Polymalgia Rheumatica

Report
Polymalgia
Rheumatica
Dr. Barb Blumenauer MD, FRCPC
Introduction
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Inflammatory condition associated with pain and morning stiffness
in neck, shoulders and hip girdle
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Can be associated with Giant Cell Arteritis (GCA)
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Generally patients > 50 with peak incidence between 70-80
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Second most common inflammatory condition (#1 RA)
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Female 2-3:1 Males
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Most common in Scandinavian and Northern European descent with
high of 113 per 100,000 in Norway
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Rare in Asians, African-Americans and Latinos
PMR and GCA
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PMR 2-3 times more common than GCA
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Approximately 50% of GCA patients have PMR
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5-30% PMR patients have GCA
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Etiology unknown but are HLA associations
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Increased interleukin 6 levels in blood of PMR patients
Signs and Symptoms
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Stiffness at least 30 minutes
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Gel phenomena
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Pain shoulder > hip and neck
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May have synovitis, bursitis, swelling, tenosynovitis or carpal tunnel
syndrome
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Antalgic decreased range of motion actively but usually not
passively (if patient can adequately relax)
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Subjective weakness but usually normal if can "ignore" pain during
exam
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Systemic symptoms
Investigations
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Elevated ESR common often greater than 40
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Elevated CRP may be more common than elevated ESR
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May have normocytic anemia; WBC/platelets usually normal
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Serology usually negative but prevalence positive RF and ANA
increase with age
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Occasionally transaminitis
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Joint x-rays normal aside from normal age changes
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US, MRI etc can show synovitis but aren't generally done
Diagnosis: Provisional
ACR/EULAR Criteria
Required Criteria
Additional Criteria
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Age 50 or above
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EMS of 45 or more minutes ( 2 points)
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Bilateral shoulder aching
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RF(-) or anti-CCP (-) (2 points)
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Abnormal ESR or CRP
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Pain or limited ROM at hip ( 1 point)
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Absence peripheral joint pain ( 1
point)
Required criteria plus score of 4 or more 78% specificity and 68% sensitivity! Adding US increases
specificity to 91%. However really meant for research versus diagnosis
Differential Diagnosis
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Main is RF(-) RA
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RS3PE Syndrome
•
•
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Multifocal local MSK disease
Bone disease ( multiple myeloma,
metastatic disease, osteomalacia,
hyperparathyroidism etc)
Drug induced myalgias/myositis i.e.
statins etc
•
Inflammatory myopathy like
dermatomyositis
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Fibromyalgia
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Endocarditis
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Hypothyroidism
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Paraneoplastic syndrome
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ANCA associated vasculitis
RA versus PMR
RA usually symmetrical with small joint involvement
RA less responsive to Prednisone
Up to 20 % patients diagnosed with PMR may eventually be changed
to RA
Often difficult to taper prednisone in RA patients without DMARD
therapy
PMR should have negative serology
PMR non erosive
The disease with the least agreement between a rheumatologist and a
non rheumatologist
PMR versus Fibromyalgia
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20 year olds don't get PMR
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No "true" EMS/ gel phenomenon i.e. fibromyalgia (FM)worse with
activity, better with rest which is opposite of PMR
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FM usually widespread pain and usually chronic not acute
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Labs normal in FM
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Classic tender points in FM, not in PMR
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PMR usually pain free passive ROM
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PMR not tender to touch
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FM not overly prednisone (PDZ) responsive " takes the edge off"
whereas PMR by definition exquisitely PDZ sensitive
PMR Versus Myositis
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Myositis presents with PAINLESS weakness
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PMR presents with pain and NO WEAKNESS aside from antalgic
weakness
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Almost always elevated CPK in myositis and not in PMR
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Patients with PMR can get weakness secondary to steroid myopathy
but it gets better with PDZ reduction whereas myositis weakness
gets worse with PDZ reduction
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Myositis can have rash and other findings such as Gottren's plaques,
mechanics hands etc
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Myositis can have extra-articular manifestations (lung disease etc.)
and PMR does not
Rotator Cuff Tendinitis(RCT)
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Can mimic PMR but RCT no systemic symptoms and normal
labwork
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When tapering PDZ consider RCT if otherwise well and
inflammatory markers normal
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Usually positive rotator cuff impingement signs
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If present intra-articular steroids well settle it allowing PDZ taper
Vitamin D Deficiency
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Can can cause myalgias but usually diffuse without systemic
symptoms, EMS, gel etc
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Common in Canadians especially elderly so may coexist with PMR
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Vitamin supplementation for all patients especially with PDZ use
Treatment
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Main stay treatment is PDZ
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Exquisitely PDZ sensitive often 10-15 mg, rarely 20 mg daily
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If patient not 100% settled with 20 you either have the wrong
diagnosis or something else is going on
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Usually need treatment for 12-18 months
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Once settled decrease PDZ at monthly intervals or longer, usually
2.5 decrease until you reach 10 mg daily and then usually 1 mg
monthly thereafter as tolerated
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If you can't get the patient off PDZ rethink the diagnosis – it might
be RA!
PMR Treatment Continued
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No good evidence for any other drug reducing steroid cumulative
dose, flares etc.
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A few studies show minimal effect of methotrexate
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Some evidence for anti-interleukin 6 treatment working in GCA and
perhaps effect in PMR but cost prohibitive!
Don't Forget About GCA
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Patients need to be aware of warning symptoms of GCA and to seek
immediate attention if occurs
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Common symptoms include scalp pain, painful scalp sores, jaw
claudication, severe NEW headache and sudden visual loss
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Requires urgent treatment EVEN BEFORE diagnosis confirmed to
prevent permanent bilateral blindness – visual loss does not settle
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Usually elevated ESR but could be partially treated if on PDZ for
PMR
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Can have GCA even if temporal artery biopsy negative
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Beware involvement of other vessels and be aware of increased risk
or aortic artery its branches developing aneurysms.
GCA Treatment
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PDZ 60-80 mg daily
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ASA 325 daily may decrease chance of blindness but beware additive
risk of GI bleeding – consider PPI gastroprotection
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Watch for PDZ induced DM or glaucoma
Osteoporosis Prophylaxis
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Lose majority of bone in the first 3 months so start ASAP
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Can use bisphosphonate, Prolia or Forteo
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Parenteral administration usually more effective
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Vitamin D 2000 IU daily
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Calcium 500 mg p.o. bid with meals
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Baseline BMD and repeat q 6/12 or as indicated. If losing bone
consider other causes of osteopenia, compliance with treatment,
absorption etc and consider changing route of drug administration or
class of drug
Prednisone Plea
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If you think patient needs PDZ for MSK disease please consider
urgent rheumatology referral BUT if GCA symptoms treat ASAP pre
rheumatology assessment
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Want to ensure patient doesn't have RA as PDZ not effective disease
modifying agent and treatment patients get within the first 12 weeks
of symptom onset determines how patient do forever! ( forever
scarred)
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We have the BEST DRUGS and early treatment is abolishing
damage for many patients – besides the orthopods are too busy to
replace joints that could have been saved.

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