Presentation on theme: "Polymalgia Rheumatica"— Presentation transcript:
1Polymalgia Rheumatica Dr. Barb Blumenauer MD, FRCPC
2IntroductionInflammatory condition associated with pain and morning stiffness in neck, shoulders and hip girdleCan be associated with Giant Cell Arteritis (GCA)Generally patients > 50 with peak incidence between 70-80Second most common inflammatory condition (#1 RA)Female 2-3:1 MalesMost common in Scandinavian and Northern European descent with high of 113 per 100,000 in NorwayRare in Asians, African-Americans and Latinos
3PMR and GCA PMR 2-3 times more common than GCA Approximately 50% of GCA patients have PMR5-30% PMR patients have GCAEtiology unknown but are HLA associationsIncreased interleukin 6 levels in blood of PMR patients
4Signs and Symptoms Stiffness at least 30 minutes Gel phenomena Pain shoulder > hip and neckMay have synovitis, bursitis, swelling, tenosynovitis or carpal tunnel syndromeAntalgic decreased range of motion actively but usually not passively (if patient can adequately relax)Subjective weakness but usually normal if can "ignore" pain during examSystemic symptoms
5Investigations Elevated ESR common often greater than 40 Elevated CRP may be more common than elevated ESRMay have normocytic anemia; WBC/platelets usually normalSerology usually negative but prevalence positive RF and ANA increase with ageOccasionally transaminitisJoint x-rays normal aside from normal age changesUS, MRI etc can show synovitis but aren't generally done
6Diagnosis: Provisional ACR/EULAR Criteria Required CriteriaAdditional CriteriaEMS 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 aboveBilateral shoulder achingAbnormal ESR or CRPRequired 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
7Differential Diagnosis Main is RF(-) RARS3PE SyndromeMultifocal local MSK diseaseBone disease ( multiple myeloma, metastatic disease, osteomalacia, hyperparathyroidism etc)Drug induced myalgias/myositis i.e. statins etcInflammatory myopathy like dermatomyositisFibromyalgiaEndocarditisHypothyroidismParaneoplastic syndromeANCA associated vasculitis
8RA versus PMRRA 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
9PMR versus Fibromyalgia 20 year olds don't get PMRNo "true" EMS/ gel phenomenon i.e. fibromyalgia (FM)worse with activity, better with rest which is opposite of PMRFM usually widespread pain and usually chronic not acuteLabs normal in FMClassic tender points in FM, not in PMRPMR usually pain free passive ROMPMR not tender to touchFM not overly prednisone (PDZ) responsive " takes the edge off" whereas PMR by definition exquisitely PDZ sensitive
10PMR Versus Myositis Myositis presents with PAINLESS weakness PMR presents with pain and NO WEAKNESS aside from antalgic weaknessAlmost always elevated CPK in myositis and not in PMRPatients with PMR can get weakness secondary to steroid myopathy but it gets better with PDZ reduction whereas myositis weakness gets worse with PDZ reductionMyositis can have rash and other findings such as Gottren's plaques, mechanics hands etcMyositis can have extra-articular manifestations (lung disease etc.) and PMR does not
11Rotator Cuff Tendinitis(RCT) Can mimic PMR but RCT no systemic symptoms and normal labworkWhen tapering PDZ consider RCT if otherwise well and inflammatory markers normalUsually positive rotator cuff impingement signsIf present intra-articular steroids well settle it allowing PDZ taper
12Vitamin D DeficiencyCan can cause myalgias but usually diffuse without systemic symptoms, EMS, gel etcCommon in Canadians especially elderly so may coexist with PMRVitamin supplementation for all patients especially with PDZ use
13Treatment Main stay treatment is PDZ Exquisitely PDZ sensitive often mg, rarely 20 mg dailyIf patient not 100% settled with 20 you either have the wrong diagnosis or something else is going onUsually need treatment for monthsOnce settled decrease PDZ at monthly intervals or longer, usually decrease until you reach 10 mg daily and then usually 1 mg monthly thereafter as toleratedIf you can't get the patient off PDZ rethink the diagnosis – it might be RA!
14PMR Treatment Continued No good evidence for any other drug reducing steroid cumulative dose, flares etc.A few studies show minimal effect of methotrexateSome evidence for anti-interleukin 6 treatment working in GCA and perhaps effect in PMR but cost prohibitive!
15Don't Forget About GCAPatients need to be aware of warning symptoms of GCA and to seek immediate attention if occursCommon symptoms include scalp pain, painful scalp sores, jaw claudication, severe NEW headache and sudden visual lossRequires urgent treatment EVEN BEFORE diagnosis confirmed to prevent permanent bilateral blindness – visual loss does not settleUsually elevated ESR but could be partially treated if on PDZ for PMRCan have GCA even if temporal artery biopsy negativeBeware involvement of other vessels and be aware of increased risk or aortic artery its branches developing aneurysms.
16GCA Treatment PDZ 60-80 mg daily ASA 325 daily may decrease chance of blindness but beware additive risk of GI bleeding – consider PPI gastroprotectionWatch for PDZ induced DM or glaucoma
17Osteoporosis Prophylaxis Lose majority of bone in the first 3 months so start ASAPCan use bisphosphonate, Prolia or ForteoParenteral administration usually more effectiveVitamin D 2000 IU dailyCalcium 500 mg p.o. bid with mealsBaseline 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
18Prednisone PleaIf you think patient needs PDZ for MSK disease please consider urgent rheumatology referral BUT if GCA symptoms treat ASAP pre rheumatology assessmentWant 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.