Granulomatosis with polyangiitis
This is an uncommon disease in which the body's own immune system attacks the walls of small blood vessels.
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Medicines commonly prescribed for Granulomatosis with polyangiitis
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Dosage form: ORAL SOLUTION/SUSPENSION, 4 mg/mlActive substance: prednisoloneManufacturer: Laboratorio Aldo Union S.L.Prescription requiredDosage form: TABLET, 100 mgActive substance: azathioprineManufacturer: Dr. Falk Pharma GmbhPrescription requiredDosage form: TABLET, 50 mgActive substance: azathioprineManufacturer: Teofarma S.R.L.Prescription required
This is an uncommon disease in which the body's own immune system attacks the walls of small blood vessels. An inflamed vessel stops feeding its patch of tissue, and a pocket of destruction forms there, most often in the nose and sinuses, the lungs and the kidneys. It used to be called Wegener's granulomatosis. Half a century ago it left almost no way out; today the great majority of patients reach remission and live with it for many years. The whole difference lies in timing: the earlier the diagnosis is made, the fewer organs are damaged beyond repair.
What is actually happening inside
The disease belongs to the vasculitides, the inflammations of the blood vessel wall. Here it is the smallest vessels that suffer: capillaries, arterioles and venules. In the blood of most patients a particular kind of antibody is found, aimed at the contents of the person's own neutrophils and known by the abbreviation ANCA. These antibodies make blood cells stick to the vessel wall and unload destructive enzymes into it.
The name has two halves. Polyangiitis means inflammation of many vessels at once. Granulomatosis refers to the clusters of inflammatory cells, nodules that grow inside tissue and eat it away from within; they are the reason holes appear in the nasal septum and cavities in the lungs.
The cause is unknown. It is not catching, and it is not inherited in the ordinary sense, though a predisposition plays its part. It affects a few people per million each year, at any age, though more often in middle and later life.
Where it usually begins
The opening is nearly always misleading, and that is the heart of the problem: for the first months people are treated for a cold and sinusitis, because that is exactly what it looks like.
Stubborn complaints from the nose and ears deserve attention:
- a blocked nose lasting weeks that does not yield to the usual remedies;
- hard bloody crusts inside the nose that have to be cleared out every day;
- repeated nosebleeds;
- pain over the sinuses and chronic sinusitis that fails to respond to antibiotics;
- middle ear inflammation that drags on, with loss of hearing;
- hoarseness, a metallic-sounding cough and noisy breathing on exertion, which points to narrowing of the airway below the vocal cords.
Alongside this there are almost always general features that rarely get taken seriously: fever with no obvious cause, night sweats, aching joints and muscles, loss of appetite, weight loss and the sense of a long illness that never quite ends. Taken singly, all of that accompanies a dozen commonplace conditions. What counts is the combination: several weeks of unexplained ill health together with a nose that will not settle is a reason to arrange tests, not to change the drops.
How far it reaches
The set of organs involved is different in every person, and nobody has the full list.
- Lungs: a lasting cough, breathlessness, chest pain, blood in the sputum. Scans show nodules and cavities that are often mistaken for tuberculosis or a tumour.
- Kidneys, the most treacherous site of all. They do not hurt for a long time and give nothing away; the changes show only in the urine test, as protein and red cells. By the time swelling, high blood pressure and fatigue appear, half the kidney function has already gone. That is why, when this disease is suspected, everybody has a urine test and it is repeated regularly.
- Eyes: persistent redness with severe pain, a feeling of pressure, double vision, an eye that pushes forward, loss of sight.
- Nerves: a foot or a hand that suddenly drops, a numb patch of skin, caused by inflammation of the vessels supplying the nerve.
- Skin: purple spots and blotches, mostly on the lower legs, nodules and ulcers that will not heal.
- Less often the bowel, the heart and the coverings of the brain are involved.
When it cannot wait until tomorrow
Some manifestations unfold over days, and here the count is not in weeks. Seek emergency care at once, at the emergency department or by calling the European emergency number 112, if there is:
- blood in the sputum, especially with breathlessness that keeps increasing, which may mean bleeding into the lungs;
- breathlessness at rest, blue lips;
- noisy, whistling breathing on drawing air in, with the feeling that air is not getting past the throat;
- a sudden drop in vision, or eye pain with redness;
- a sharp fall in the amount of urine, urine the colour of meat washings, swelling that builds quickly;
- sudden weakness in a foot or hand, a drooping face, confusion;
- a high fever with shivering in someone taking medicines that suppress the immune system.
If the diagnosis is already made and old symptoms return or new ones appear, contact your specialist rather than waiting for the scheduled appointment: a flare is far easier to put out at the start.
How it is confirmed
There is no single test that answers yes or no, and reaching the diagnosis usually takes time.
What gets done:
- a blood test for ANCA. A positive result supports the diagnosis strongly, but a negative one does not rule it out: roughly one person in ten has no such antibodies, particularly when the disease is confined to the nose and sinuses;
- a urine test including the sediment, and assessment of kidney function from blood;
- a biopsy, a piece of tissue from an affected site: the lining of the nose, the kidney or the lung. This is the firmest evidence there is;
- computed tomography of the sinuses and chest, and other imaging if required;
- inflammatory markers and a full blood count.
Some investigations are repeated: sometimes the disease has to be caught as it develops, and a first biopsy from the nose often misses it, because the material turns out to be uninformative.
Treatment: first stop it, then hold it
Such patients are looked after by a specialist, usually a rheumatologist, working with kidney and ear, nose and throat colleagues. Treatment falls into two stages.
The first is to put out the active inflammation. Corticosteroids are used together with a drug that damps down the immune system: this may be a monoclonal antibody against B lymphocytes, or a cytotoxic drug. In severe disease threatening the kidneys and lungs, treatment starts in hospital, and sometimes a drug that blocks one of the inflammatory pathways is added, or a plasma cleaning procedure. In milder forms the regimen is gentler.
The second stage is keeping the disease from coming back. Doses come down, steroids are gradually withdrawn, and the maintenance drug carries on for several more years. Stopping it on your own once you feel well is the commonest cause of relapse.
Flares happen, and they are not a defeat: they are settled by going back to a more intensive regimen. Consequences are dealt with separately: a narrowed airway is widened endoscopically, severe kidney damage may call for dialysis or a transplant, and a collapsed bridge of the nose is corrected by plastic surgery, but only once the inflammation is reliably quiet.
Living on maintenance treatment
The drugs that hold the disease also weaken the defences against infection, and it is infection rather than the vasculitis itself that most often complicates the course. What that means in practice:
- any fever is dealt with by phoning the doctor rather than waiting it out, because on this treatment the usual signs of inflammation are muted;
- the doctor may prescribe an antibiotic to prevent a particular form of pneumonia that occurs only in people with weakened immunity;
- vaccinations are needed and planned ahead: live vaccines are generally avoided on this treatment, while the rest matter more than usual;
- with long-term steroids the bones are protected and blood pressure and blood sugar are watched;
- before a cytotoxic drug is started, preserving the chance of having children is discussed with men and women of reproductive age;
- regular blood and urine tests are not a formality: a kidney flare is caught exactly this way, long before it causes any symptoms.
Smoking is particularly harmful in this disease, whereas ordinary physical activity is beneficial and encouraged.
Online consultation
A remote appointment is a sensible place to go over a drawn-out picture that has not yet produced a diagnosis: a nose that has been bleeding and crusting for months, sinusitis that will not respond, a cough with weight loss and fever. The doctor lines the complaints up together, says which tests are worth arranging first and which specialist to see, so that weeks are not lost on repeated courses of drops. Where the diagnosis already exists, online is a convenient place to go through monitoring results, discuss side effects, plan vaccinations and work out which new sensation is a flare and which is not. The signs on the emergency list are not for remote assessment: they call for help straight away.
This material is for information only and does not replace medical advice.
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