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Introduction
Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition
Characterized
clinically by :
aching and morning stiffness in the shoulders,
hip girdle, and neck.
It can be associated with giant cell (temporal) arteritis (GCA), and the two disorders may represent different manifestations of a shared disease process
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Epidemiology
Disease of adults over the age of 50,
with a prevalence that increases progressively with advancing age
The
peak incidence of PMR occurs between ages 70 and 80
PMR is relatively common. The lifetime risk of PMR is second only to rheumatoid arthritis (RA) as a systemic rheumatic disease in adults
Women are affected two to three times more often than men
Cases of familial aggregation are recognized
The incidence is highest in Scandinavian countries and in people of northern European descent
PMR appears to be uncommon in Asian, African-American, and Latino populations, though all racial and ethnic groups may be affected.
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Association with GCA
PMR occurs in about 50% of
patients with GCA
The percentage of patients with PMR
who experience GCA at some point is ~10%
The two disorders may not be active synchronously
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Pathogenesis
The cause of polymyalgia rheumatica (PMR) is unknown
Both
environmental and genetic factors appear to play a role
Both
PMR and GCA are associated with specific alleles of human leukocyte antigen (HLA)-DR4
Some studies have suggested a cyclical pattern in incidence and seasonal variation
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Signs and Symptoms
Aching and morning stiffness
Shoulders, hip
girdle, neck, and torso are involved
Patients over the age
of 50
Symptoms are usually symmetric
Recent, discrete change in musculoskeletal symptoms
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Signs and Symptoms
Morning stiffness > 30 minutes
Stiffness at
the shoulders and hips may cause trouble with dressing
Stiffness
may be so severe that there is difficulty turning over in bed at night or arising from bed in the morning.
The ‘gel’ phenomenon, stiffness after inactivity, is often notably severe in PMR
An inability to actively abduct shoulders past 90 degrees because of stiffness is a typical finding
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Signs and Symptoms
Shoulder pain is more common at
presentation than hip pain
Pain is worse with movement and
may interfere with sleep
Synovitis and bursitis - in peripheral joints, such as the knees, wrists, and MCPs
Synovitis and bursitis are thought to be the causes of the discomfort and stiffness
Swelling and tenosynovitis – Some patients develop swelling and pitting edema of the hands, wrists, ankles, and top of the feet
Tenosynovitis can also cause carpal tunnel syndrome
Decreased range of motion – There may be decreased active and passive range of motion of the shoulders, neck, and hips.
Muscle tenderness – not a prominent feature, and what tenderness there may be about the shoulders is more likely due to synovial or bursal inflammation than muscle involvement
Normal muscle strength
Systemic signs and symptoms – malaise, fatigue, depression, anorexia, weight loss, and low-grade fever.
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Laboratory findings
Elevated ESR, CRP (although can be normal)
Normocytic
anemia
Thrombocytosis
Serologic tests, such as ANA, RF, ACPA are typically
negative
Increase in liver enzymes, especially alkaline phosphatase, although these abnormalities are more common in patients with GCA than PMR alone
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Imaging
Routine radiographs of inflamed joints do not show
abnormalities in patients with PMR
MRI and US can demonstrate
synovial inflammation, with a predilection for extra-articular synovial structures (bursitis, tenosynovitis)
Synovitis is never erosive
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Extra-articular involvement
Biceps tenosynovitis subdeltoid bursitis
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Evaluation of the patient
Medical history
Anemnesis
Physical examination
Assessment of the
response to low-dose glucocorticoids.
Symptoms are generally 50 to
70 % better within 3 days of prednisone (10 to 20 mg/day) and
Almost all patients respond completely within two weeks of initiation of therapy
MRI or US may be helpful to assess whether there is underlying bursitis or other evidence for inflammation
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Diagnosis
No pathognomonic test or established diagnostic criteria for
polymyalgia rheumatica (PMR)
Presence of all of the following, after
exclusion of alternative disease:
Age 50 years or older at disease onset
Proximally and bilaterally distributed aching and morning stiffness for at least two weeks
2/3 areas: neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the thighs
ESR ≥40 mm/h
Rapid resolution of symptoms with low-dose glucocorticoids.
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Evaluation for GCA
Patients with clinically "pure" PMR lack
the classic findings of GCA:
temporal artery tenderness,
headache,
jaw
pain, visual symptoms
arm claudication.
GCA may appear at any point during the clinical course of PMR
At every follow-up visit the patient should be monitored for GCA signs and symptoms
Evaluation, including biopsy, should be performed if symptoms of GCA develop, even if patients are on glucocorticoids
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Diffrential Diagnosis
Rheumatoid arthritis - symmetric polyarthritis of the
small joints of the hands and feet, which is
persistent and only partially responsive to low doses of prednisone. In PMR fewer joints are swollen, and swelling subsides completely in response to low-dose prednisone
RS3PE syndrome - Remitting Seronegative Symmetrical Synovitis with Pitting Edema, also described as the puffy edematous hand syndrome or distal extremity swelling with pitting edema
Inflammatory myopathy - Dermatomyositis or polymyositis present with symmetric proximal muscle weakness
Fibromyalgia
Infective endocarditis
Lyme disease may present with nonspecific constitutional symptoms that include myalgias and arthralgias
Malignancy
Vasculitis
