Diffuse
Polymyalgia Rheumatica
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Polymyalgia Rheumatica
, PMR
See Also
Temporal Arteritis
Epidemiology
Common over age 50 years (peaks between ages 70 to 80 years old)
Incidence
: 50 per 100,000
Prevalence
: One in 143 persons
Most common in white persons of Northern European descent
Rare in Asian and black patients
Women predominate by 2:1 ratio
Associated with HLA-DR4 and Cw3 haplotypes
Seasonal outbreaks suggest an infectious trigger
Associated with
Temporal Arteritis
Temporal Arteritis
is occurs in 18-26% of Polymyalgia Rheumatica cases
Polymyalgia Rheumatica is 2-3 times more common than
Temporal Arteritis
Symptoms
Severe
Muscle
ache and stiffness
Duration
Minimum of 2 week duration for diagnosis
Typically 1 month or longer at presentation
Usually insidious onset
Location (symmetric, bilateral involvement)
Shoulder
s (affected in 95% of cases)
Neck
Pelvic girdle and hips
Characteristics
Ache worse at night and with movement
Stiffness
Timing
More prominent in morning or after inactivity
Morning stiffness lasts >45 minutes for diagnosis
Associated systemic symptoms (30-50% of cases)
Malaise
Anorexia
Weight loss
Low grade fever
Depressed mood
Night Sweats
Signs
Unremarkable physical exam
Symptoms are usually well out-of-proportion to exam
No true motor weakness
Strength limitation in PMR should be due to pain and disuse atrophy
True
Muscle Weakness
suggests alternative diagnosis
Mild findings (variably present)
Limited range of motion in affected joints
Limited by proximal myalgias
Shoulder
or hip
Bursitis
Localized tenderness over
Shoulder
s and hips
Other findings which may be present (50% of cases)
Asymmetric knee or wrist
Arthritis
Carpal Tunnel Syndrome
Distal extremity edema (wrists, hands, ankles, feet)
Precautions
Red Flags (suggestive of
Temporal Arteritis
)
See
Temporal Arteritis
Abrupt
Headache
onset
Jaw Claudication
(or tongue
Claudication
)
Limb
Claudication
Temporal artery abnormalities (prominence, beading, decreased pulse, tenderness)
Visual disturbance
Upper
Cranial Nerve
deficit
Differential Diagnosis
PMR is a diagnosis of Exclusion (other disorders make PMR less likely)
Inflammatory
Arthritis
See
Shoulder Pain
See
Rheumatologic Causes of Shoulder Pain
Myopathy
See
Myopathy Causes
See
Polymyositis Differential Diagnosis
See
Medication Causes of Myositis
Thyroid
Myopathy
(
Hypothyroidism
,
Hyperthyroidism
)
Hyperparathyroidism
Polymyositis
Parkinsonism
or other neurologic disorder
Prominent systemic symptoms (e.g. weight loss, night pain)
Malignancy
Multiple Myeloma
Lymphoma
Prostate Cancer
Stomach Cancer
Ovarian Cancer
Renal cell cancer
Lung Cancer
and paraneoplastic syndrome
Infection
Subacute Bacterial Endocarditis
Tuberculosis
HIV Infection
Hepatitis C
Diagnosis
British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) Criteria
Age over 50 years old
Bilateral ache in
Shoulder
and/or pelvic girdle
Morning stiffness >45 minutes in
Shoulder
and/or pelvic girdle
Duration of symptoms >2 weeks
Acute phase reactant increase (i.e. CRP or ESR)
Labs
Acute phase reactant increased (obtain both
C-RP
and ESR)
C-Reactive Protein
(
C-RP
)
Better
Test Sensitivity
for Polymyalgia Rheumatica than ESR
ESR however is preferred for predicting relapse
Erythrocyte Sedimentation Rate
(ESR)
Normal upper limit ESR is typically age/2 for men and (age+10)/2 for women
ESR >40 mm/h in >91% of Polymyalgia Rheumatica
False Negative
ESR in 6-20% of patients with Polymyalgia Rheumatica
C-RP
is typically positive in these
False Negative
cases
ESR >50 mm/h in most cases (mean 65 mm/h)
ESR 83 mm/h is average for
Giant Cell Arteritis
ESR >100 mm is associated with higher likelihood of
Giant Cell Arteritis
(or underlying malignancy)
Labs to evaluate differential diagnosis
Complete Blood Count
Thyroid Stimulating Hormone
(TSH)
Comprehensive metabolic panel
Includes
Electrolyte
s,
Renal Function
tests and
Liver Function Test
s
Creatine Phosphokinase
(CPK) Normal
Differentiate from
Polymyositis
Rheumatoid Factor
Urinalysis
Serum Protein Electrophoresis
or
Urine Protein
electrophoresis
Nonspecific Lab findings
Moderate
Anemia
Decreased
Serum Albumin
Mild hepatic dysfunction
Imaging
Chest XRay
Consider if paraneoplastic syndrome associated with
Lung Cancer
is suspected
Associated Conditions
Temporal Arteritis
Occurs in 18-26% of PMR patients
Risk of blindness
Consider
Temporal Arteritis
in all PMR patients
Factors suggesting concurrent
Temporal Arteritis
Age over 70 years
New onset
Headache
Jaw Claudication
Raised liver enzymes
Abnormal temporal arteries on exam
References
Rodriguez-Valverde (1997) Am J Med 102:331-6 [PubMed]
Management
Gene
ral measures
Consider concurrent
Temporal Arteritis
(See above)
NSAID
s
Prednisone
(key to management)
See
Corticosteroid Associated Osteoporosis
Efficacy: 90% response
Dramatic improvement within first week (especially in first 48 hours)
Acute phase reactants (
C-RP
and ESR) normalize within first 4 weeks
If no response to steroids
Reconsider diagnosis
Consider
Methotrexate
(see below)
Polymyalgia alone
Dose: 15-20 mg orally daily
Prednisone
15 mg is sufficient for most patients and no added benefits to higher dose
Prednisone
10 mg is associated with increased relapse rate
Example
Prednisone
course
Prednisone
15 mg daily for 3 weeks, then
Prednisone
12.5 mg daily for 3 weeks, then
Prednisone
10 mg daily for 4-6 weeks, then
Prednisone
taper by 1 mg/day every 4-8 weeks over 1-2 years
Alternative: Example
Methylprednisolone
IM course
Methylprednisolone
(Depo Medrol) 120 mg IM every 3-4 weeks
Taper by 20 mg/dose every 2-3 months
Polymyalgia with
Temporal Arteritis
Dose: 40-60 mg orally daily
Symptoms and signs remit within 1 month
Decrease dose by 10% each week after improvement
Course (mean total length of treatment 1.8 years)
Initial: Maintain starting dose for 1 month
First steroid taper (depends on clinical response)
Taper by 2.5 mg per month down to 10 mg/day then
Taper 1 mg per 4-6 weeks down to 5 to 7.5 mg/day
Final steroid taper
Indicated when symptom free for 6-12 months
Do not taper until sedimentation rate normalizes
Taper by 1 mg every 6-8 weeks until done
Anticipate 2-6 year course of steroids
Relapse common in first 18 months of steroid use
Patients off steroids at 2 years: 25%
Monitoring
Follow
C-RP
and anticipate decreased levels after initiating therapy
Prevention of
Corticosteroid
related
Osteoporosis
See
Corticosteroid Associated Osteoporosis
Vitamin D
Supplementation
Calcium Supplementation
Consider
DEXA Scan
while starting
Corticosteroid
s
Consider bisphosphonate on starting
Prednisone
Strongly consider for high risk of
Fracture
(over 65 years old or prior
Fracture
)
Also consider if
DEXA Scan
T-Score
-1.5 or less
Adjunctive medications
Methotrexate
10 mg weekly
Associated with lower steroid doses and lower relapse rates
Etanercept
(
Enbrel
)
Management
Follow-up
Rheumatology referral indications (most cases referred in U.S.)
Age <60 years old at onset
Chronic presentation >2 years
Other
Rheumatologic Disease
Poor response to
Corticosteroid
s
Significant systemic symptoms (e.g. weight loss, neurologic symptoms)
Significant increases in acute phase reactants (e.g. ESR >100 mm/h)
Absent key PMR features
Minimal morning stifffness
No
Shoulder
involvement
Clinic Visits
Timing
One week after starting steroids, then
Three weeks after starting steroids, and then
Every 3 months
Labs (each visit)
Complete Blood Count
Erythrocyte Sedimentation Rate
(ESR)
C-Reactive Protein
(
C-RP
)
Basic chemistry panel (
Electrolyte
s,
Renal Function
tests, and
Serum Glucose
)
Evaluation
Relapse symptoms
Proximal
Muscle
pain (
Shoulder
or pelvic girdle)
Morning stiffness
Fatigue
Giant Cell Arteritis
symptoms
Temporal
Headache
Jaw Claudication
(or tongue
Claudication
)
Vision
changes
Adverse effects to treatment (
Corticosteroid
adverse effects)
Gastritis
or peptic ulcer
Bone density
Hyperglycemia
Prognosis
Self limited course over years (usually 3-6 years)
References
Caylor (2013) Am Fam Physician 88(10):676-84 [PubMed]
Dasgupta (2010) Rheumatology 49(1): 186-90 [PubMed]
Hernandez-Rodriguez (2009) Arch Intern Med 169: 1839-50 [PubMed]
Ostor (2002) Practitioner 246:756-63 [PubMed]
Selvarani (2002) N Engl J Med 347:261-71 [PubMed]
Unwin (2006) Am Fam Physician 74:1547-58 [PubMed]
Weyand (2003) Ann Intern Med 139:505-15 [PubMed]
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