Polymyalgia Rheumatica and Giant Cell Arteritis

Common, Dangerous, Treatable

By Ruth Werner
[Pathology Perspectives]

Have you ever had any senior clients who came to you for help with shoulder pain, hip pain, or a headache?
Have they ever been slow to respond to massage, or even become worse?
Have you considered that their situation may be more complicated than it appears, and that without appropriate treatment, they may be at risk for blindness, stroke, or aneurysm?
Polymyalgia rheumatica is among the most common types of inflammatory joint pain seen in older people. It is often accompanied by another condition, giant cell arteritis, which can lead to irreversible vision changes and worse. In this column, we’ll take a look at these two fairly common, poorly understood, potentially dangerous, but highly treatable conditions that affect older adults.

Definitions: What Are They?
Polymyalgia rheumatica (PMR): it’s a complicated name. Its etymology suggests that it means “multiple muscle pains and problems with flow of the humors,” although modern usage applies the word rheumatism to nonspecific joint problems. And the leading symptoms of PMR do indeed look like muscle pain—although, as we will see, the problems begin elsewhere.
Giant cell arteritis (GCA) refers to inflammation and abnormally large white blood cells found in the lining of affected medium and large arteries. It used to be called temporal arteritis because it was frequently found in the temporal artery, but tissue studies reveal it affects many other arteries as well.
What is the link between PMR and GCA? These two conditions are autoimmune problems, and they often occur together. Statistics vary, but most specialists agree that 15–20 percent of all people who have PMR also develop GCA, and of those who have GCA, it appears that about 50 percent of them have also had PMR. Further, studies of people with PMR often reveal the same subtle tissue changes that are seen with GCA, but they are asymptomatic. These correlations have led some researchers to propose that the two conditions exist on a continuum, with PMR on the mild end and GCA on the more severe end. However, it is important to point out that a person can experience one without the other, and it is possible that a person can have symptoms of both conditions simultaneously, or in reverse order.

Who is at Risk?
PMR and GCA have some extremely reliable demographic distributions. They are seen in Caucasians far more often than in other races, and their global incidence demonstrates a higher-than-average concentration of cases in Northern Europe, especially Scandinavia.
PMR and GCA are rare in people under 50. The average age at onset is about 72. Women develop these conditions about twice as frequently as men.
It is difficult to pin down how many people in this country have PMR or GCA. Some estimates suggest they are diagnosed about 47,000 times each year, and about 1.6 million people currently live with one or both of these challenges. Three features make these conditions a topic that massage therapists should know about:
• Early symptoms include shoulder and hip pain, and possible headaches—all of which may prompt affected people to seek massage.
• Both PMR and GCA respond well to medical intervention, so individuals should pursue this quickly (and the hoped-for benefits of massage may delay their seeking help from a primary care provider).
• GCA in particular is associated with some serious complications that appropriate treatment can prevent—a delay in treatment can lead to permanent damage.

Etiology: What Happens?
PMR and GCA have been painstakingly studied for many years, and we still don’t completely understand how these conditions come about. It is clear they both involve inappropriate immune system activity, perhaps in reaction to a common viral trigger. They appear to have a predictable chemical profile with consistently high levels of a pro-inflammatory cytokine called IL-6. Further, IL-6 levels appear to correlate with the severity of symptoms, and this finding may help in the development of more treatment options.

In PMR, the inappropriate immune system activity appears to begin in the synovia of the proximal limb joints: shoulders and hips, and sometimes the neck. Inflammation may affect the joint linings, bursae, and synovial sheaths in the area. The individual often interprets this as muscle pain and weakness, but strength tests and muscle biopsies are normal. And here is more good news: the inflammation of joint capsules in the shoulders and hips is non-erosive—no permanent damage accrues.
In rare cases, PMR may cause some swelling in distal tissues of the arms or legs, but this is the exception rather than the rule.
Untreated PMR may persist for many months, up to a couple of years. At that time, it may spontaneously resolve with no lasting consequences, but about one-fifth of people with PMR also develop GCA.

In GCA, we see the accumulation of abnormal white blood cells that proliferate in the lining of medium and large arteries. These are the “giant cells” found in tissue biopsies. We don’t know why this occurs, but the result of this type of vasculitis is a risk for obstruction of the artery, and tissues downstream of the damaged vessel may degenerate and die.
Arteries that branch off the carotid are the most commonly affected by GCA. This condition is not limited to the arteries that supply the head, however; it can also affect the subclavian artery, and it may weaken the ascending arch of the aorta.

Signs and Symptoms

Signs and symptoms of PMR look as if someone engaged in a lot of unusual activity one day, and then woke up the next feeling terribly stiff and sore. Pain centers in the lower neck, shoulders, and hips. It is worse after rest and in the morning, and it can make activities of daily living—including getting out of bed or standing up from using the toilet—extremely difficult.
About half of all patients report a sudden onset, but the other half experience a slower development of symptoms. Pain may begin on one side, but eventually becomes bilateral.
About one-third of all PMR patients also have unexplained fever, cough, sore throat, and other subtle signs that might suggest low-grade vasculitis. In some cases, pain spreads to affect the upper arms and thighs, and, in rare versions, patients may experience substantial swelling in the extremities, with pitting edema.

GCA is usually identified by long-lasting headache that is accompanied by scalp tenderness and jaw pain. Inflammation of the throat and the tongue are common. Low-grade fever, weight loss, and depression may also develop. And any visual changes—dizziness, difficulty focusing, double vision or cloudy vision, or any loss of balance or coordination—need to be referred to a doctor immediately: damage to the optic nerve is irreversible, and permanent blindness is a real possibility.
One final observation about PMR and GCA is that their onset appears to occur most often in the spring and summer, and falls off in the autumn and winter.

This is not universally accurate, but a client who reports these symptoms in the spring or summer, and who doesn’t get relief from massage, is a good person to refer to a primary care physician.
See the “Diagnostic Criteria for PMR and GCA,” page 37 for more details.

Comorbidities, Complications, and Differentials
PMR and GCA are conditions with a well-recognized etiology and a predictably positive response to treatment with steroidal anti-inflammatories. But they can resemble many other conditions, and they can be comorbid with other conditions as well. And to have a successful outcome, other confusing overlapping problems must be ruled out, or identified and treated separately. This allows for treatment before complications develop, which is important.

Consider a female client who is Caucasian and middle-aged. Can you think of conditions that are common in this population that present with achy joints and/or headaches? Of course you can: the short list includes osteoporosis, osteoarthritis, fibromyalgia, hypothyroidism, and depression, among others—any of which can be confused with or comorbid with PMR and GCA. The challenge is that treatment strategies for these overlapping conditions are very different, and unless all the issues are addressed, the person is likely to be stuck in a painful state.

Sometimes complications of diseases are also their signs and symptoms. This is the case for GCA, which, as we’ve discussed, can lead to headaches and scalp pain; jaw, throat, and tongue inflammation; low fever; and general malaise. GCA is also associated with distal edema and Raynaud’s phenomenon (a condition that is discussed in the March/April 2017 edition of Pathology Perspectives “Raynaud’s Syndrome,” page 40).
But in addition to these, GCA has some potentially disabling complications. It can lead to blindness if the nerves that supply the eye are deprived of blood flow, and this vision loss is permanent. Other obstructions in cranial arteries might lead to mild or severe stroke. And weakness in arterial walls can cause the inner layers of the ascending thoracic aorta to split: this is called a dissecting aneurysm. This allows blood between layers to accumulate and thicken, and when fragments break loose, they can cause ischemic damage wherever they land. Increased mortality due to cardiovascular events is a known consequence of GCA.

Differential Diagnoses
A few conditions can present similarly to PMR or GCA, and they may only be found when the typical treatments (low or high doses of steroidal anti-inflammatories) don’t work well. Among the conditions that may be revealed this way, we find hypothyroidism, amyloidosis, rheumatoid arthritis, and various types of cancer.

Implications for Massage Therapy
PMR affects up to 1.6 million people in the United States, and it rises in frequency as people age. It usually presents like a musculoskeletal problem—it could easily be mistaken for signs of arthritis, muscle fatigue, fibromyalgia, headache, or several other common complaints for which people seek massage. It is entirely likely that a senior client may come to a trusted massage therapist, complaining of new shoulder or hip pain: “I must have really overdone it at my job/in the garden/on the golf course/playing with grandkids,” and so on.
But this pain is unlikely to be addressed successfully with massage therapy or bodywork. Indeed, massage may make this worse, and that is an important piece of information. If the joint-related aches and pains don’t improve with massage, and if they continue to worsen—especially if they begin to include headaches, scalp pain, jaw or tongue pain, or vision changes—it is absolutely vital that the person see a specialist as soon as possible. Any further delay may lead to irreversible changes in vision, and a substantial risk of stroke, aneurysm, or other complications.
What if a person has already been diagnosed and is currently treating their condition? Clients who have been diagnosed with either PMR or GCA are likely to take prednisone or prednisolone to manage their symptoms, and steroidal anti-inflammatory use presents further concerns for massage therapists. These drugs are extremely effective for the management of both conditions, but they are typically used for several months, or maybe years. The side effects of prednisone and prednisolone involve issues that impact bodywork choices, including easy bruising and an increased risk of diabetes and osteoporosis. See “Consequences of Long-Term Steroid Use” for more on this topic. This doesn’t mean we can’t work with clients who are in treatment for PMR or GCA, but we do have to make appropriate adjustments.
Do you have clients who are over 50? Do they ever ask for your help with shoulder or hip pain or headaches—especially with new and worsening symptoms? If the answers are yes, then PMR and GCA need to be on your radar—and now they are.

Consequences of Long-Term Steroid Use
Steroids work
PMR and GCA respond so well to oral corticosteroids (specifically prednisone or prednisolone) that this treatment is often used as a diagnostic confirmation. Left untreated, PMR-related pain may persist for months or years, and GCA can lead to life-threatening complications. But with appropriate steroid use, the period of pain and risk can be reduced to a matter of days; these are truly miraculous drugs for this situation.

But there’s a catch
A person with PMR or CGA is typically treated with steroids for many months, and sometimes years. Primary care physicians must slowly taper off doses to allow the body to return to full function—this may take a long time. And the longer steroid treatment lasts, the higher the risk for adverse events.

Here is a short list of adverse events associated with steroidal anti-inflammatories that are particularly relevant to massage therapists:
Increased bruising and slowed wound healing
Myopathy (muscle wasting)
Bone thinning and vertebral collapse (especially since most patients are middle-aged and elderly Caucasian women—exactly the same group most at risk for osteoporosis)
Avascular necrosis (the head of the femur degenerates due to poor blood supply)
Steroid-induced diabetes
High blood pressure
Extreme mood swings
Increased vulnerability to infections

How do massage therapists make appropriate accommodations for these risks? It is impossible to fully catalog all the possibilities. But let’s be sure that when we gather information, we find out both what medications our clients use and what side effects those medications may be causing. Then, we can be sure to offer the pressure, positioning, and type of massage therapy that is most likely to magnify the benefits of our work, while minimizing the risks.

Ruth Werner, BCTMB, is a former massage therapist, a writer, and an NCBTMB-approved provider of continuing education. She wrote A Massage Therapist’s Guide to Pathology (Lippincott Williams & Wilkins, 2016), now in its sixth edition, which is used in massage schools worldwide. Werner is available at www.ruthwerner.com.