Polymyalgia Rheumatica

pitfalls in diagnosis
PMR & GCA
  Closely linked conditions

 ? Different phases of the same disease

PMR ↔ GCA
 there is In situ production of cytokines
in the temporal arteries of patients
with PMR who do not have histological
evidence of arteritis

 This suggests the presence of a

subclinical vasculitis in PMR
Clinical features of PMR
 Systemic:
 Fever
 Anorexia
 Malaise
 Weight loss
 May present as PUO
 Proximal:
 Stiffness & pain in
 Shoulders
 Neck

 Hips

 Worse with activity

 Difficulty getting out of bed
 Distal:
 Non-erosive arthritis (wrists & knees)
 CTS
 RS3PE
Clinical features of GCA
 Headache: temporal / occipital
 Scalp tenderness
 ? Thickened, nodular, tender TA
 ? ↓ / absent pulse
 Jaw claudication: internal maxillary artery
 Tingling tongue: lingual artery
 Amaurosis fugax
 Unilateral permanent loss of vision  other eye
affected within 1-2 wks (ophthalmic & posterior
ciliary arteries)
 Diagnosis:
 ESR  /CRP - normal in 10%
 Hb. -anaemia of “chronic disease”
 Alk.phos.
 TA Bx
 Fragmentation
 of internal
elastic lamina
 
Inflammatory infiltrate
 
giant cell
Diagnostic criteria of PMR
Bird et. al:
 Age > 65
 ESR > 40
 Onset within 2 weeks
 Bilateral shoulder pain &/or stiffness
 Morning stiffness > 1 hour
 Bilateral upper arm tenderness
 Depression &/or Wt loss
 Diagnosis requires Any 3 of 7:

 Sensitivity 92%

 Specificity 80%
Problems in applying diagnostic criteria

 40% have symptoms>3 months before

referral

 20% delay in hospital diagnosis>1 month

 10% PMR with normal ESR

Sensitivity 92%
FN = 1 – sensitivity = 8%

Specificity 80%
FP = 1 – specificity = 20%
conditions frequently confused
with polymyalgia?
Shoulder pain & stiffness
 Frozen shoulders
 Rheumatoid arthritis
 Fibromyalgia
 Myxoedema
Proximal weakness &
tiredness
 Polymyositis
 Thyrotoxic myopathy
 Osteomalacia
 Carcinomatous myopathy
Raised ESR
 Myeloma
 Malignancy
 Prospective study of 50
patients in Norway with PMR
symptoms, followed up between
1995-1997

Haugeberg et.al
PMR 40
TA 2
collaginosis 1
coxarthosis 1
Shoulder tendinitis 1
Prostate ca 1
Liver mets unknown 1° 1
myelodysplasia 2
Lymphoma 1
Total 50
Haugeberg,et. al
The observed frequency of malignancy
in these patients was compared with the
frequency of expected malignancy in the
Norwegian population over the same
period adjusted for age and sex, given
by the national cancer registry
 Age & sex-adjusted frequency of
malignancy in general population =
1.6%
 Frequency in study population = 10%
 A two-tailed Fisher’s exact test:

P = 0.0013
PMR 40
TA 2
collaginosis 1
coxarthosis 1
Shoulder tendinitis 1
Prostate ca 1
Liver mets unknown 1° 1
myelodysplasia 2
Lymphoma 1
Total 50
Haugeberg,et. al
 Total non (PMR, TA) = 8 = 16%
 Specificity = 80%
 FP = 20%
PMR, A tricky disease
 May present as PUO
 May have normal ESR
 May mimic hidden malignancy
 EORA may present as PMR: Anti-CCP
may differentiate
 GCA May present with different
arterial syndromes
Features raising suspicion of
other diagnoses:
S Siebert et. al

 younger presenting age(< 65)

 normal inflammatory markers
 non-response to low dose steroids
 persistently raised ALP
 Patients with suspected PMR should
have baseline: FBC, ESR, CRP, U&Es,
LFTs TFT, Ca, CK, serum
electrophoresis and dipstick urinalysis

 ALP should ↓ to normal after 3/52

treatment, if it does not investigate
further
 Failure to respond to steroids should raise
doubts about the diagnosis of PMR

 A good response to steroids does not

necessarily confirm the diagnosis

 The presence of any atypical features should

prompt more detailed investigation