1. Polymalgia Rheumatica
Dr. Barb Blumenauer MD, FRCPC

2. Introduction
Inflammatory condition associated with pain and morning stiffness in neck, shoulders and hip girdle
Can be associated with Giant Cell Arteritis (GCA)
Generally patients > 50 with peak incidence between 70-80
Second most common inflammatory condition (#1 RA)
Female 2-3:1 Males
Most common in Scandinavian and Northern European descent with high of 113 per 100,000 in Norway
Rare in Asians, African-Americans and Latinos

3. PMR and GCA PMR 2-3 times more common than GCA
Approximately 50% of GCA patients have PMR
5-30% PMR patients have GCA
Etiology unknown but are HLA associations
Increased interleukin 6 levels in blood of PMR patients

4. Signs and Symptoms Stiffness at least 30 minutes Gel phenomena
Pain shoulder > hip and neck
May have synovitis, bursitis, swelling, tenosynovitis or carpal tunnel syndrome
Antalgic decreased range of motion actively but usually not passively (if patient can adequately relax)
Subjective weakness but usually normal if can "ignore" pain during exam
Systemic symptoms

5. Investigations Elevated ESR common often greater than 40
Elevated CRP may be more common than elevated ESR
May have normocytic anemia; WBC/platelets usually normal
Serology usually negative but prevalence positive RF and ANA increase with age
Occasionally transaminitis
Joint x-rays normal aside from normal age changes
US, MRI etc can show synovitis but aren't generally done

6. Diagnosis: Provisional ACR/EULAR Criteria
Required Criteria
Additional Criteria
EMS of 45 or more minutes ( 2 points)
RF(-) or anti-CCP (-) (2 points)
Pain or limited ROM at hip ( 1 point)
Absence peripheral joint pain ( 1 point)
Age 50 or above
Bilateral shoulder aching
Abnormal ESR or CRP
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

7. Differential Diagnosis
Main is RF(-) RA
RS3PE Syndrome
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
Fibromyalgia
Endocarditis
Hypothyroidism
Paraneoplastic syndrome
ANCA associated vasculitis

8. 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

9. PMR versus Fibromyalgia
20 year olds don't get PMR
No "true" EMS/ gel phenomenon i.e. fibromyalgia (FM)worse with activity, better with rest which is opposite of PMR
FM usually widespread pain and usually chronic not acute
Labs normal in FM
Classic tender points in FM, not in PMR
PMR usually pain free passive ROM
PMR not tender to touch
FM not overly prednisone (PDZ) responsive " takes the edge off" whereas PMR by definition exquisitely PDZ sensitive

10. PMR Versus Myositis Myositis presents with PAINLESS weakness
PMR presents with pain and NO WEAKNESS aside from antalgic weakness
Almost always elevated CPK in myositis and not in PMR
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
Myositis can have rash and other findings such as Gottren's plaques, mechanics hands etc
Myositis can have extra-articular manifestations (lung disease etc.) and PMR does not

11. Rotator Cuff Tendinitis(RCT)
Can mimic PMR but RCT no systemic symptoms and normal labwork
When tapering PDZ consider RCT if otherwise well and inflammatory markers normal
Usually positive rotator cuff impingement signs
If present intra-articular steroids well settle it allowing PDZ taper

12. Vitamin D Deficiency
Can can cause myalgias but usually diffuse without systemic symptoms, EMS, gel etc
Common in Canadians especially elderly so may coexist with PMR
Vitamin supplementation for all patients especially with PDZ use

13. Treatment Main stay treatment is PDZ
Exquisitely PDZ sensitive often mg, rarely 20 mg daily
If patient not 100% settled with 20 you either have the wrong diagnosis or something else is going on
Usually need treatment for months
Once settled decrease PDZ at monthly intervals or longer, usually decrease until you reach 10 mg daily and then usually 1 mg monthly thereafter as tolerated
If you can't get the patient off PDZ rethink the diagnosis – it might be RA!

14. PMR Treatment Continued
No good evidence for any other drug reducing steroid cumulative dose, flares etc.
A few studies show minimal effect of methotrexate
Some evidence for anti-interleukin 6 treatment working in GCA and perhaps effect in PMR but cost prohibitive!

15. Don't Forget About GCA
Patients need to be aware of warning symptoms of GCA and to seek immediate attention if occurs
Common symptoms include scalp pain, painful scalp sores, jaw claudication, severe NEW headache and sudden visual loss
Requires urgent treatment EVEN BEFORE diagnosis confirmed to prevent permanent bilateral blindness – visual loss does not settle
Usually elevated ESR but could be partially treated if on PDZ for PMR
Can have GCA even if temporal artery biopsy negative
Beware involvement of other vessels and be aware of increased risk or aortic artery its branches developing aneurysms.

16. GCA Treatment PDZ 60-80 mg daily
ASA 325 daily may decrease chance of blindness but beware additive risk of GI bleeding – consider PPI gastroprotection
Watch for PDZ induced DM or glaucoma

17. Osteoporosis Prophylaxis
Lose majority of bone in the first 3 months so start ASAP
Can use bisphosphonate, Prolia or Forteo
Parenteral administration usually more effective
Vitamin D 2000 IU daily
Calcium 500 mg p.o. bid with meals
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

18. Prednisone Plea
If you think patient needs PDZ for MSK disease please consider urgent rheumatology referral BUT if GCA symptoms treat ASAP pre rheumatology assessment
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)
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.