Polymyalgia rheumatica (PMR)

Polymyalgia rheumatica is inflammatory: the immune system, its reasons unknown, inflames the joint linings and the fluid-filled sacs, the bursae, around the shoulders and hips, and the neck and back can ache with them. Genes and an outside trigger are each thought to contribute; an infection sometimes seems to set it off, but in most no trigger ever shows itself.

Its pattern is well recognised:

• It seldom starts before 50, and most people who develop it are over 65
• More women than men develop it
• It is seen most in people whose ancestry is northern European
• It is one of the commonest inflammatory conditions of later life

PMR and giant cell arteritis, the inflammation of the larger arteries that affects the same age group, go together: somewhere between one in twenty and one in five people with PMR develop GCA, the estimates vary, and up to half of those with GCA have PMR symptoms at some point.

PMR is felt in the shoulders, the neck and the hips, usually on both sides, and it is at its worst on waking:

• Aching and stiffness through the shoulders and upper arms, the neck, and the hips and thighs, on both sides as a rule
• Morning stiffness that lasts three-quarters of an hour or more and eases as you get moving
• Trouble with the movements that need the shoulders and hips: dressing, washing your hair, turning in bed, rising from a seat or climbing the stairs
• Pain that wakes you at night, and stiffness that returns after sitting still
• Sometimes tiredness out of proportion to everything else, a low fever, night sweats, a poor appetite or falling weight, and low mood

Some people have swollen hands, wrists or feet as well, or aching in the hand joints.

A new headache, a tender scalp, pain in the jaw or tongue when chewing or any change in how you see is another matter entirely: these are the signs of giant cell arteritis. They need attention the same day: the headache, scalp and jaw from an urgent GP appointment or NHS 111, and any change in sight, including sight that faded and came back on its own with none of the other signs of a stroke, at an eye casualty or urgent eye clinic, while 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.

PMR has no test of its own. The pattern makes the diagnosis: someone over 50, with new pain and stiffness in the shoulders or hips on both sides that has lasted two weeks or more, morning stiffness of over three-quarters of an hour and blood tests showing inflammation; the diagnosis then rests on the rapid response to steroid treatment. Just as much of the assessment goes into ruling out the conditions that imitate it, rheumatoid arthritis first among them, but also thyroid problems, infections, an inflamed-muscle condition and giant cell arteritis, which is why a wider set of blood tests, and sometimes a urine test or an X-ray, are part of the first consultation.

Ultrasound helps: it shows the inflamed linings and bursae around the shoulders and hips that PMR produces, and the joint changes that would point to arthritis instead. Point-of-care ultrasound is part of the consultation with Dr Borukhson, which usually means the shoulders and hips can be scanned when you first come; the ultrasound clinic page shows what happens.

Prednisolone tablets are the treatment, and the response is part of the diagnosis: with a moderate dose, most people are markedly better within days and close to normal within a month. A course that does not do that is a reason to think again about the diagnosis.

The dose is then brought down in stages, over months, with the size of each step and the time spent on it set by your symptoms and your blood tests. The whole course usually lasts one to two years and sometimes longer, and stopping too soon or too fast lets the inflammation return; steroids are never brought to a sudden halt, as the steroids in rheumatology guide describes. Over a course of that length, bone protection is considered from the outset, and your weight, blood pressure and blood sugar are kept under watch.

When lowering the dose brings the stiffness back, when flares keep returning, or when the steroid is doing more harm than the condition, a medicine that spares steroid is added: the usual choice is methotrexate, a disease-modifying medicine (DMARD) taken once a week, which holds the condition while the steroid comes down. Methotrexate damps down the immune system, so it can make an infection likelier. A biologic that blocks the action of a messenger called interleukin-6 is the next step for the smaller number whose PMR stays active despite this, under specialist supervision: in the UK sarilumab holds a licence for PMR that has not responded adequately to steroids or that flares as they are reduced, and tocilizumab, the related medicine that giant cell arteritis is treated with, has been studied in PMR as well. Both need blood tests at intervals and make infection likelier. Biologics that block TNF have no place in PMR. Paracetamol, and a short run of an anti-inflammatory where another condition such as osteoarthritis is adding to the pain, can help while the dose is falling.

A long, individual course needs regular specialist review, which finds the lowest dose that keeps the condition quiet and keeps bone protection and the other checks on schedule. You and Dr Borukhson settle the plan together.

Treatment works well in PMR. Within days of the first tablets most people feel much better, and in the end most stop the steroid altogether, although about two years is the usual course and some people need longer. A few habits make it easier:

• Keep moving, gently and often: comfortable everyday movement, with some stretching, keeps the shoulders and hips supple, and walking, the weight-bearing exercise that suits most people with PMR, protects the bones as well. A hot shower first thing can loosen the morning stiffness; on a long drive, stop and stretch.
• Look after your bones and your general health on steroids: calcium and vitamin D in sufficient amounts, weight-bearing exercise, no smoking and little alcohol each lower the risk of osteoporosis, and the bone protection guide goes into the detail.
• Take prednisolone as a single dose with breakfast, every day, and never stop or reduce it on your own, even when you feel well; your steroid card goes with you.
• Keep a note of how stiff you are as the dose falls, since not every ache is a flare: some are the ordinary wear and tear of the joints that the steroid had been hiding, and knowing which is which guides the reduction.

Pace the days that ask most of you, and be patient with a reduction that feels slower than it should: both make the course easier to see through.

PMR itself runs on planned reviews; the signs that cannot wait mostly belong to giant cell arteritis and to the steroid treatment.

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; 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
• An eye that suddenly and completely loses its sight, since giant cell arteritis can starve the eye of blood, and the loss is usually permanent
• 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 giant cell arteritis, which can follow PMR, can block an artery to the brain; sight that faded and came back on its own, with none of the other signs, belongs to the second list, for an eye casualty or urgent eye clinic the same day
• 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
• 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
• A blackout, since a heartbeat unsettled by steroids, or giant cell arteritis, which can follow PMR, can be behind it
• Sudden severe pain in the abdomen or back, since the pancreas can react to prednisolone
• 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 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 new headache, a scalp that is tender, or pain in the jaw or tongue when chewing, since each one can point to giant cell arteritis, which has to be treated the day it is suspected
• Sight that has blurred, doubled or dimmed, that is lost in part, or that faded and came back, which needs an eye casualty or urgent eye clinic the same day, since with giant cell arteritis these are the signs that the eye is at risk
• 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
• New chest pain, new palpitations, or breathlessness that is new or getting worse, since new breathlessness and a changed heartbeat are steroid effects as well as heart signs; palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting, belong to the first list
• 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, black or dark red stools, 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
• 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

Between reviews, let your team know promptly about the stiffness returning or spreading as the dose is lowered; about night sweats, or weight or appetite lost for no reason, which are checked because other illnesses can imitate PMR; and about any other steroid side effect that is bothering you, indigestion among them, which your GP or pharmacist would rather hear about than have you put up with. Symptoms that steroids no longer settle are a reason to revisit the diagnosis. If you cannot tell how urgent something is, asking is never a mistake.

Relief in PMR usually comes within days of starting treatment. The reasons to be seen quickly are the weeks of stiffness it saves, the conditions that imitate it and need a different treatment, and the possibility that giant cell arteritis is present or follows, which changes the urgency entirely. Shoulders and hips that have stiffened after 50, worst in the morning, are worth a specialist's eye, and the sooner the better.

Is PMR something I need to be seen about quickly?

Soon, from your GP, though not as a matter of hours. The stiffness can make dressing, washing and getting out of bed a struggle, and it eases quickly once treatment begins, so an early assessment spares you weeks of it. Just as important, the assessment checks for giant cell arteritis, the artery inflammation that accompanies PMR in a minority and that has to be treated the day it is suspected, by the routes the When to seek help section gives.

What does the first appointment involve?

Dr Borukhson listens to how the stiffness began and what it stops you doing, then examines you. Blood tests follow, to show the inflammation PMR usually produces and to rule out the conditions that mimic it. Point-of-care ultrasound is part of the consultation with Dr Borukhson, so the inflamed linings and fluid-filled sacs around the shoulders and hips that support the diagnosis can usually be looked for at the same visit. She then talks the findings through with you and proposes a plan, including the starting dose and the shape of the reduction to come.

For how long are steroids needed?

Longer than the symptoms suggest. Most people feel a marked difference within days of starting prednisolone and are far better within a week or two, but the dose is then lowered in small steps over months, guided by how you feel and by your blood tests; the whole course commonly runs to about two years, some people need a low dose for longer, and about a quarter are still on steroids four years on. Reduce too fast and the stiffness comes back, which somewhere between a quarter and a half of people see happen at some stage, and the answer is to go back up a step for a time. As the course is long, protecting your bones is looked at from the outset, and the tablets are never stopped without a plan.

How will I be monitored on treatment?

Through regular reviews while the dose comes down: every few weeks to begin with, then every few months through the first year, each one checking the stiffness, the inflammation markers in the blood and any steroid side effects, and setting the next step down. They also keep bone protection, and the checks on blood pressure and blood sugar, on track. Stiffness that comes back between appointments is a reason to contact the team before the next review, since a step back up may be needed; and symptoms that no longer answer to the steroid call the diagnosis into question.

When should I get help urgently?

For anything that could be giant cell arteritis: a new headache, a scalp that is tender, pain in the jaw or tongue when chewing, or a change in how you see. An eye whose sight goes 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. Call 999 and do not drive yourself to A&E. The headache, scalp and jaw symptoms need an urgent GP appointment or NHS 111 the same day. Sight that changes, including sight lost in part and sight that faded and came back on its own with none of the other signs of a stroke, needs an eye casualty or urgent eye clinic the same day. On steroids, methotrexate or a biologic, a fever is a same-day matter too, for your GP, your rheumatology team or NHS 111, and so is feeling generally unwell, since an infection needs treating early, and with the signs of sepsis in the first list under When to seek help it is a 999 or A&E emergency. The lists under When to seek help hold the full set, among them the signs of a stroke and the steroid signs.