Polymyalgia rheumatica
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Polymyalgia rheumatica (PMR) is a syndrome experienced as pain or stiffness, usually in the neck,
shoulders The human shoulder is made up of three bones: the clavicle (collarbone), the scapula (shoulder blade), and the humerus (upper arm bone) as well as associated muscles, ligaments and tendons. The articulations between the bones of the shoulder mak ...
, upper arms, and
hips In vertebrate anatomy, hip (or "coxa"Latin ''coxa'' was used by Celsus in the sense "hip", but by Pliny the Elder in the sense "hip bone" (Diab, p 77) in medical terminology) refers to either an anatomical region or a joint. The hip region ...
, but which may occur all over the body. The pain can be sudden or can occur gradually over a period. Most people with PMR wake up in the morning with pain in their
muscles Skeletal muscles (commonly referred to as muscles) are organs of the vertebrate muscular system and typically are attached by tendons to bones of a skeleton. The muscle cells of skeletal muscles are much longer than in the other types of musc ...
; however, cases have occurred in which the person has developed the pain during the evenings or has pain and stiffness all day long. People who have polymyalgia rheumatica may also have
temporal arteritis Temporal may refer to: Entertainment * Temporal (band), an Australian metal band * ''Temporal'' (Radio Tarifa album), 1997 * ''Temporal'' (Love Spirals Downwards album), 2000 * ''Temporal'' (Isis album), 2012 * ''Temporal'' (video game), a 200 ...
(
giant cell arteritis Giant cell arteritis (GCA), also called temporal arteritis, is an inflammatory autoimmune disease of large blood vessels. Symptoms may include headache, pain over the temples, flu-like symptoms, double vision, and difficulty opening the mouth. ...
), an inflammation of blood vessels in the face which can cause blindness if not treated quickly. The pain and stiffness can result in a lowered quality of life, and can lead to depression. It is thought to be brought on by a viral or bacterial illness or trauma of some kind, but genetics play a role as well. Persons of
Northern European The northern region of Europe has several definitions. A restrictive definition may describe Northern Europe as being roughly north of the southern coast of the Baltic Sea, which is about 54°N, or may be based on other geographical factors ...
descent are at greater risk. There is no definitive laboratory test, but
C-reactive protein C-reactive protein (CRP) is an annular (ring-shaped) pentameric protein found in blood plasma, whose circulating concentrations rise in response to inflammation. It is an acute-phase protein of hepatic origin that increases following interleukin- ...
(CRP) and
erythrocyte sedimentation rate The erythrocyte sedimentation rate (ESR or sed rate) is the rate at which red blood cells in anticoagulated whole blood descend in a standardized tube over a period of one hour. It is a common hematology test, and is a non-specific measure of ...
(ESR) can be useful. PMR is usually treated with corticosteroids taken by mouth. Most people need to continue the corticosteroid treatment for two to three years. PMR sometimes goes away on its own in a year or two, but medications and self-care measures can improve the rate of recovery. PMR was first established as a distinct disease in 1966 by a case report on 11 patients at Mount Sinai Hospital in New York, NY. It takes its name from the
Greek Greek may refer to: Greece Anything of, from, or related to Greece, a country in Southern Europe: *Greeks, an ethnic group. *Greek language, a branch of the Indo-European language family. **Proto-Greek language, the assumed last common ancestor ...
word Πολυμυαλγία ''polymyalgia'', which means "pain in many muscles".


Signs and symptoms

A wide range of symptoms can indicate if a person has polymyalgia rheumatica. The classic symptoms include: * Pain and stiffness (moderate to severe) in the neck, shoulders, upper arms, thighs, and hips, which inhibits activity, especially in the morning/after sleeping. Pain can also occur in the groin area and in the buttocks. The pain can be limited to one of these areas as well. It is a disease of the "girdles" meaning shoulder girdle or pelvic girdle. * Fatigue and lack of appetite (possibly leading to weight loss) *
Anemia Anemia or anaemia (British English) is a blood disorder in which the blood has a reduced ability to carry oxygen due to a lower than normal number of red blood cells, or a reduction in the amount of hemoglobin. When anemia comes on slowly, t ...
* An overall feeling of illness or flu-like symptoms. * Low-grade (mild) fever or abnormal temperature is sometimes present. * In most people, it is characterized by constant fatigue, weakness and sometimes exhaustion. About 15% of people who are diagnosed with polymyalgia rheumatica also have
temporal arteritis Temporal may refer to: Entertainment * Temporal (band), an Australian metal band * ''Temporal'' (Radio Tarifa album), 1997 * ''Temporal'' (Love Spirals Downwards album), 2000 * ''Temporal'' (Isis album), 2012 * ''Temporal'' (video game), a 200 ...
, and about 50% of people with temporal arteritis have polymyalgia rheumatica. Some symptoms of temporal arteritis include
headaches Headache is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of depression in those with severe headaches. Headaches can occur as a result ...
, scalp tenderness,
jaw The jaw is any opposable articulated structure at the entrance of the mouth, typically used for grasping and manipulating food. The term ''jaws'' is also broadly applied to the whole of the structures constituting the vault of the mouth and serv ...
or facial soreness,
distorted vision Distorted may refer to: * Anything subject to distortion * Distorted (band), a progressive deathdoom metal band from Bat-Yam, Israel * ''Distorted'' (EP), an extended play by the band Distorted * ''Distorted'' (film) (2018) * Distorted Music Fest ...
, or aching in the limbs caused by decreased blood flow, and fatigue.


Causes

The cause of PMR is not well understood. The pain and stiffness result from the activity of inflammatory cells and proteins that are normally a part of the body's disease-fighting immune system, and the inflammatory activity seems to be concentrated in tissues surrounding the affected joints. During this disorder, the white blood cells in the body attack the lining of the joints, causing inflammation. Inherited factors also play a role in the probability that an individual will develop PMR. Several theories have included viral stimulation of the
immune system The immune system is a network of biological processes that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to parasitic worms, as well as cancer cells and objects such as wood splint ...
in genetically susceptible individuals. Infectious diseases may be a contributing factor. This would be expected with sudden onset of symptoms, for example. In addition, new cases often appear in cycles in the general population, implying a viral connection. Studies are inconclusive, but several somewhat common viruses were identified as possible triggers for PMR. The
viruses A virus is a submicroscopic infectious agent that replicates only inside the living cells of an organism. Viruses infect all life forms, from animals and plants to microorganisms, including bacteria and archaea. Since Dmitri Ivanovsky's ...
thought to be involved include the adenovirus, which causes respiratory infections; the human parvovirus B19, an infection that affects children; and the
human parainfluenza virus Human parainfluenza viruses (HPIVs) are the viruses that cause human parainfluenza. HPIVs are a paraphyletic group of four distinct single-stranded RNA viruses belonging to the ''Paramyxoviridae'' family. These viruses are closely associated wit ...
. Some patients attribute the onset of PMR to stress. Persons having the
HLA-DR4 HLA-DR4 (DR4) is an HLA- DR serotype that recognizes the DRB1*04 gene products. The DR4 serogroup is large and has a number of moderate frequency alleles spread over large regions of the world. Serology The serological identification of DR4 is ...
type of
human leucocyte antigen The human leukocyte antigen (HLA) system or complex is a complex of genes on chromosome 6 in humans which encode cell-surface proteins responsible for the regulation of the immune system. The HLA system is also known as the human version of t ...
appear to have a higher risk of PMR.Page 255 in:


Diagnosis

No specific test exists to diagnose polymyalgia rheumatica; many other diseases can cause inflammation and pain in muscles, but a few tests can help narrow down the cause of the pain. Limitation in shoulder motion or swelling of the joints in the wrists or hands, are noted by the doctor. A patient's answers to questions, a general physical exam, and the results of tests can help a doctor determine the cause of pain and stiffness. One blood test usually performed is the
erythrocyte sedimentation rate The erythrocyte sedimentation rate (ESR or sed rate) is the rate at which red blood cells in anticoagulated whole blood descend in a standardized tube over a period of one hour. It is a common hematology test, and is a non-specific measure of ...
(ESR) which measures how fast the patient's red blood cells settle in a test tube. The slower the red blood cells settle, the higher the ESR value (measured in mm/hour), which means inflammation is present. Many conditions can cause an elevated ESR, so this test alone is not proof that a person has polymyalgia rheumatica. Another test that checks the level of
C-reactive protein C-reactive protein (CRP) is an annular (ring-shaped) pentameric protein found in blood plasma, whose circulating concentrations rise in response to inflammation. It is an acute-phase protein of hepatic origin that increases following interleukin- ...
(CRP) in the blood may also be conducted. CRP is produced by the liver in response to an injury or infection, and people with polymyalgia rheumatica usually have high levels. However, like the ESR, this test is also not very specific. Polymyalgia rheumatica is sometimes associated with temporal arteritis, a condition requiring more aggressive therapy. To test for this additional disorder, a
biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a dise ...
sample may be taken from the temporal artery.


Treatment

Prednisone Prednisone is a glucocorticoid medication mostly used to suppress the immune system and decrease inflammation in conditions such as asthma, COPD, and rheumatologic diseases. It is also used to treat high blood calcium due to cancer and ad ...
is the drug of choice for PMR, and treatment duration is frequently greater than one year. If the patient does not experience dramatic improvement after three days of 10–20 mg oral prednisone per day, the diagnosis should be reconsidered. Sometimes relief of symptoms occurs in only several hours. Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen are ineffective in the initial treatment of PMR, but they may be used in conjunction with the maintenance dose of corticosteroid. Along with medical treatment, patients are encouraged to exercise and eat healthily, helping to maintain a strong immune system and build strong muscles and bones. A diet of fruits, vegetables, whole grains, and low-fat meat and dairy products, avoiding foods with high levels of refined sugars and salt is recommended. Research in the UK has also suggested that people with polymyalgia rheumatica would benefit from a falls assessment when first diagnosed, and regular treatment reviews.


Epidemiology

No circumstances are certain as to which an individual will get polymyalgia rheumatica, but a few factors show a relationship with the disorder: * Usually, PMR only affects adults over the age of 50. * The average age of a person who has PMR is about 70 years old. * Women are twice as likely to get PMR as men. * Caucasians are more likely to get this disease. It is more likely to affect people of Northern European origin; Scandinavians are especially vulnerable. * About 50% of people with temporal arteritis also have polymyalgia rheumatica.


See also

*
Remitting seronegative symmetrical synovitis with pitting edema Remitting seronegative symmetrical synovitis with pitting edema (or sometimes RS3PE) is a rare syndrome identified by symmetric polyarthritis, synovitis, acute pitting edema (swelling) of the back of the hands and/or feet, and a negative serum r ...
*
Tocilizumab Tocilizumab, sold under the brand name Actemra among others, is an immunosuppressive drug, used for the treatment of rheumatoid arthritis, systemic juvenile idiopathic arthritis, a severe form of arthritis in children, and COVID19. It is a huma ...


References


External links


Questions and Answers about Polymyalgia Rheumatica and Giant Cell Arteritis
nbsp;- US National Institute of Arthritis and Musculoskeletal and Skin Diseases {{Soft tissue disorders Steroid-responsive inflammatory conditions Systemic connective tissue disorders