Polymyalgia rheumatica
| Polymyalgia rheumatica | |
| ICD-10 | M35.3 |
|---|---|
| ICD-9 | 725 |
| MedlinePlus | 000415 |
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Overview
Polymyalgia rheumatica (PMR) - (in Greek meaning “pain in many muscles”) is an inflammatory condition of the muscles, which causes pain or stiffness, usually in the neck, shoulders, and hips. The pain can be very sudden, or can occur gradually over a period of time.
Most PMR sufferers wake up in the morning with pain in their muscles; however, there have been cases in which the patient has developed the pain during the evenings.[1] Along with this disorder, there have been reports that patients who have polymyalgia rheumatica also have temporal arteritis, which causes inflammation that damages large and medium size arteries. PMR usually goes away within a year or two after treatment.[2]
Causes
The cause of this disorder is unknown; however, studies have shown that during this disorder, the white blood cells in the body attack the lining of muscle joints, causing inflammation.[2] Recent studies have found that inherited factors also play a role in the probability that an individual will become sick with polymyalgia rheumatica. Several theories have included viral stimulation of the immune system in genetically susceptible individuals.[3]
Manifestation of gaintcell arteritis as polymyalgia rheumatica is associated with the increased frequency of HLA DR4.[4].PMR and GCA also share the associated sequence polymorphism encoded by the second hypervariable region(HVR) of the HLA DRB1 gene unlike rheumatoid arthritis in which the sequence is encoded by third HVR.[5]. Isolated PMR has positive association with TNFb3, independent of HLA DRB1 association.[6]
Several viruses are thought to be linked to polymyalgia rheumatica,including the adenovirus, which causes respiratory infections; the human parvovirus B19, an infection that affects children; and the human parainfluenza virus..[2]
Differential Diagnosis
- Rheumatoid arthritis - Polymyalgia rheumatica and late onset rheumatoid arthritis can initially present with similar clincal features like synovitis. These patients are treated initially as PMR with gluococorticoids. RA treatment is started when there is no improvement or when it evolves into charecteristic RA or when there is a persistently raised plasma viscosity.[7] [8]
- Polymyositis and Dermatomyositis - Patients with dermatomyositis or polymyositis present with tenderness and weakness of proximal muscles, while PMR patients present with pain and stiffness prominently. This differentiation may be difficult in elderly patients. Proper history,complete physical examination, ESR,creatine kinase levels and muscle biopsy help in establishing proper diagnosis.[9] [10]
- Malignancy (myeloma, others) - Patients with malignancy sometimes present with PMR like symptoms and have poor response to steroid therapy[11]. This is in fact paraneoplastic syndrome presenting as PMR.[12]
- Fibromyalgia - Fibromyalgia is commonly presented in age groups 20-50 years and patients have characteristic tender points. The active phase protiens and ESR are normal unlike PMR.
- Hyperparathyroidism - Hyperparathyroidism presents with proximal stiffness and bone pain with elevated parathyroid hormone levels and often calcium levels without elevation of ESR levels.
- Chronic infection (subacute bacterial endocarditis (SBE)) - Rheumatologic symptoms seen in infective endocarditis can present a clinical picture suggesting polymyalgia rheumatica hindering the correct diagnosis.[13]
- Hypothyroidism - These patients have signs like muscle and joint pain and weakness similar to PMR. Delayed relaxation of deep tendon reflexes is seen in hypothyroidism with elevated TSH levels and low T4 levels.
- Remitting seronegative symmetrical synovitis with pitting edema - RS3PE presents with symmetrical synovitis and pitting edema,usually in patients over 50 years of age and lack rheumatoid factor. The symptoms are commonly manifested distally unlike PMR.
Epidemiology and Demographics
- Mean age at onset ~ 70 (range 50-90)
- F:M ratio = 2:1
Risk Factors
There are no certain circumstances for which an individual will get polymyalgia rheumatica, but there are a few factors that show a relationship with the disorder.
- Usually affects adults over the age of 50[1]
- The average age of a person who has PMR is about 70 years old[1]
- Women are twice as likely to get PMR as men[1]
- The vast majority of people affected are white[1]
- 50% of people with temporal arteritis also have polymyalgia rheumatica[1]
- Smoking, sun exposure, infections, nulliparity are also considered risk factors.[14]
History and Symptoms
There are a wide range of symptoms that indicate if a person has polymyalgia rheumatica. The symptoms include pain and stiffness in the muscles, usually in the neck, shoulders, and hips. The pain is moderate to severe, and may inhibit the activity of the person. These symptoms usually occur in the morning, or after sleeping. Fatigue and lack of appetite are also signs of polymyalgia rheumatica. Lack of appetite could lead to unintentional weight loss. Anemia is another sign of polymyalgia rheumatica. An overall feeling of illness and a slight fever are also signs of this disorder.[2]
Studies have shown that about 15% of people who are diagnosed with polymyalgia rheumatica also have temporal arteritis, and about 50% of people with temporal arteritis have polymyalgia rheumatica. Some symptoms of temporal arteritis include severeheadaches, scalp tenderness, jaw or facial soreness, distorted vision or aching in the limbs caused by decreased blood flow, and fatigue.[1]
- Symmetric aching/stiffness of axial & proximal limb musculature
- Shoulder girdle, neck, hip girdle
- Morning stiffness, gelling
- Muscle strength intact, though may be limited by pain
- Synovitis in knees, wrists, sterno-clavicular (SC) joints, hips/shoulders
- Distal extremity swelling (tenosynovitis)
- Malaise, fatigue, weight loss in > 50%
Relation to Giant Cell Arteritis (GCA)
- 25% of patients with GCA have PMR as presenting symptom
- 40-60% of patients with GCA have PMR during disease course
- 10-15% of patients with PMR have GCA by temporal artery (TA) biopsy
| Symptoms GCA | Signs GCA | ||
| Headache | 68% | Decreased TA pulsations | 46% |
| Jaw claudication | 45% | Fever | 42% |
| Transient visual symptoms | 16% | Tenderness over temporal artery | 27% |
| Fixed visual loss | 14% | Nodular or swollen scalp arteries | 23% |
| Dysphagia | 8% | Large artery bruits | 21% |
| Tongue claudication | 6% | Ophthalmoscopic abnormalities | 18% |
| Visual loss | 14% |
Diagnosis
There is no specific test to diagnose polymyalgia rheumatica. There are many other diseases which cause inflammation and pain in muscles, but there are a few tests that 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.[3] The doctor will assess the patient’s pain, and may perform one of the following tests to determine if polymyalgia rheumatica is indeed the cause of the pain.[2]
One test that is usually performed is the erythrocyte sedimentation rate test (also known as the ESR or SED rate), which is a blood test that checks the erythrocyte sedimentation rate. This test measures how fast the patient’s red blood cells settle in a test tube. The faster the blood cells settle, the higher the SED rate, which means that there is inflammation. Because many conditions can cause an elevated SED rate, this test alone is not a good determinant if a person has with polymyalgia rheumatica.[2]
Another test that checks the level of C-reactive protein in the blood may also be conducted. C-reactive protein is produced by the liver in response to an injury or infection. People with polymyalgia rheumatica usually have high levels of C-reactive protein, which is a response due to inflammation.[2]
Because people with polymyalgia rheumatica are sometimes associated with temporal arteritis, doctors may perform a test to determine if a person has temporal arteritis. A sample from the scalp artery in the temple is taken and examined under a microscope. If a person is positive for temporal arteritis, the doctor may prescribe a medicine that treats both polymyalgia rheumatica and temporal arteritis.[2]
As a summary;
- Age ≥ 50 at onset
- Symtoms in ≥ 2/3 areas (shoulder girdle, hip girdle, neck) x ≥ 1 month
- Erythrocyte sedimentation rate (ESR) ≥ 40 mm/hr
- Exclusion of alternative diagnosis
2012 provisional criteria for Polymyalgia rheumatica by EULAR/ACR : patient >50 years old presenting with bilateral shoulder pain, not explained by an alternative pathology can be classified as PMR in the presence of morning stiffness >45 minutes, elevated CRP and/or ESR and new hip pain.[15]
Laboratory Findings
- Mild normochromic, normocytic anemia (during active phase)
- White blood cell (WBC) normal, platelets often increased
- Markedly elevated ESR
- Antinuclear antibody (ANA), rheumatoid factor (RF) usually negative
- Elevation of alkaline phosphatase of liver origin is seen in one third to half of patients with PMR associated with GCA.[16]
- Creatine kinase (CK), aldolase, electromyogram (EMG), muscle biopsy all normal
Other Diagnostic Studies
Temporal Artery Biopsy
- Not indicated if no symptoms suggestive of GCA
- Negative predictors of GCA
- Age < 70
- Absence of headache or jaw claudication
- Clinically normal temporal arteries
- 3 negative predictors--1.7% risk GCA
- If ≤ 2 negative predictors--55% risk GCA
- Can still demonstrates arteritis after 2-4 weeks of steroid treatment
Ultrasound and MRI
- Ultrasonography is effective in confirming bilateral subacromial and subdeltoid bursitis in PMR patients.[17]
- MRI is used in the assessment of hip synovitis and iliopsoas bursitis. It is sensitive than [[ultrasonography].[18]
- MRI and Ultrasound of the shoulder facilitate the proper diagnosis in patients with the typical proximal symptoms of PMR with normal ESR values.[19]
- Ultrasound is useful in monitoring the patients' response to the corticosteroid treatment and detects any subclinical inflammation.[20].Power Doppler ultrasound is useful in detecting patients with high inflammation who have a high risk of relapses or recurrences.[21]
PET Scan
PET scan has no proven clinical value in patient care. 18-fluorodeoxyglucose accumulation in the blood vessels suggests arteritis,mostly in the large vessels. Region of index analysis is sensitive tool in detecting such inflammation.[22]FDG focal uptake is seen in ligament inflammation.[23]
Treatment and Medication
Anti-inflammatory medicine such as aspirin or ibuprofen is typically prescribed to treat mild cases of polymyalgia rheumatica. For more serious cases, with extreme pain and discomfort, steroids are prescribed to patients. Prednisone is the typical steroid used to treat polymyalgia rheumatica. The steroids are normally distributed in low doses (10-15 mg per day), and results are usually seen within the first few days of taking the medication.[24] The patient's SED rate is monitored throughout the medication process, and other blood tests are conducted to make sure the patient does not experience any side effects from the treatment. Once the SED rate is back to normal, the patient will receive lower doses of the steroids in order to avoid any long term health effects from the steroids.[2] Intramuscular methylprednisolone(40-120 mg)has similar effects like oral corticosteroids and the cumulative steroid dose is also less.[25]
Some side effects from the steroids may occur. Studies have shown that steroids increase the patient’s blood pressure. For this reason, the patient’s blood pressure is monitored throughout the treatment process. Also, the steroids lower the patient’s immune system, making them more susceptible to infection. The doctor should be notified of any signs of sickness.[2] Methylprednisolone has better side effect profile with respect to fracture rate and weight gain.[26] Prophylaxis for osteoporosis with calcium and vitamin D should be started along with steroid therapy.
Along with medical treatment, patients can increase their chances of recovery by exercising and eating healthy foods. Exercise will help strengthen the weak muscles, and help to prevent weight gain. A healthy diet will help to keep a strong immune system, and also help build strong muscles and bones.[2]
Treatment lasts as long as needed; however, it normally takes patients several years to get off of the steroids. The symptoms may come back when the dosage is lowered.[3]
Acute Pharmacotherapies
- NSAIDs helpful in mild disease
- Glucocorticoids if no response to non-steriod anti inflammatory drugs (NSAIDs)
- Prednisone 5-20 mg every day--rapid improvement in symptoms
- Start taper after symptoms remit and ESR returns to normal (2-4 weeks)
- Gradual dose reduction (eg, by 2.5 mg) q 1-4 weeks until dose = 5-10 mg every day
- Even slower taper once dose < 5-10 mg (reduce dose by 1 mg q month)
- Monitor for relapse--occurs in 25-50%, usually because taper too rapid
- Careful observation for signs of arteritis
Other therapies
Glucocorticoids are considered the most effective medication for polymyalgia rheumatica. Other medications are considered in steroid resistant cases i.e no good response with 20mg/day prednisone, atypical cases and in patients at high risk for steroid related toxicity.
- Methotrexate is the commonly used steroid sparing agent.Prednisone plus methotrexate treatment is associated with shorter prednisone treatment.[27]
- Infliximab use in PMR has not been proved beneficial and it may be harmful.[28]
- Etanercept may be safe and useful in relapsing PMR. It is modestly effective in PMR associated with giantcell arteritis than in isolated PMR
EULAR response criteria for PMR comprise a set of core markers for monitoring therapeutic response which include :
- Visual analogue scale of patient's pain
- Ability to elevate the upper limbs.
A disease activity score <7 indicates low activity, 7-17 suggest medium disease activity, >17 indicates high disease activity.[29]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 http://arthritis.webmd.com/polymyalgia-rheumatica-temporal-arteritis Gelfand, Jonathan L MD. "Polymyalgia Rheumatica and Temporal Arteritis." WedMD. 17 Nov. 2007. Cleveland Clinic. 16 Mar. 2008
- ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441/DSECTION=1 "Polymyalgia Rhuematica." MayoClinic. 17 May 2006. 15 Mar. 2008
- ↑ 3.0 3.1 3.2 http://www.medicinenet.com/polymyalgia_rheumatica/article.htm Shiel, William C. MD, FACP, FACR. "Polymyalgia Rheumatica (PMR) & Giant Cell Arteritis (Temporal Arteritis)." MedicineNet. 3 Mar. 2008. 14 Mar. 2008
- ↑ Cid MC, Ercilla G, Vilaseca J; et al. (1988). "Polymyalgia rheumatica: a syndrome associated with HLA-DR4 antigen". Arthritis Rheum. 31 (5): 678–82. PMID 3259885. Unknown parameter
|month=ignored (help) - ↑ Weyand CM, Hunder NN, Hicok KC, Hunder GG, Goronzy JJ (1994). "HLA-DRB1 alleles in polymyalgia rheumatica, giant cell arteritis, and rheumatoid arthritis". Arthritis Rheum. 37 (4): 514–20. PMID 8147928. Unknown parameter
|month=ignored (help) - ↑ Mattey DL, Hajeer AH, Dababneh A; et al. (2000). "Association of giant cell arteritis and polymyalgia rheumatica with different tumor necrosis factor microsatellite polymorphisms". Arthritis Rheum. 43 (8): 1749–55. doi:10.1002/1529-0131(200008)43:8<1749::AID-ANR11>3.0.CO;2-K. PMID 10943865. Unknown parameter
|month=ignored (help) - ↑ Pease CT, Haugeberg G, Montague B; et al. (2009). "Polymyalgia rheumatica can be distinguished from late onset rheumatoid arthritis at baseline: results of a 5-yr prospective study". Rheumatology (Oxford). 48 (2): 123–7. doi:10.1093/rheumatology/ken343. PMID 18980958. Unknown parameter
|month=ignored (help) - ↑ Pease CT, Haugeberg G, Morgan AW, Montague B, Hensor EM, Bhakta BB (2005). "Diagnosing late onset rheumatoid arthritis, polymyalgia rheumatica, and temporal arteritis in patients presenting with polymyalgic symptoms. A prospective longterm evaluation". J. Rheumatol. 32 (6): 1043–6. PMID 15940765. Unknown parameter
|month=ignored (help) - ↑ Sørensen CD, Hansen LH, Hørslev-Petersen K (2010). "[Myositis as differential diagnosis in polymyalgia rheumatica]". Ugeskr. Laeg. (in Danish). 172 (42): 2899–900. PMID 21040663. Unknown parameter
|month=ignored (help) - ↑ Hopkinson ND, Shawe DJ, Gumpel JM (1991). "Polymyositis, not polymyalgia rheumatica". Ann. Rheum. Dis. 50 (5): 321–2. PMC 1004419. PMID 2042988. Unknown parameter
|month=ignored (help) - ↑ Manganelli P, Borghi L, Coruzzi P, Novarini A, Ambanelli U (1986). "[Paraneoplastic polymyalgia rheumatica. Case contribution]". Minerva Med. (in Italian). 77 (38): 1739–41. PMID 3774196. Unknown parameter
|month=ignored (help) - ↑ Kwiatkowska B, Filipowicz-Sosnowska A (2008). "[Polymyalgia rheumatica mimicking neoplastic disease--significant problem in elderly patients]". Pol. Arch. Med. Wewn. (in Polish). 118 Suppl: 47–9. PMID 19562970.
- ↑ Auzary C, Le Thi Huong D, Delarbre X; et al. (2006). "Subacute bacterial endocarditis presenting as polymyalgia rheumatica or giant cell arteritis". Clin. Exp. Rheumatol. 24 (2 Suppl 41): S38–40. PMID 16859595.
- ↑ Cimmino MA, Zaccaria A (2000). "Epidemiology of polymyalgia rheumatica". Clin. Exp. Rheumatol. 18 (4 Suppl 20): S9–11. PMID 10948749.
- ↑ Dasgupta B, Cimmino MA, Maradit-Kremers H; et al. (2012). "2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative". Ann. Rheum. Dis. 71 (4): 484–92. doi:10.1136/annrheumdis-2011-200329. PMC 3298664. PMID 22388996. Unknown parameter
|month=ignored (help) - ↑ Kyle V (1991). "Laboratory investigations including liver in polymyalgia rheumatica/giant cell arteritis". Baillieres Clin Rheumatol. 5 (3): 475–84. PMID 1807822. Unknown parameter
|month=ignored (help) - ↑ Cantini F, Salvarani C, Olivieri I; et al. (2001). "Shoulder ultrasonography in the diagnosis of polymyalgia rheumatica: a case-control study". J. Rheumatol. 28 (5): 1049–55. PMID 11361188. Unknown parameter
|month=ignored (help) - ↑ Cantini F, Niccoli L, Nannini C; et al. (2005). "Inflammatory changes of hip synovial structures in polymyalgia rheumatica". Clin. Exp. Rheumatol. 23 (4): 462–8. PMID 16095113.
- ↑ Cantini F, Salvarani C, Olivieri I; et al. (2001). "Inflamed shoulder structures in polymyalgia rheumatica with normal erythrocyte sedimentation rate". Arthritis Rheum. 44 (5): 1155–9. doi:10.1002/1529-0131(200105)44:5<1155::AID-ANR198>3.0.CO;2-N. PMID 11352249. Unknown parameter
|month=ignored (help) - ↑ Jiménez-Palop M, Naredo E, Humbrado L; et al. (2010). "Ultrasonographic monitoring of response to therapy in polymyalgia rheumatica". Ann. Rheum. Dis. 69 (5): 879–82. doi:10.1136/ard.2009.113555. PMID 19773289. Unknown parameter
|month=ignored (help) - ↑ Macchioni P, Catanoso MG, Pipitone N, Boiardi L, Salvarani C (2009). "Longitudinal examination with shoulder ultrasound of patients with polymyalgia rheumatica". Rheumatology (Oxford). 48 (12): 1566–9. doi:10.1093/rheumatology/kep286. PMID 19808693. Unknown parameter
|month=ignored (help) - ↑ Moosig F, Czech N, Mehl C; et al. (2004). "Correlation between 18-fluorodeoxyglucose accumulation in large vessels and serological markers of inflammation in polymyalgia rheumatica: a quantitative PET study". Ann. Rheum. Dis. 63 (7): 870–3. doi:10.1136/ard.2003.011692. PMC 1755055. PMID 15194587. Unknown parameter
|month=ignored (help) - ↑ Adams H, Raijmakers P, Smulders Y (2012). "Polymyalgia rheumatica and interspinous FDG uptake on PET/CT". Clin Nucl Med. 37 (5): 502–5. doi:10.1097/RLU.0b013e3182485098. PMID 22475905. Unknown parameter
|month=ignored (help) - ↑ http://www.rheumatology.org/public/factsheets/pmr_new2.asp "POLYMYALGIA RHEUMATICA." American College of Rheumatology. June 2006. American College of Rheumatology. 11 Mar. 2008
- ↑ Dasgupta B, Gray J, Fernandes L, Olliff C (1991). "Treatment of polymyalgia rheumatica with intramuscular injections of depot methylprednisolone". Ann. Rheum. Dis. 50 (12): 942–5. PMC 1004588. PMID 1768166. Unknown parameter
|month=ignored (help) - ↑ Li C, Dasgupta B (2000). "Corticosteroids in polymyalgia rheumatica--a review of different treatment schedules". Clin. Exp. Rheumatol. 18 (4 Suppl 20): S56–7. PMID 10948765.
- ↑ Caporali R, Cimmino MA, Ferraccioli G; et al. (2004). "Prednisone plus methotrexate for polymyalgia rheumatica: a randomized, double-blind, placebo-controlled trial". Ann. Intern. Med. 141 (7): 493–500. PMID 15466766. Unknown parameter
|month=ignored (help) - ↑ Salvarani C, Macchioni P, Manzini C; et al. (2007). "Infliximab plus prednisone or placebo plus prednisone for the initial treatment of polymyalgia rheumatica: a randomized trial". Ann. Intern. Med. 146 (9): 631–9. PMID 17470831. Unknown parameter
|month=ignored (help) - ↑ Nothnagl T, Leeb BF (2006). "Diagnosis, differential diagnosis and treatment of polymyalgia rheumatica". Drugs Aging. 23 (5): 391–402. PMID 16823992.
External links
- Polymyalgia Rheumatica - WebMD.com
- Polymyalgia Rheumatica - The Arthritis Society
- Patient Education - Polymyalgia Rheumatica - American College of Rheumatology
- National Medical Research Foundation Support groups for people with polymyalgia rheumatica
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