Vasculitis

Also called Inflammation of the blood vessels, GPA, ANCA vasculitis

Inflammation of blood vessels, affecting almost any organ. Diagnosis is often late because it looks like a virus that will not go away.

What it is

A group of conditions in which the immune system attacks blood vessels, restricting blood flow and damaging the organs they supply.

Which vessels are involved determines everything. Giant cell arteritis affects large arteries in the head and threatens sight. ANCA-associated vasculitis, including granulomatosis with polyangiitis, affects small vessels and typically involves the sinuses, lungs and kidneys. Henoch-Schönlein purpura mainly affects children, causing a rash on the legs and buttocks, joint pain and abdominal pain, and usually resolves. Kawasaki disease is a vasculitis of childhood affecting the coronary arteries. Behçet's disease causes recurrent mouth and genital ulcers with eye inflammation.

Diagnosis is frequently delayed by months, because the early picture, fatigue, fevers, weight loss, aches, sinus problems, looks like a virus or a chest infection that will not settle. People are given repeated antibiotic courses while kidney function quietly deteriorates.

The clinical urgency is real. Untreated ANCA vasculitis can destroy kidneys within weeks and cause life-threatening lung bleeding. Treatment with steroids and immunosuppression, including rituximab, is highly effective, and the outlook has transformed over three decades.

The combination of persistent sinus or nasal symptoms with blood in the urine or a falling kidney function is a pattern that should prompt urgent testing.

Signs you might notice

General: fatigue, fever, night sweats, weight loss, muscle and joint aches, feeling persistently unwell.

Nose and sinuses: persistent blockage, crusting, bloody discharge, nosebleeds, or a change in the shape of the nose.

Lungs: cough, breathlessness, coughing blood.

Kidneys: blood or protein in the urine, often with no symptoms at all, found only on testing.

Skin: a purple non-blanching rash, particularly on the legs; ulcers; nodules.

Nerves: sudden numbness or weakness in a hand or foot, called foot drop or wrist drop.

Eyes: redness, pain, visual change. See uveitis.

999 or urgent: coughing blood; sudden vision change; rapidly reducing urine output; a rash that does not fade under a glass with fever; new weakness or numbness; or severe headache with scalp tenderness over 50.

How it can affect day-to-day life

The delay to diagnosis leaves many people with lasting organ damage that was avoidable, and legitimate anger about it.

Treatment is a long course of steroids plus immunosuppression, so the burdens of both apply: infection risk, bone protection, blood sugar, and the appearance and mood effects of steroids. See Cushing's syndrome.

Regular blood and urine monitoring is not optional; it is how relapse is caught before it does damage, and adherence to monitoring matters as much as adherence to drugs.

Relapse is common, and people learn their own early warning signs. Those should be written down and taken seriously by services.

Fatigue persists long after inflammation is controlled and is the commonest ongoing complaint.

Nasal crusting and sinus damage often persists and needs ongoing ENT care and daily saline irrigation.

Pneumocystis prophylaxis is standard with certain immunosuppressive regimens and is sometimes forgotten.

Supporting someone well

Take a persistent unwell picture seriously: weeks of fatigue, fevers, weight loss and sinus symptoms that antibiotics do not fix.

Ask for a urine dip and kidney function. Blood or protein in the urine with these symptoms is the finding that changes everything.

Ask for urgent rheumatology or nephrology referral, and for ANCA testing.

Once diagnosed, keep every monitoring appointment; that is how relapse is caught.

Ask for bone, stomach and infection prophylaxis alongside immunosuppression.

Keep vaccinations up to date, avoiding live vaccines while immunosuppressed.

Write down personal early warning signs of relapse and share them with the team.

Report any new symptom promptly rather than waiting for the next appointment.

Ask about fatigue management and about a specialist vasculitis centre.

Where to get help

A GP practice urgently for the pattern above; rheumatology or nephrology for diagnosis and treatment.

999 for coughing blood, sudden vision loss, or rapid deterioration.

Vasculitis UK run a helpline and are the specialist charity, with excellent patient information.

Kidney Care UK where the kidneys are involved.