Dr Toby Helliwell is a practising GP and Researcher at the Research Institute for Primary Care and Health Sciences. As part of the 2017 Musculoskeletal Education Day, he provides an update of the diagnosis on treatment of Polymyalgia Rheumatica and Giant cell Arteritis from a GP's perspective

1. It’s the Keele difference.
Dr Toby Helliwell
Delivering high quality multidisciplinary research in primary care.
Polymyalgia Rheumatica (PMR)
and Giant Cell Arteritis (GCA)
A GP Update

2. Delivering high quality multidisciplinary research in primary care.
Contents
• Introduction
• PMR: current guidance
• PMR: latest research
• PMR: challenges in General Practice
• GCA: current guidance
• GCA: latest research
• GCA: challenges in General Practice
• Discussion

3. Guess the diagnosis
• 65 year old lady with aching in both shoulders
• Takes 45 minutes to get going in the morning
and had started getting up early to account for
this
• Muscle aches and pain
• Impacting on activity, housework, hobbies and
struggling to do her part time job

4. Guess the diagnosis 2
• PMHx: Hypertension (on amlodipine 5mg)
• Examination
– Unable to lift arms above shoulder
– Bilateral upper arm tenderness
• CRP 43

5. What happened next
• Treated with 15mg prednisolone daily
• One month later:
– Feeling completely better
– Blood tests normal
• Six months later:
– Still feeling completely better

6. GP 22 (15, M, P)
“I mean, nobody ever comes in
saying that they think they’ve got
PMR……except those that have had it
before. Or those that have got, a
close relative or friend with PMR.”

7. A possibly more typical general
practice scenario
• 63 year old lady attending believing she has
PMR (she read an article in the Saga magazine),
several months of worsening pain and stiffness
significantly affecting the shoulders but both of
her hips too
• Noted recent muscle aches (no better after
stopping statin for 3 days)
• Worse in the morning
• Impacting on daily activity but has just tried to
put up with it

8. A possibly more typical general
practice scenario 2
• Known “wear and tear” in her right shoulder and
“arthritis” in her right hip
• PMHx: IHD (on aspirin), Pre-diabetes,
Hypertension (ramipril, amlodipine), hypothyroid,
overweight, on atorvastatin but wants to stop
because of muscle aches
• Additional information: Ex smoker, gave up after
recent flu like illness in the last few weeks with
residual on-going mild cough

9. A possibly more typical general
practice scenario 3
• Examination shows reduced ROM of shoulders,
chest clear, apyrexial
• CXR, possible mild early COPD
• CRP 17 (previous result normal)
• Convinces you to start 15mg of prednisolone
• After 1 week she is “cured” and so stops her
medication abruptly without a relapse in
symptoms.

10. Who gets PMR?
• Average age at onset is around 70yrs
• More common Northern Europe
– Incidence 112/100,000 Scandinavia
– Incidence 12.7/100,000 in Northern Italy
• Cause unknown
– Viral triggers?
– Genetic predisposition?
– Stress related?
– Medication?
• Over 80% of PMR patients are
exclusively diagnosed and managed by
their GP

11. What is PMR?
• Inflammatory rheumatic disorder
• Affects older patients (>50 years)
• 1/1000 people aged >50 develop PMR/year
• Often associated with Giant Cell Arteritis (GCA)
• More common in women than men
• Non-specific symptoms mimic other disorders
• No diagnostic blood test
• Diagnosis can be easily missed

12. BSR/BHPR guidelines

13. Process of inclusion and
identification

14. Features
• Bilateral shoulder pain and stiffness
• Hip girdle pain and stiffness
• Early morning stiffness
• Difficulties getting from chair/turning over in bed
• Systemic features
– Malaise
– Fever
– Weight loss
– Fatigue
• Inflammatory response

15. Features used for diagnosis
Information removed as it
contains unpublished data

16. Exclusion of other causes for
symptoms

17. Differential Diagnosis
• Rheumatoid athritis
• Osteoarthritis
• Shoulder Synovitis
• Rotator cuff
tendonitis
• Subdeltoid bursitis
• Capsulitis
• Depression
• Giant cell arteritis
• Hypothyroidism
• Polymyositis
• Chronic pain
syndrome
• Acute or chronic
infection
• Endocarditis
• Malignancy
• Parkinson
disease
• RS3PE

18. Making a diagnosis
• Challenging for GPs and specialists
• Lack of a diagnostic ‘gold standard’ test
• Non-specific signs and symptoms
•Exclusion of other
causes

19. Investigations

20. Important Exclusions

22. Is PMR associated with cancer?
• New evidence from Keele
• Data from general practice consulters
• 2877 PMR patients, 9942 non-PMR patients
• Higher rate of cancer diagnosis in PMR patients
• Statistically significant higher rate of cancer in the first 6
months after diagnosis
• No clear type of cancer associated
• True association or related to misdiagnosis?

23. Specialists have problems too…..

24. Specialists have problems too…..
• Testing classification criteria for research
• International experts identified 128 PMR
patients
• 10 didn’t have PMR
• 1/3 of the sample was difficult to classify
• Overlap of polymyalgic symptoms in
patients with a range of other disorders
• Specialists find making the diagnosis
challenging
Dasgupta et al, 2012. Ann Rheum Dis

27. General Practice Diagnosis

28. General Practice Managment

33. When to refer
• Age (consider if <60 years)
• Lack of shoulder involvement
• Lack of inflammatory stiffness
• Prominent systemic features,
weight loss, night pain,
neurological signs
• Features of other rheumatic
disease
• Normal or extremely high
acute-phase response
• Treatment dilemmas such as: .
– Incomplete, poorly sustained or
non-response to corticosteroids
– Inability to reduce
corticosteroids
– Contraindications to
corticosteroid therapy
– The need for prolonged
corticosteroid therapy (>2
years)

34. Barber
Annals of Rheumatic Diseases 1957
“It must be emphasised that the
diagnosis, based largely on negative
findings, can only be made after a
lengthy period of observation”

35. Giant Cell Arteritis
• Commonly coexists with PMR
• A diagnosis not to be missed
• Incidence 2/10,000 >50 years per year
• Similar geography / age / gender patterns to
PMR
• Visual damage often irreversible – occurs in 15-
20% patients

37. Giant Cell Arteritis
• ACR classification criteria
– Age >50 years
– New headache
– Abnormality of temporal arteries (beaded, tender,
pulseless)
– ESR >50 mm/hr
– Positive temporal artery biopsy
– 3 of the above – sensitivity 97%
Hunder et al, 1990,
Arthritis & Rheumatism

38. Giant Cell Arteritis
• Presentation often not classical
• Other symptoms
– Scalp tenderness
– Jaw claudication
– Visual disturbances
– Weight loss, fever, anorexia, malaise, depression

40. Challenges in Diagnosis

41. The Pit-falls
• Only 50% have typical temporal headache
• 25% have no headache/head pain at all
• Atypical features associated with significant
delays in diagnosis
• Atypical features associated with higher chance
of developing irreversible blindness

42. Challenges in Treatment

44. Take Home Messages (PMR)
• Careful follow up and or low threshold for
referral in cases of atypia
• Focused and vigilant follow up assessments
• Appropriate initiating prednisolone dose
• Review for treatment adverse effects and
associations

45. Take Home Messages (GCA)
• Consider the full range of features of GCA, not
just headache
• Review local pathways for further/specialist
assessment
• Initiate treatment if any delays in review

46. Discussion

47. Thank you
Research Institute for Primary Care and
Health Sciences
David Wetherall Building
Keele University
Newcaslte-under-Lyme
ST5 5BG
Tel: 01782 733905
Fax: 01782 734719
www.keele.ac.uk/pchs