POLYMYALGIA

RHEUMATICA
dr. Monalisa, SpPD
Polymyalgia rheumatica

 Didiagnosa pada pasien dengan:

 Nyeri dan kaku pada otot di leher, shoulder, and pelvic selama paling
kurang 4 minggu.

The myalgias are combined with signs of systemic inflammation

manifesting clinically as
 malaise,
 weight loss,
 sweats,
 and low-grade fever.
Polymyalgia rheumatica

 Most patients have laboratory abnormalities such as

Elevated ESR,
elevated CRP,
and anemia,
 which are indicative of a systemic inflammatory syndrome.
 Upregulation of acute phase reactants sangat menolong dalam
membedakan PMR dari sindroma nyeri yang lain.
 No pathognomic test for PMR is available;
 exclusion of other diseases with similar clinical presentations is
essential.
 The systemic inflammatory syndrome associated with PMR is
exquisitely sensitive to glucocorticoid therapy.
Epidemiology

 Epidemiological studies are difficult.

 Perempuan lebih sering dibandingkan laki-laki
 Sering mengenai pada usia lebih dari 50 th.
high-risk populations,
 Scandinavians and other peoples of Northern European descent,
annual incidence rates have been estimated at 20 to 53 per 100,000
persons over the age of 50 years.
 In low-risk populations, such as Italians, the annual incidence rates for
individuals aged 50 years and older are only 10 cases per 100,000.
Pathogenesis

 inflammation is suspicious for an infectious etiology,

 Human leukocyte antigen (HLA) polymorphisms that are genetic risk
factors for Giant Cell Arteritis( GCA) are also associated with PMR.
 There is no evidence that the HLA has a role in determining whether
the disease process will remain limited to PMR or progress to fully
developed GCA.
Continue…

 Polymyalgia rheumatica appears to be associated with a global

activation of the innate immune system, including circulating
monocytes that produce IL-1 and IL-6.
 Activated dendritic cell (DC)s render arteries susceptible to
vasculitis.
 In many patients with PMR, in situ cytokine production can be
demonstrated in biopsy specimens,
Etiology

 Probably polygenic in which multiple environmental and genetic

factors influence susceptibility and severity.
 Possible infectious triggers:
 Viruses: adenovirus, RSV, parvovirus, parainfluenza
 Bacteria: Mycoplasma, Chlamydia pneumoniae
Continue….

 Genetic component probable

 HLA-DRB1*04 and -DRB1*01 appear to be most associated with
susceptibility to PMR
 Genetic polymorphisms of additional genes involved in initiation and
regulation of inflammatory reaction:
 ICAM-1, TNF, IL-1 receptor antagonists
 Possible subclinical vasculitis
Manifestasi klinik

 nyeri pada otot leher, shoulders, lower back, hips,

 In typical cases, the onset is abrupt and the myalgias symmetrical;
they usually affect the shoulders first.
 Often the patients have pain during the night and have difficulties
rising and dressing themselves.
 Weight loss, anorexia, malaise, and depression are common.
Continue….

 Fever and chills should raise the suspicion of fully developed GCA.
 PMR sering kali sulit dibedakan dengan seronegative polyarthritis.
 sebagian kasus , pada pasien laki –laki terdapat nyeri proximal
dan edema diffuse tangan dan kaki yang sangat respon dengan
glucocorticoid.
 Patients with PMR must be carefully evaluated for possible GCA.
 A negative temporal artery biopsy does not exclude the possibility
of large vessel vasculitis targeting primarily the subclavian and
axillary arteries and the aorta.
 Signs of vascular insufficiency,
 including claudication in the extremities,
 bruits over arteries,
 and discrepant blood pressure readings should alert the physician to the
possibility of GCA .
MRA can be helpful in confirming the concomitant diagnosis of large
vessel vasculitis.
 In PMR patients with inflammation of periarticular structures,
 the most prominent findings are subdeltoid and subacromial bursitis
 Biceps tendonitis and glenohumeral synovitis may also be present.
 Ultrasonography reveals fluid accumulation in the bursae;
 MRI shows thickening and edema.
 These involved areas show increased uptake on PET scans.
Continue…

Figure 2. Ultrasonography (Panel A) and Magnetic Resonance Imaging (Panel B) of the

Shoulder of a Patient with Untreated Polymyalgia Rheumatica.
In Panel A, ultrasonography reveals the presence of fluid within the subacromial bursa (arrows)
and surrounding the long bicepstendon groove (arrowheads).
In Panel B, an axial T2-weighted section shows subacromial and subdeltoid bursitis (arrowheads),
joint effusion (arrow), and tenosynovitis of the long head of the biceps (curved arrow).

Cantini, F. Polymyalgia Rheumatica and Giant Cell Arteritis. NEJM. 2002. Vol. 347,
No. 4, pp. 261-271.
 Clinical Manifestations

Figure 3. The Hands of a Patient with Untreated Polymyalgia Rheumatica.

Cantini, F. Polymyalgia Rheumatica and Giant Cell Arteritis. NEJM. 2002. Vol. 347, No. 4, pp. 261-271.
• Panel A shows bilateral, diffuse swelling of the
hands and fingers with pitting edema.
• Panel B shows a bilateral magnetic resonance image
of the patient’s hands in a praying position. An axial
T2-weighted section through the midpoint of the
palm shows subcutaneous edema in the dorsum
(arrows), as well as fluid collection in the extensor
synovial sheaths (open arrows) and the flexor
synovial sheaths (curved arrow).
 The clinical symptoms of PMR can be mimicked by
a number of arthropathies,
 shoulder disorders,
 inflammatory
 myopathies,

 hypothyroidism, and Parkinson’s disease.

 The differential diagnosis also includes malignancies and
infections.
 No clear guidelines have been developed to determine
whether patients with PMR should be screened for
occult malignancies.
 Lack of the typical and impressive improvement upon
initiation of therapy can provide a clue towards
reevaluating the diagnosis of PMR.
terapi

 Two thirds of patients can be expected to

respond with remission of pain and stiffness when
started on 20 mg/day or less prednisone
 Some patients will need doses as high as 40
mg/day for complete clinical control.
 Such patients may be at higher risk of full-blown
GCA.
 Patients initially controlled on 20 mg/day of prednisone can usually
taper the dose by 2.5 mg every 10 to 14 days.
 More protracted tapering may be necessary once daily doses of 7
to 8 mg prednisone are attained.
 Dose adjustments should be based mainly on clinical evaluation,
 not exclusively on laboratory abnormalities.
 In many patients, PMR can go into long-term remission, and
prednisone can be discontinued.
 Successful suppression of recurrent myalgias and stiffness
may only be achieved by giving very low doses of
prednisone over an extended period.
 Patients should be warned about the potential of PMR
progressing to GCA and should be monitored for
vascular complications, particularly when discontinuing
glucocorticoid
Prognosis

 The prognosis of patients with PMR is good.

 In the majority of patients, the condition is self-limited.
 A proportion of patients will eventually present with typical
symmetrical polyarthritis, fulf lling the criteria for the diagnosis of
seronegative rheumatoid arthritis.
 Such patients may require disease-modifying antirheumatic drug
(DMARD) therapy.
terimakasih