Polimialgia Rheumatik

Dr. Zurriyani, SpPD

 Epidemiology
• relatively common chronic inflammatory

• unknown etiology

• that affects elderly individuals (>70 yr)

• almost never seen in people under the age of 50

• It is characterized by proximal myalgia of the hip and shoulder girdles with

accompanying morning stiffness that lasts for more than 1 hour

• Approximately 15% of patients with PMR develop giant cell arteritis (GCA),

• 40-50% of patients with GCA have associated PMR. relationship between

GCA and PMR is not yet clearly established.
 Epidemiology
The incidence :in patients over 50 is about 100
per 100 000.
 Patophysiology & Etiology
• polymyalgia rheumatica is unknown cause

• genetic and environmental factors contribute to disease

susceptibility and severity.

• suggests an environmental infectious trigger, such as parvovirus

B19, Mycoplasma pneumoniae, and Chlamydia pneumoniae.

• It is linked to the HLA DR4 allele in white populations

• monocyte activation, which helps determine the production of

cytokines that induce manifestations characteristic of PMR and
GCA.
 Patophysiology & Etiology

• An autoimmune process may play a role in PMR

development. PMR is associated with the HLA-DR4
haplotype.

• A high level of IL-6 is associated with increased

disease activity.

• Many investigators believe that nonerosive synovitis

and tenosynovitis are responsible for many symptoms
of PMR.
 Clinical
• bilateral shoulder, hip girdle pain and stiffness
of acute or subacute onset with bilateral upper
arm tenderness.
• The stiffness may be so severe that the patient
may have a great difficulty rising from a chair,
turning over in bed, or raising the arms above
shoulder height.
• Stiffness after periods of rest (gel phenomenon)
as well as morning stiffness of more than 1 hour
typically occurs
 Clinical
• Muscle weakness is not a feature of PMR. However, this can be
difficult to assess in the setting of pain, especially if symptoms are
protracted and untreated, resulting in disuse atrophy.

• Patients may also describe distal peripheral joint swelling or, more
rarely, limb edema. Carpal tunnel syndrome can occur in some
patients.

• Most patients report systemic features as listed below.

• including fatigue, loss of appetite, weight loss, low grade fever,

and sometimes depression. Patients are always over the age of 50
and usually over 65.
 Diagnostic
• In 2012 EULAR and ACR , 2012
• This collaborative initiative resulted in a scoring algorithm
based on the following criteria:
– Morning stiffness >45 minutes (2 points)
– Hip pain/limited range of motion (1 point)
– Absence of rheumatoid factor and/or anti–citrullinated protein
antibody (anti-CCP) (2 points)
– Absence of peripheral joint pain (1 point)

• A score of ≥4 points has a 68% sensitivity and 78%

specificity for discriminating PMR
 Diagnostic
• There is also an additional ultrasound criteria
(1 point if positive findings), which can add up
to a score of ≥5 points that is associated with a
66% sensitivity and 81% specificity for PMR.
 Diagnostic
• Systemic findings in more than 50% of patients
are as follows:
– Low-grade fever and weight loss
– Malaise, fatigue, and depression
– Difficulty rising from bed in the morning
– Difficulty getting up from the toilet or out of a
chair
– Difficulty completing daily life activitiesHigh,
spiking fevers (rare, should prompt evaluation for
underlying infection, malignancy, or vasculitis)
• Musculoskeletal findings are as follows :
– Morning stiffness for ≥1 hour, often more prolonged
– Muscle stiffness after prolonged inactivity
– Synovitis of proximal joints and periarticular structures
– Peripheral arthritis (in 25% of patients)
– Carpal tunnel syndrome (in about 15% of patients)
– Distal extremity swelling (in approximately 12%)
– Possible development of arthralgia and myalgia up to 6
months after onset of systemic symptoms
 Defferential diagnostic

• Acute or chronic infection

• Infective endocarditis
• Bursitis/tendinitis
• Cervical spondylosis
• Dermatomyositis
• Malignancy
• Myopathy
• Parkinson disease
• Remitting seronegative symmetrical synovitis with pitting edema (RS3PE)
• Shoulder disorders (eg, shoulder synovitis, rotator cuff tendinitis, and
subdeltoid bursitis)
• Calcium pyrophosphate deposition disease
• Late-onset ankylosing spondylitis
• Vasculitis (eg, giant cell arteritis)
 Defferential diagnostic

• AA (Inflammatory) Amyloidosis
• Depression
• Fibromyalgia
• Giant Cell Arteritis (Temporal Arteritis)
• Hypothyroidism
• Multiple Myeloma
• Osteoarthritis
• Paraneoplastic Syndromes
• Polymyositis
 laboratory

• Rheumatoid factor and/or anti-cyclic citrullinated peptide

antibodies (anti-CCP)
• C-reactive protein (CRP) and/or erythrocyte
sedimentation rate (ESR)
• Complete blood cell count (CBC) with differential
• Blood glucose
• Serum creatinine
• Liver function test
• Bone profile (including calcium and alkaline phosphatase)
• Dipstick urinalysis
 Therapy

The joint EULAR/ACR guidelines include the following key treatment

recommendations [23] :

• Glucocorticoids are the preferred treatment.

• Short-term NSAIDs and/or analgesics may be used to treat pain related to
comorbid conditions such as osteoarthritis.
• Initial glucocorticoid therapy should be 12.5-25 mg/day of prednisone or
the equivalent. 
• Intramuscular methylprednisolone may be considered as an alternative to
oral glucocorticoid therapy.
• Early introduction of methotrexate (MTX) in addition to glucocorticoid
therapy, is conditionally recommended for patients at high risk of relapse
and/or prolonged therapy, as well as in those with risk factors for adverse
effects of glucocorticoid therapy.
• Use of MTX may be considered during follow-up in patients with a relapse,
lack of a significant response to glucocorticoid therapy, or development of
glucocorticoid adverse events
 Therapy

• The EULAR/ACR guidelines recommend individualized glucocorticoid dose-

tapering schedules, based on regular monitoring of disease activity,
laboratory markers, and adverse events.   Both guidelines agree that
the target dosages for tapering are 10 mg/day of prednisone or the
equivalent within 4-8 weeks. If relapse occurs, increase to the pre-relapse
dosage and decrease gradually (within 4-8 weeks) to the dosage at which
the relapse occurred. Once remission of PMR is achieved, taper the dosage
by 1 mg every 4 weeks (or by 1.25-mg decrements using schedules such as
10/7.5 mg on alternate days) until treatment is discontinued. [

• EULAR/ACR guidelines strongly recommend against the use of TNF-α

inhibitors in PMR. [23]

• Symptomatic palliation of pain with analgesic therapy alone with close

monitoring may be preferable in patients with intolerable adverse effects
from corticosteroids (eg, uncontrolled diabetes mellitus, severe
symptomatic osteoporosis, psychosis).
 Therapy

• Calcium and vitamin D supplementation

should be initiated in all patients with PMR
who are starting corticosteroid therapy.
• Generally, activity restriction is unnecessary.
Physical therapy is recommended for those
with difficulty achieving good mobility despite
adequate medical therapy.
 Complications

• PMR usually has a limited course of several months to 5 years.

Untreated patients often feel unwell and have an impaired quality of
life,
• PMR is not associated with serious complications. Patients treated
with corticosteroids are at risk for long-term complications of
corticosteroid therapy.
• Relapses are common and may occur in up to 25% of all treated
patients.
• Arteritic relapse in a patient who presented exclusively with PMR is
unusual. 
• Every patient should be considered at risk for giant cell arteritis
(GCA). 
• Several cases of systemic amyloidosis–associated PMR have been
reported. Rare cases of bilateral ocular inflammation (episcleritis,
scleritis, or anterior uveitis) developing during steroid tapering have
been reported
 prognosis

• PMR is usually self-limited. With prompt

diagnosis and adequate therapy, the condition
has an excellent prognosis.