What is ANCA-associated Vasculitis — GPA, MPA and EGPA?
ANCA-associated vasculitis is inflammation of small blood vessels driven by antibodies against neutrophils. It commonly affects the sinuses, lungs, kidneys and nerves, and it can damage kidney function within weeks. Once fatal in most cases, it is now a treatable disease — but the outcome depends heavily on how quickly it is recognised.
Also called: AAV, GPA, Wegener granulomatosis, MPA, EGPA, Churg-Strauss
Who gets ANCA-associated Vasculitis — GPA, MPA and EGPA?
Most often adults between 45 and 65, affecting men and women roughly equally. Three forms are recognised: granulomatosis with polyangiitis (GPA), which favours the sinuses, lungs and kidneys; microscopic polyangiitis (MPA), which favours kidneys and lungs; and eosinophilic granulomatosis with polyangiitis (EGPA), which occurs in people with adult-onset asthma and nasal polyps.
What are the symptoms of ANCA-associated Vasculitis — GPA, MPA and EGPA?
Not everyone has every symptom below, and having one does not confirm the diagnosis. These are the features that most often lead to assessment:
- Persistent blocked or bloody nose, crusting, sinus pain or recurrent sinusitis not responding to antibiotics
- Hearing loss, ear discharge or a change in the shape of the nasal bridge
- Cough, breathlessness, or coughing blood
- Blood or protein in the urine, reduced urine output, or swelling
- Foot drop, wrist drop, or patchy numbness and burning pain in the limbs
- Red or purple spots on the skin, or ulcers that will not heal
- Red painful eyes, or a bulging eye
- Fever, weight loss, night sweats and fatigue
- Worsening asthma with a high eosinophil count, in EGPA
How is ANCA-associated Vasculitis — GPA, MPA and EGPA diagnosed?
ANCA testing — PR3-ANCA and MPO-ANCA — supports the diagnosis, though a small proportion of patients are ANCA-negative and negative results do not exclude it.
Urgent urine testing for blood and protein, and kidney function, are done at first suspicion. Rapidly progressive kidney failure is the complication that determines outcome, and it is silent until advanced.
CT of the chest and sinuses, nerve conduction studies where nerves are involved, and — where feasible — a tissue biopsy of kidney, lung, sinus or nerve confirm the diagnosis. In EGPA the eosinophil count is markedly raised.
Infection is excluded rigorously, since it can mimic the presentation and the treatment is profoundly immunosuppressive.
How is ANCA-associated Vasculitis — GPA, MPA and EGPA treated?
Treatment has two phases. Induction brings the disease under control with corticosteroids plus either rituximab or cyclophosphamide; plasma exchange is used in selected severe cases. Avacopan is a newer agent that reduces the steroid burden.
Maintenance follows for at least two years, usually with rituximab or azathioprine, because relapse is common — particularly in PR3-positive disease.
Alongside this: prophylaxis against pneumocystis infection, bone protection, vaccination, and close monitoring of blood counts and kidney function. Infection is the leading cause of harm in the first year, and prevention is part of the treatment rather than an afterthought.
Living with ANCA-associated Vasculitis — GPA, MPA and EGPA
Long-term follow-up is essential, since relapse can occur years later and is easier to treat when caught early. Kidney function, urine and ANCA levels are monitored. Damage from the initial disease — hearing loss, nasal changes, reduced kidney function, nerve damage — may persist and is managed alongside the disease itself. Any new symptom suggestive of relapse should prompt prompt review rather than a wait-and-see approach.
When should you see a rheumatologist?
Persistent bloody or crusting nasal symptoms, unexplained cough with blood, blood in the urine, or a sudden foot drop should be assessed urgently. In someone with adult-onset asthma, new nerve symptoms or a rash needs prompt review.
Common questions about ANCA-associated Vasculitis — GPA, MPA and EGPA
How quickly does this need treating?
Urgently. Kidney function can decline over days to weeks and is often irreversible once lost. Assessment is arranged immediately when the diagnosis is suspected rather than waiting for an outpatient slot.
Can I have ANCA vasculitis with a negative ANCA test?
Yes. A minority of patients, particularly with disease limited to the sinuses or lungs, are ANCA-negative. The diagnosis rests on the clinical picture and, where possible, a biopsy.
Will it come back after treatment?
Relapse is common, especially in PR3-positive disease, which is why maintenance treatment continues for at least two years and monitoring continues indefinitely. Relapse caught early is much easier to treat.
Why am I on an antibiotic if I have an autoimmune disease?
Co-trimoxazole is given to prevent pneumocystis pneumonia, a serious infection that can occur during strong immunosuppression. In GPA it may also reduce relapses affecting the nose and sinuses.
This page is general information to support a consultation. It is not a diagnosis, and it does not replace an assessment by a doctor who has examined you.