

Polymyalgia rheumatica sits alongside them: a close relative of giant cell arteritis, sometimes counted in the same group.

Medium-vessel vasculitis

• Polyarteritis nodosa, very rare, which inflames the medium-sized arteries to the gut, the kidneys and the nerves, and can be linked to hepatitis B
• Kawasaki disease, which mostly affects children under five and can inflame the coronary arteries

Small-vessel vasculitis

• Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), which affect the lungs and the kidneys, and beyond them the nose, sinuses and ears in GPA and the nerves in MPA, and are marked by ANCA antibodies in the blood
• Eosinophilic granulomatosis with polyangiitis (EGPA), which begins with adult-onset asthma and allergy and can reach the nerves and the heart
• IgA vasculitis, also called Henoch-Schönlein purpura, with its purple rash over the legs and buttocks, joint pain, abdominal pain and, in some, the kidneys, most often in children and usually settling on its own
• Cryoglobulinaemic vasculitis, in which abnormal proteins that clump in the cold block the small vessels of the skin, nerves and kidneys, sometimes after hepatitis C

Vasculitis can also accompany another condition, rheumatoid arthritis for example, and a rash caused by a reaction to a medicine is a vasculitis of a kind that passes once the medicine stops. Telling a mild, limited form from a serious one that needs strong treatment quickly is the point of a careful assessment.

No one test settles it; the picture comes from what you report and what the examination shows; from blood tests for inflammation, for anaemia, for the ANCA antibodies that mark GPA, MPA and EGPA, and for the kidneys and liver; from urine tests for blood and protein, often the first hint that a kidney is inflamed; from X-rays and CT or MRI scans of the organs involved; from a heart tracing and an echocardiogram where the heart may be affected; and, where a tissue can be sampled, from a biopsy of the skin, a kidney, a muscle, a lung or the lining of the nose to confirm inflammation in the vessel wall. In the large-vessel forms, scans of the arteries themselves show where the walls are inflamed. Everything that follows turns on the type and on how far it has spread, so the assessment goes into detail, and moves fast when the illness looks serious, because those forms cannot wait.

Ultrasound has a place where joints or the tissues around them are inflamed, and in the large-vessel forms the arteries themselves can be scanned. Point-of-care ultrasound is part of the consultation with Dr Borukhson; the ultrasound clinic page walks through the scan.

The treatment is matched to the type and the severity, and it has three jobs: to bring the inflammation under control, to protect the organs under attack, and then to hold the illness quiet. Vasculitis limited to the skin may need no more than treating the infection or stopping the medicine that set it off. The large-vessel forms are treated with prednisolone tablets, sometimes with methotrexate or the biologic tocilizumab added to bring the steroid down. The small- and medium-vessel forms that reach the organs are treated in two stages: first the inflammation is suppressed with steroids together with rituximab, a biologic, or cyclophosphamide, a chemotherapy medicine, and, for some, avacopan, a newer tablet that lets the steroid course be cut short; then the remission is kept, usually with rituximab or with azathioprine or methotrexate, mycophenolate where those do not suit, for two to four years, with the doses brought down as low as safety allows. A few of the most severe kidney cases need plasma exchange or dialysis; EGPA can be treated with a biologic that blocks the action of a messenger called interleukin-5, from the start in its milder forms and, in any form, to hold remission and bring the steroid down; Kawasaki disease is treated with immunoglobulin given by drip, and aspirin.

These medicines are powerful. Infection becomes likelier on them, and blood tests are part of the routine; cyclophosphamide can harm fertility in both men and women, and so is swapped for a gentler medicine as soon as the vasculitis is controlled; and a long steroid course means protecting the bones. A systemic vasculitis is usually followed for years, so a specialist team stays with it throughout, and the plan changes with the illness and is discussed with you at every review.

Vasculitis can affect the lungs, the kidneys, the nose and ears, the eyes, the nerves and the skin, so one consultant, usually a rheumatologist, holds the plan as a whole and the other specialties join for the organs involved. Dr Borukhson practises within a world-renowned tertiary centre, where the kidney, respiratory, ear, nose and throat, eye and skin specialists a vasculitis may call for are consultants she works with directly; the hardest cases come to a multidisciplinary team meeting (MDT), where those specialists decide the plan, so that decisions are reached together and promptly.

The treatment of a systemic vasculitis is in your specialist team's hands; how you live with it is in yours:

• Medicines are taken as prescribed; a steroid is reduced only under guidance, never stopped on your own; and any change you have in mind is asked about first
• Keep every review and every blood and urine test, since they catch a quiet relapse, and treatment side effects, before either does harm
• Have the flu and pneumonia vaccines; have no live vaccine while on the medicines that suppress immunity, and ask before any other; a fever, or contact with measles, chickenpox or shingles, while the medicines have your defences down is a same-day matter, as the next section says
• Stop smoking: it narrows blood vessels that are already under attack and worsens lung inflammation
• Long-term steroids push up blood pressure and blood sugar and thin the bones, so all three are watched; a balanced diet with enough calcium helps the bones
• Rest when the illness demands it and rebuild strength gently as it settles, with walking and swimming among the good choices
• If you are hoping to become pregnant, talk to your specialist first: methotrexate is stopped and swapped first, cyclophosphamide can affect fertility, and the illness should be quiet; the pregnancy guide lists the medicines

Most people do well on treatment and many recover fully, though a relapse later is possible. Fatigue is a major effect of vasculitis for many, often overwhelming, and it can persist, so balance rest with gentle activity, be patient with yourself, and lean on your GP, your family and the patient organisations for vasculitis.

With organs at stake, some of what vasculitis and its treatment can bring cannot wait for a planned review; what follows is that part.

Treat any of these as a 999 or A&E emergency: call 999 and do not drive yourself to A&E.

• Severe illness with suspected sepsis: a high fever with shaking, confusion, rapid breathing, or feeling dramatically worse over hours
• With a fever or feeling unwell, a rash that does not fade when you press it, a stiff neck with eyes that cannot bear bright light, or skin, lips or tongue turning blue, grey, pale or blotchy, since these can be signs of meningitis or sepsis, and with a fever or feeling unwell a rash of this kind cannot be assumed to be the rash of vasculitis; on black or brown skin the rash and the change of colour may be easier to see on the palms of the hands or the soles of the feet
• Chest pain that is crushing, tight or like a pressure, that spreads to an arm, the jaw, the neck, the stomach or the back, that comes with breathlessness, sweating, feeling sick or feeling light-headed, or that came on suddenly and does not go away
• Breathlessness that is severe, with gasping, choking or no breath to get words out; or coughing up blood, since the small-vessel forms can bleed into the lungs
• Palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting, since a heart whose muscle or rhythm is affected has to be checked at once
• Stridor or any noisy breathing, stridor being a harsh, high-pitched sound as you breathe in, since GPA can narrow the windpipe below the voice box
• A blackout, since the heart's rhythm can be disturbed when vasculitis reaches it
• Sudden weakness or numbness on one side, a drooping face or difficulty speaking, a sudden severe headache, sudden confusion or a seizure, or sudden loss of vision, any of which can be a stroke, even if it stops after a short time, and vasculitis can involve the arteries to the brain; in giant cell arteritis, sight that faded and came back on its own, with none of the other signs of a stroke, belongs to the second list, for an eye casualty or urgent eye clinic the same day
• An eye that suddenly and completely loses its sight, since the artery feeding the eye can block in giant cell arteritis and some other forms
• Sudden severe pain in the abdomen or back, since an artery to the gut that is inflamed, or a weakened and bulging artery wall, can block, burst or leak
• An arm or leg that has suddenly gone cold, pale, numb or very painful and stays so, since in the large- and medium-vessel forms the artery feeding a limb can block
• Lips, mouth, throat or tongue that suddenly swell, a throat that feels tight, very fast breathing or a struggle to breathe or to swallow, skin, tongue or lips turning blue, grey or pale (on black or brown skin this may be easier to see on the palms of the hands or the soles of the feet), becoming suddenly very confused, drowsy or dizzy, or fainting and not being able to be woken, since this can be a severe allergic reaction, anaphylaxis, which can follow a dose of any medicine
• Vomiting blood, or bleeding from the back passage that is heavy or will not stop, most of all with faintness, confusion or pain in the abdomen, or vomit that is black or dark brown, since the gut can bleed when its vessels are inflamed and a stomach ulcer on prednisolone can bleed
• Thoughts of harming yourself or ending your life, which steroids can bring on

Any of these needs an assessment that is urgent, on the same day, from your GP, your rheumatology team or NHS 111:

• A fever, or feeling generally unwell, on immunosuppressant, biologic or steroid treatment, since an infection has to be treated early
• A joint that is hot and swollen, with a fever or feeling unwell, since an infected joint has to be ruled out at once, at A&E or through NHS 111
• A joint that has become hot and very swollen suddenly, or is worsening quickly, even without a fever, since an infected joint does not always announce itself with one; a joint that will take no weight at all, or that cannot be moved, is a 999 or A&E emergency, whether or not there is a fever
• A new headache, a scalp that is tender, or pain in the jaw or tongue when chewing, in someone over 50, since giant cell arteritis, which cannot wait a day, can announce itself with any one of them
• A new red, painful eye, or sight that has blurred, dimmed or changed, which needs an eye casualty or urgent eye clinic the same day, as does an eye that light hurts, since vasculitis can inflame the eye or starve it of blood, and early treatment protects sight; in pregnancy, any change in sight, blurring or flashing lights among them, is also for your midwife or maternity unit straight away, as the pregnancy line below says, since it can be a sign of pre-eclampsia
• New chest pain, new palpitations, or breathlessness that is new or getting worse, since vasculitis can involve the heart as well as the lungs; palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting, belong to the first list; in pregnancy, breathlessness that comes on suddenly, or when you lie down, or with dizziness, is for your midwife or maternity unit straight away, as the pregnancy line below says
• A persistent dry cough with a fever, with new breathlessness, or that is worsening over hours to days
• Marked swelling of the face or ankles, or frothy or bloody urine, or a marked fall in how much urine you pass or a blood pressure reading far above your usual, since the kidneys' small vessels can be inflamed, with damage under way by the time these signs show
• A new rash of purple or red spots that do not fade when pressed, in someone who otherwise feels well, since it can be the vasculitis itself starting or returning, and the kidneys are checked with it
• Numbness, pins and needles, burning pain or weakness in a hand, arm, foot or leg that is new, with none of the stroke signs above, since a nerve can be starved of its blood supply, and the sooner it is treated the less damage is done
• Hearing loss that has come on suddenly, or dizziness or a spinning sensation that is new, since vasculitis can strike the inner ear
• Black or dark red stools, or bloody diarrhoea, since bleeding from the gut has to be found
• A marked change in mood or behaviour, confusion, or seeing or hearing things that are not there, on steroid treatment, since prednisolone can do this, the more so the higher the dose; confusion that comes on suddenly is a 999 or A&E emergency, as the first list says
• Unusual thirst or hunger, passing urine more often, or feeling sleepy or confused, on steroids, or swelling of the arms or legs, bruising or bleeding that is not normal, severe stomach pain, severe back pain, a severe upset stomach, being sick, or very bad dizziness, since each can be a steroid effect that needs checking without delay; pain in the abdomen or back that comes on suddenly and severely is a 999 or A&E emergency, as the first list says; in pregnancy, bleeding that is not normal, and stomach pain that is severe, belong to the pregnancy line below
• Contact with measles, chickenpox or shingles, since these infections can be severe on steroids and a medicine to protect you may be needed
• Being near someone with measles, chickenpox or shingles, while you take a medicine that weakens the immune system, since measles and chickenpox can be dangerous when the immune system is weakened, and you can catch chickenpox from someone with shingles if you have never had chickenpox; NHS 111 can tell you what to do that day
• In pregnancy, any bleeding, stomach pain that is severe or does not go away after a rest, reduced baby movements, a severe headache or visual disturbance, sudden swelling of the face, hands, legs or feet, or breathlessness that comes on suddenly, or when you lie down, or with dizziness: contact your midwife or maternity unit straight away, whatever the time; bleeding that is heavy, or that comes with severe pain in the tummy, pain in the shoulder, or feeling sick, faint or dizzy, or passing out, is a 999 or A&E emergency: call 999 and do not drive yourself to A&E; pain in the abdomen or back that comes on suddenly and severely is a 999 or A&E emergency, as the first list says
• A high temperature in pregnancy, whatever medicines you are taking, since a fever can be a sign of an infection that needs checking; your midwife or maternity unit can advise

A persistent dry cough on its own, or breathlessness that has drifted slowly, over weeks or months, is something to tell your team promptly, without waiting for a routine slot. So are nosebleeds or crusting inside the nose, a voice that has turned hoarse, sinus pain that lingers, hearing that is slowly getting worse, skin spots that break down into ulcers, a new ache in the abdomen after eating that is not severe, and sweats or a loss of weight creeping back, any of which can be the illness stirring, and it is simpler to bring back under control when caught early. Outside pregnancy, in someone not on immunosuppressant, biologic or steroid treatment, a fever that goes on with no explanation is a prompt matter too, for your GP or your team, since vasculitis and infection can each be behind it.

Aches or tiredness creeping back on their own, with none of the signs above, are reasons to ask your team for a review brought forward rather than left to the routine appointment. If you cannot tell how urgent something is, asking is never a mistake.

Vasculitis ranges from a rash that clears on its own to an illness that can damage the kidneys or lungs if treatment is late, and the two can start with the same general symptoms: fever, sweats, tiredness, weight loss, aches. What decides the outcome is how early the type is identified and treatment begun, since damage done before then is often permanent, while most types respond well once treatment starts. If you have unexplained symptoms of that kind with inflammation in your blood, a specialist review can establish whether vasculitis is behind them, which type it is and what it has reached, and begin the treatment that fits.

How is vasculitis diagnosed?

Nothing settles it in one test. The diagnosis is built from your account, the examination, blood tests for inflammation and for the antibodies that mark some types (the ANCA antibodies chief among them), urine tests for blood and protein that show whether the kidneys are caught up in it, scans of whichever organs are affected and, often, a biopsy of an affected tissue. The treatment depends on the type and on the organs it has reached, which is why the assessment leaves nothing out. Ultrasound earns its place too, for inflamed joints and the tissues round them, and for the arteries themselves in the large-vessel forms. Point-of-care ultrasound is part of the consultation with Dr Borukhson, and can often be done at the first visit.

Is vasculitis serious?

The type decides: it ranges from minor to serious. Vasculitis limited to the skin usually clears as the inflammation subsides, and IgA vasculitis in a child commonly gets better on its own. The forms that reach the kidneys, lungs, heart or nerves are serious, and are treated from the day they are found, because damage done before treatment is often permanent. Most types respond well to treatment, many people recover fully, and a relapse, if it comes, can be treated again. Working out where on that range your illness sits is what the specialist assessment is for.

Is vasculitis cured, or is it a long-term condition?

Some forms come once and go. Most of the systemic forms are long-term conditions in which treatment brings the inflammation under control, protects the organs and then holds the illness quiet, often for years, with the medicines reduced as far as they safely can be. Many people do very well and settle into long stretches of good health, though a relapse is possible, which is why reviews continue. The approach is discussed with you at each stage.

Why might other specialists join my care?

It depends on the organs. Because a vasculitis can involve the kidneys, lungs, nose and ears, eyes, nerves or skin, one consultant holds the plan as a whole, usually a rheumatologist, and the other specialties are brought in for the organs affected. Dr Borukhson is based in a world-renowned tertiary centre, with consultant colleagues in kidney, lung, ear, nose and throat, eye and skin medicine she can call on directly, and the hardest cases are taken to a multidisciplinary team meeting so that decisions are reached together and quickly.