Polymyalgia Rheumatica

JL Nam

Mrs GP




71yrs
HT
LVF
Osteoporosis
Lumbar spinal stenosis ( L3/L4 on MRI)
PMR – Diagnosed in November 2002

Treatment DATE 11/02 08/04/03 22/04/03 03/06/03 02/09/03 25/11/03 30/03/04 Prednisone 0 15 30 30 15 10 15 Symptoms ++ ++ Better Better + + + ESR 83 59 17 30 62 55 50 .

Clinic visit on 30 March 2004     Pain in shoulders & arms esp. with lifting Painful left hip Painful swollen left ankle x 4/7 EMS few minutes in the pelvic girdle .

Examination  Shoulders:     Left trochanteric bursitis Left ankle   FROM Pain with active abduction – in upper arms rather than shoulders Warm +. swelling + PMR vs Periarticular structures ?? .

Investigations       ESR 50 CRP 10.9 Creat 140 LFTs: TB 5 TP 80 Alb 47 ALP 100 ALT 13 Hepatitis B & C : negative Uric Acid: 0.52 .5 U & E : Na 142 K 5 Ur 11.

Management   IAI shoulders & left trochanteric bursa MTX commenced .

Learning points    Treatment of PMR Monitoring of response to treatment Synovitis in PMR .

Polymyalgia Rheumatica      Clinical syndrome of the middle aged & elderly Characterized by pain & stiffness in the neck. shoulder & pelvic girdles First description probably made by Bruce in 1888 Barber suggested the present name in 1957 Paulley & Hughes reported 67 cases of PMR in patients with GCA .

Epidemiology    Not well defined Prevalence  3300 per 100 000 in pts > 65 yrs Affects middle aged & elderly   seldom diagnosed in pts under 50 yrs Mortality similar to general population .

Pathogenesis  Probably polygenetic  Environmental factors   Viral cause suspected. not confirmed Genetic influence .

Immunogenetics    prevalence in those of European background PMR associated with HLA class II genes    but varies from 1 population to another Relapsing PMR more common in pts with the HLA.DRB1*04 allelle Positive association with TNF -3 .

Clinical features    Mean age of onset 70 yrs F:M 2:1 Onset     Dramatic or insidious (weeks/ months) sometimes related to recent bereavement Constitutional symptoms – fever. LOW & depression PUO .anorexia. fatigue.

Musculoskeletal involvement  Pain & stiffness      Stiffness    Shoulder region & neck – most common Shoulder & pelvic girdles Distal involvement unusual Bliateral & symmetric predominant feature worse after rest Muscle pain   Often diffuse & worse with movement Pain at night is common .

tendons.Musculoskeletal involvement  Muscle strength   Muscle atrophy    Late stages Restriction of joint movement ( improves with steroids) Tenderness of involved structures including periarticular structures   Usually unimpaired eg bursae. joint casules Painful arc from subacromial bursitis .

Synovitis  Distal manifestations in about ½   Asymmetric peripheral arthritis           Reported incidence varies form 0 – 100% Knees. knees wrists & hands Synovial tissue : non-specific inflammatory changes Synovial fluid – mild inflammatory exudate Improve with steroids May occur in pts with PMR with a normal ESR . shoulders. sternoclavicular joints – most common Transient & mild Usually nonerosive Erosive changes in joints or sclerosis of SI joints reported Carpal tunnel syndrome Abnormal technetium pertechnate scintigrams: widespread uptake over joints esp. wrists.

Pitting oedema  Dorsum of hands & wrists. ankles & tops of feet .

Presence of just 3 confirms a sensitivity of 92% & specificity of 80% .Classification/ Diagnostic criteria  Cause unknown No single diagnostic test  Bird & Coworkers’ Criteria for the diagnosis of PMR         Age > 65 yrs ESR > 40mm/hr Bilateral upper arm tenderness Morning stiffness > 1 hour Onset of illness within 2 weeks Depression or weight loss or both Dx requires 3 of 7 listed features .

10 – 15% have positive temporal biopsy findings PMR observed in 40 –60% of pts with GCA . identical biopsy findings .Relationship between PMR & GCA      Closely related Spectrum of disease Similar age & sex distributions & systemic features. similar lab features & response to steroids In pts with PMR & no symptoms of GCA.

Differential Diagnosis  Joint disease     Bone disease   Osteomyelitis Muscle disease    OA esp. C spine RA Connective tissue disease Polymyositis Myopathy RS3PE  Infections         Eg infective endocarditis Hypothyroidism Neoplastic disease Multiple myeloma Leukemia Lymphoma Parkinsonsm Functional PMR is a diagnosis of exclusion .

Investigations  ESR: usually greatly increased    CRP – more sensitive indicator of disease activity Anaemia       usually mild hypochromic. less frequently  1 & globulin Abnormalities in TFT & LFTs  ALP common Liver biopsies – portal & intralobular inflammation with focal liver cell necrosis & small epitheloid granulomas  ACLA in a significant number . Occassionally a more marked normochromic anaemia Protein electrophoresis   7-20% have a normal ESR Nonspecific  in 2 globulin.

Treatment  Prednisone   Drug of choice Rapid response   Resolution of sympytoms after a few days Lack of response – rethink diagnosis .

4g MTX & Aza as steroid sparing agents  Overall consensus .Management . 33-50 % of patients discontinue steroids after 2 yrs of Rx Warn pts of expected Rx for at least 2 yrs Most stop after 4-5yrs Median cumulative dose of prednisone : 4.not very effective . Frequent relapses occurred with 10 mg dly.5-5.prednisone  Adequate dose for the first month   Little info on the rate of reduction after symptom control    Weekly decrements not > 5 mg More gradual when 10mg dly reached – 1mg every 2-4 weeeks Aim for a maintenance dose of < 10mg after 6 months Expected duration ?      prospective study by Kyle & Hazleman showed that PMR was well controlled with an initial dose of 20 mg dly.

Most pts require Rx for 3-4 yrs but withdrawal after 2 yrs is worth attempting Recurrence of symptoms requires an  in prednisone dose Aza & MTX have modest steroid sparing effects  In pts who cannot reduce prednisone dosage because of recurring symptoms or who develop serious steroid S/E .Treatment .5mg every 2 – 4 weeks to 10 mg dly then 1mg every 4-6 weeks ( or until symptoms return) 5-7mg dly for 6-12 months. Reduced by 2. Final reduction 1mg every 6-8 weeks.prednisone  Initial dose     Maintenance dose      10 – 20 mg initially for 1 month.

50 % may experience serious steroid S/Es  risk of osteoporosis   Assess risk of OP before starting Rx Calcium & vitamin D supplements (minimum) .Complications   20.

Relapses         30 – 50 % have spontaneous exacerbations Most likely within 1st 18 months of Rx No way of predicting pts at risk Occurs if steroid is reduced or withdrawn too quickly Dx made on clinical basis ESR & CRP often not increased Or may be ed as a result of other causes Monitor for relapses for 6/12 to 1 yr after stopping Rx .