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 10.5
U & E : Na 142 K 5 Ur 11.9 Creat 140
LFTs: TB 5 TP 80 Alb 47 ALP 100 ALT 13
Hepatitis B & C : negative
Uric Acid: 0.52

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,

fatigue,anorexia, LOW & depression
PUO

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

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, tendons, joint casules

Painful arc from subacromial bursitis

Synovitis

Distal manifestations in about

Asymmetric peripheral arthritis

Reported incidence varies form 0 100%

Knees, wrists, 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. shoulders, 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

Pitting oedema

Dorsum of hands &

wrists, ankles & tops
of feet

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 . Presence of just 3

confirms a sensitivity of 92% & specificity of 80%

Relationship between PMR & GCA

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

Occassionally a more marked normochromic anaemia

Protein electrophoresis

7-20% have a normal ESR

Nonspecific in 2 globulin, 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

Treatment

Prednisone

Drug of choice
Rapid response

Resolution of sympytoms after a few days

Lack of response rethink diagnosis

Management - 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. Frequent relapses
occurred with 10 mg dly.

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 : 4.5-5.4g

MTX & Aza as steroid sparing agents

Overall consensus - not very effective

Treatment - prednisone

Initial dose

Maintenance dose

10 20 mg initially for 1 month,

Reduced by 2,5mg 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.
Final reduction 1mg every 6-8 weeks.
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

Complications

20- 50 % may experience serious

steroid S/Es
risk of osteoporosis

Assess risk of OP before starting Rx

Calcium & vitamin D supplements
(minimum)

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