Patient education: Polymyalgia rheumatica and giant cell arteritis (Beyond the Basics)

INTRODUCTION — Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA, also known as Horton disease, cranial arteritis, and temporal arteritis) are inflammatory conditions that affect different parts of the body. They are frequently discussed together because:

●PMR occurs in approximately 40 to 50 percent of people with GCA

●GCA occurs in approximately 10 to 20 percent of people with PMR

The two disorders do not necessarily occur at the same time.

More detailed information about PMR and GCA is available by subscription. (See 'Professional level information' below.)

POLYMYALGIA RHEUMATICA — The most common symptoms of polymyalgia rheumatica (PMR) are:

●Aching and stiffness in the morning in the shoulders and upper arms, hips and thighs, and back of the neck. Some people have trouble getting dressed (eg, fastening a bra, putting on a shirt or jacket, or pulling on stockings). Difficulty raising the arms above the shoulders is a typical symptom. Morning stiffness is often severe, can last for several hours, and can return during the day after inactivity.

●Occasional swelling or stiffness of the small joints of the hands, wrists, and knees.

●Feeling of tiredness, poor appetite, weight loss, or low-grade fever.

Symptoms of PMR come on quickly over a period of weeks or even days.

Polymyalgia rheumatica diagnosis — A combination of approaches is used to diagnose PMR, including a history, physical exam, and lab tests. Imaging tests are not required to diagnose PMR. However, in selected cases, ultrasound or magnetic resonance imaging (MRI) of the joints may be ordered by your provider to support the diagnosis.

Most people who are diagnosed with PMR:

●Are older adults in their 60s or 70s. PMR almost never occurs under the age of 50.

●Have persisting aching and morning stiffness (lasting 30 minutes or more) on both sides of the body, and involving at least two of the following three areas: neck or upper body, shoulders or upper arms, and hips or upper thighs.

●Have an elevated level of inflammation, which can be measured with a blood test (the erythrocyte sedimentation rate [ESR] or C-reactive protein [CRP]).

Polymyalgia rheumatica treatment — PMR responds quickly to low doses of glucocorticoids (also called steroids), medications that reduce inflammation. Prednisone and prednisolone are two commonly used glucocorticoids. Nonsteroidal antiinflammatory drugs (NSAIDs; such as ibuprofen or naproxen) are ineffective.

During your treatment, it is very important to call your healthcare team if you develop any new symptoms that could be caused by giant cell arteritis (GCA), such as headaches, change in vision, or pain in the jaw with chewing. (See 'Giant cell arteritis symptoms' below.)

Glucocorticoids — Most people feel better after the first few doses of a glucocorticoid. Your provider will gradually decrease the dose every two to four weeks to find the minimum dose that is needed to prevent symptoms. Most people need to take glucocorticoids for several months or up to one to two years.

Glucocorticoids can have serious side effects when taken for long periods of time. Side effects can include weight gain, weakening of the bones, and diabetes. Taking the lowest possible dose for the shortest possible time can help to reduce the chance of these side effects.

As the dose of the glucocorticoid is reduced, stiffness and pain can recur. Decreasing the dose slowly can minimize these symptoms.

GIANT CELL ARTERITIS — Giant cell arteritis, also called GCA or temporal arteritis, is a type of blood vessel inflammation that damages medium- and large-sized arteries. The most commonly affected arteries are the aorta and its major branches, particularly those that start in the neck and travel into the head and scalp. It is a disease of older adults, often occurring between the ages of 60 to 80, while it never occurs under the age of 50.

How are polymyalgia rheumatica and giant cell arteritis related? — Almost one-half of people with GCA have polymyalgia rheumatica (PMR). Up to 20 percent of people with PMR have GCA. It is not clear why the conditions often occur together.

Giant cell arteritis symptoms — The most common symptoms include new headaches, jaw pain with chewing. Temporary or permanent loss of vision can be an early sign of GCA.

Among other symptoms that can occur are fever (usually less than 101ºF or 38ºC), fatigue, weight loss, a new cough, and unexplained dental or facial pain.

●Headache – The pain, which may be mild or severe, tends to be located over the temples but can be anywhere in the front or back of the skull (figure 1). Some people also notice tenderness in the scalp.

●Jaw pain (claudication) – Up to one-half of people with GCA experience jaw pain, which develops while chewing food. This symptom occurs because of inadequate blood flow to the muscles involved in chewing.

●Vision loss – Up to 10 percent of people with GCA can develop partial or complete blindness. Temporary loss of vision can be the first sign of GCA. If untreated, loss of vision can be permanent and/or can affect the other eye.

Giant cell arteritis diagnosis — If you are an adult over the age of 50 years and you have some of the symptoms listed above, your provider will order tests for GCA. They will perform an examination and will order some blood tests, especially tests for inflammation, ie, the erythrocyte sedimentation rate (ESR or "sed rate") and C-reactive protein (CRP). If GCA is suspected, the diagnosis must be confirmed, either by biopsy of the temporal artery (figure 1) or with imaging tests (ultrasound, CT, MRI, or positron emission tomography [PET] scanning).

Giant cell arteritis treatment

Glucocorticoids — High doses of glucocorticoids (steroids) are used for the management of GCA because such treatment controls symptoms and, if started promptly, will prevent loss of vision. If GCA is strongly suspected, treatment may be started before the diagnosis has been confirmed. If there is no evidence of arteritis on biopsy or on ultrasound and other imaging studies, and if your doctor is not convinced you have GCA, they will recommend stopping glucocorticoids (see "Treatment of giant cell arteritis"). Occasionally, glucocorticoid treatment is continued, even if there is no confirmation of arteritis, especially if your doctor strongly suspects that you have GCA.

Many people continue taking glucocorticoids for one to two years, although some people require an even longer course of treatment. The dose of glucocorticoids is tapered slowly and carefully so that symptoms of GCA do not recur. Loss of vision after an initial course of high-dose glucocorticoids is very unusual.

Side effects from glucocorticoids in higher doses can occur. These include weight gain, diabetes, osteoporosis (bone loss), hypertension, and others. During the follow-up of GCA, your doctor will monitor you for these potential problems.

Other treatments — Other treatments for GCA are often recommended to allow for more rapid tapering of glucocorticoids; these are sometimes called "steroid-sparing medications." Medications such as tocilizumab or methotrexate may be given in combination with glucocorticoids, as only glucocorticoids have been shown to be effective for the prevention of loss of vision. Incorporating these other medications can allow for glucocorticoids to be tapered (reduced) more quickly, which can help prevent or decrease glucocorticoid-related side effects.

LONG-TERM OUTLOOK

Polymyalgia rheumatica — In most people, polymyalgia rheumatica (PMR) tends to run its course over time, and glucocorticoid therapy can eventually be stopped.

Giant cell arteritis — Giant cell arteritis (GCA) also tends to run its course, though occasionally headache and other symptoms can reappear during the first few months after it has been diagnosed. But it is important to remember that if vision is intact when GCA is diagnosed, and if higher-dose, daily glucocorticoid treatment is started immediately, the risk of future vision loss is very small. The dose of glucocorticoids can eventually be reduced and then eventually stopped in most patients.

Thoracic aortic aneurysm is a rare and generally late complication of GCA, usually occurring three to five years after diagnosis. It occurs when the aorta, the main artery that carries blood from the heart, develops a weak spot and bulges outward. Aneurysms do not cause symptoms but can be life-threatening if they burst. People with GCA are sometimes tested at diagnosis and during follow-up for this type of aneurysm with a CT scan of the chest or with other imaging tests.

WHERE TO GET MORE INFORMATION — Your healthcare provider is the best source of information for questions and concerns related to your medical problem.

This article will be updated as needed on our web site (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for healthcare professionals, are also available. Some of the most relevant are listed below.

Patient level information — UpToDate offers two types of patient education materials.

The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.

Patient education: Polymyalgia rheumatica and giant cell arteritis (The Basics)

Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.

This topic currently has no corresponding Beyond the Basics content.

Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.

Clinical manifestations and diagnosis of polymyalgia rheumatica
Clinical manifestations of giant cell arteritis
Diagnosis of giant cell arteritis
Treatment of giant cell arteritis
Treatment of polymyalgia rheumatica

The following organizations also provide reliable health information.

●American College of Rheumatology

(www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Polymyalgia-Rheumatica)

(www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Giant-Cell-Arteritis)

●National Library of Medicine

(https://medlineplus.gov/polymyalgiarheumatica.html)

●Vasculitis Foundation

(https://www.vasculitisfoundation.org/)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges William Docken, MD, who contributed to earlier versions of this topic review.