1. Polymyalgia Rheumatica & Giant-cell Arteritis
Bill Cayley MD

2. Learning Objectives
Participants will have medical knowledge to describe
Pathophysiology of Polymyalgia Rheumatica (PMR) and Giant-cell Arteritis (GCA)
Evaluation for PMR and GCA
Management of PMR and GCA

3. Diane Z
Diane is an 85 year old woman who had “the flu” 6 months ago, and proximal muscle weakness since then. She is fatigued and her gait is a bit unsteady. Review of systems is otherwise normal.
PMH is significant only for Bell’s Palsy and PSVT.
FH is unremarkable. No history of rheumatologic disease.
SH indicates she lives alone but has family involved with care.
What else do you want to know?
Notes for instructor: Ask participants what additional items of the history they would pursue

4. Diane Z Physical Examination
WD WN older female in NAD. Weight is 152 # (stable).
HEENT: PERRLA, EOMI, Neck supple, no LA or TM.
Cardiac Regular, no murmurs or rubs.
Chest Clear with good air entry.
Abdomen soft, normal bowel sounds
Neuro and MSK exams normal.
What else do you want to know?
Notes for instructor: Ask participants what additional items of the physical exam they would pursue

5. Diane Z Labs: Hemoglobin 12.7 TSH .82 Lyme negative ESR 33
What is your diagnosis? Why?
Notes for instructor: Ask participants what their final diagnosis would be and why

6. Jane T
46 year old woman with headaches, stabbing “all over the head”, some starting from top of head, others from left temporal region which is tender when she touches it. Requests ESR.
PMH: GERD, Depression, DM, Htn, OSA.
SH indicates she is single, lives alone, works at local factory
ROS includes occasional heartburn, occasional leg swelling. Blood sugars are stable. No skin lesions.
What else do you want to know?
Notes for instructor: Ask participants what additional items of the history they would pursue

7. Jane T Physical Examination WD WN female in NAD. Wt 309 (stable).
HEENT: PERRLA, EOMI. Left temporal region tender on palpation. Oropharynx benign.
Cardiac Regular, Lungs Clear, Abdomen Soft.
What else do you want to know?
Notes for instructor: Ask participants what additional items of the physical exam they would pursue

8. Jane T
Labs:
A1C 5.2
K 3.8
Cr 0.7
ESR 44
What is your diagnosis? What’s next?
Notes for instructor: Ask participants what their final diagnosis would be and why

9. Jane T Temporal Artery Biopsy: Negative!
Notes for instructor: Ask participants how this affects their differential or final diagnosis

10. Overview Polymyalgia Rheumatica & Giant-cell Arteritis
Inflammatory disorders.
Constitutional symptoms with increased acute phase reactants.
The syndromes are considered to be different manifestations of inflammatory vasculitis.
PMR
Pain and stiffness in girdle muscles
GCA
Unilateral headache and temporal artery tenderness
Polymyalgia Rheumatica (PMR) & Giant-cell Arteritis (GCA) are interrelated inflammatory disorders. Both are characterized by consitutional symptoms with increased acute-phase reactants. PMR usually presents with severe stiffness and pain in the girdle muscles; true muscle weakness is generally not present, rather activity is limited by the discomfort associated with movement. GCA usually presents with unilateral headache and tenderness over the temporal artery. Both syndromes are thought to be common manifestations of a common underlying inflammatory process.
Source:
Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: (

11. Epidemiology Polymyalgia Rheumatica & Giant-cell Arteritis
Generally affect people over 50
One patient may have both
50% with GCA have PMR
10% with PMR have GCA
Incidence
GCA: 18 cases per 100,000 over 50
Men: 8/100,000 Women: 24/100,000
PMR: 500 cases per 100,000 over 50
Polymyalgia Rheumatica (PMR) & Giant-cell Arteritis (GCA) generally affect those over 50 years old. Up to 50 percent of patients with GCA may also have PMR, and 10 percent of patients with PMR may also have GCA. Peak incidence for both disorders is between age 70 and 80, and both are more common in northern latitudes.
Source:
Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: (

12. Pathophysiology Activation of cellular immunity?
Immune complexes deposited in arteries?
Speculative
Influenza, Hep B, B Burgdorferi
GCA
Vasculitis of head & neck large arteries
PMR
Vasculitis may or may not be present
The pathophysiology of GCA and PMR is unclear. Both are characterized by activation of cellular immunity, and inflammation may be due to immune complex deposition in arteries. Speculation has included influenza viruses, hepatitis B, and borrelia burgdorferi as possible inciting agents, but none of these agents has been conclusively linked to PMR or GCA. GCA is due to vasculitis of head and neck large arteries, while in PMR vasculitis may or may not be present.
Sources:
Epperly, TD, Moore KE, Harrover JD. Polymyalgia Rheumatica and Temporal Arteritis. Am Fam Physician 2000;62:789-96,801. (

13. Clinical Features of GCA
History
Consitutional symptoms:
Fever, night sweats, depression, malaise
Temporal or occipital headache
Jaw claudication, tongue pain
Mandibular or dental discomfort
Painless vision loss, amaurosis fugax, diplopia
Tinnitus, vertigo
Features of the clinical history suggesting GCA include constitutional symptoms such as fever, night sweats, depression, or malaise. Patient may also have a temporal or occipital headaache, and complain of jaw claudication, tongue pain, or dental discomfort with chewing. Visual symptoms include painless vision loss, amaurosis fugax, or diplopia, and patients may also have tinnitus or vertigo.
Source:
Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: (

14. Clinical Features of GCA
Examination and labs
Temporal or occipital scalp tenderness
Swollen temporal artery
Anemia
ESR greater than 50 mm/hour
Physical examination findings suggesting GCA include temporal or occipital scalp tenderness, and a swollen or “beaded” temporal artery. Patients may have anemia, or a sedimentation rate greater than 50 mm/hr.
Source:
Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: (

15. GCA: History, Exam and Lab
Finding
LR (+)
LR (-)
Jaw Claudication
4.2
.72
Diplopia
3.4
.95
Temporal Artery – “Beaded”
4.6
.93
Temporal Artery – Prominent
4.3
.67
Temporal Artery – Absent Pulse
2.7
.71
Temporal Artery – Tender
2.6
.82
ESR abnormal
1.1 .2
ESR > 100
1.9 .8
This chart lists the performance characteristics of individual elements of the history and physical examination for diagnosis of temporal arteritis.
Adapted from:
Smetana GW, Shmerling RH. Does this patient have temporal arteritis? JAMA Jan 2;287(1): PMID: (

16. Clinical Features of PMR
History
Consitutional symptoms:
Fever, night sweats, depression, malaise
Pain and stiffness in shoulder and/or pelvic girdle
Morning stiffness of 45 minutes or more
Difficulty getting up, doing ADLs
Clinical findings suggestive of PMR include constitutional symptoms such as fever, night sweats, depression, or malaise. Patients typically have pain or stiffness in the shoulder or hip girdle muscles, morning stiffness of 45 minutes duration or more, and difficulty getting up and about and doing ADLs.
Source:
Michet CJ, Matteson EL. Polymyalgia rheumatica. BMJ Apr 5;336(7647): PMID: (

17. Clinical Features of PMR
Exam
Affected muscle groups TENDER but not WEAK
Laboratory investigations
ESR > 40 mm/hour
On physical examination, patients with PMR typically have TENDERNESS in the shoulder or hip girdle muscles, but not true weakness. Laboratory investigations usually show an elevated sedimentation rate.
Source:
Michet CJ, Matteson EL. Polymyalgia rheumatica. BMJ Apr 5;336(7647): PMID: (

18. Differential Diagnoses
Giant-cell Arteritis
Dental conditions
Trigeminal neuralgia
Sinus disease
Otological conditions
Retinal vascular accident
Polymyalgia Rheumatica
Neoplastic disease
Cervical spondylosis
Rheumatoid arthritis
Connective tissue dz
Myeloma
Leukaemia
Osteomyelitis
Hypothyroidism
Miliary tuberculosis
Typical differential diagnoses to also consider when evaluating for GCA or PMR
Sources:
Meskimen S, Cook TD, Blake RL. Management of Giant Cell Arteritis and Polymyalgia Rheumatica. Am Fam Physician 2000;61:2061-8, (
Epperly, TD, Moore KE, Harrover JD. Polymyalgia Rheumatica and Temporal Arteritis. Am Fam Physician 2000;62:789-96,801. (
Swannell AJ. Polymyalgia rheumatica and temporal arteritis: diagnosis and management. BMJ May 3;314(7090): PMID: (

19. Laboratory Investigations
Erythrocyte Sedimentation Rate (ESR)
GCA
May be > 100
Sometimes only 40-50
Rarely normal
PMR
Generally > 40
Up to 20 % of patients may have normal ESR
Sources:
Meskimen S, Cook TD, Blake RL. Management of Giant Cell Arteritis and Polymyalgia Rheumatica. Am Fam Physician 2000;61:2061-8, (
Epperly, TD, Moore KE, Harrover JD. Polymyalgia Rheumatica and Temporal Arteritis. Am Fam Physician 2000;62:789-96,801. (
Swannell AJ. Polymyalgia rheumatica and temporal arteritis: diagnosis and management. BMJ May 3;314(7090): PMID: (

20. Erythrocyte Sedimentation Rate
The height of the layer of RBCs that settle in a tube of anticoagulated blood in one hour. Normals Men: Age/2 Women: (Age +10)/2
Inflammatory disorders produce abnormal proteins which bind to RBCs and make them "stick" together and settle out of the plasma more rapidly
Source:
Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: (
Brigden ML. Clinical utility of the erythrocyte sedimentation rate. Am Fam Physician Oct 1;60(5): PMID: (

21. Laboratory Investigations
Other tests to consider
Chemistries, glucose & liver enzymes
CBC - Normocytic anemia may be present
Rhematoid Factor & ANA (r/o RA)
TSH (r/o hypothyroidism)
Blood Cx (r/o endocarditis)
CPK (r/o polymyositis)
SPEP (r/o myeloma)
Other testing to consider when evaluating for GCA or PMR to rule out differential diagnoses.
Sources:
Michet CJ, Matteson EL. Polymyalgia rheumatica. BMJ Apr 5;336(7647): PMID: (
Meskimen S, Cook TD, Blake RL. Management of Giant Cell Arteritis and Polymyalgia Rheumatica. Am Fam Physician 2000;61:2061-8, (
Epperly, TD, Moore KE, Harrover JD. Polymyalgia Rheumatica and Temporal Arteritis. Am Fam Physician 2000;62:789-96,801. (
Swannell AJ. Polymyalgia rheumatica and temporal arteritis: diagnosis and management. BMJ May 3;314(7090): PMID: (

22. Biopsy for Giant-cell Arteritis
Biopsy shows
Severe intimal thickening
Reduction in vessel lumen
Internal elastic lamina is disrupted with fragmentation and sometimes destruction.
Normal biopsy appearances do not exclude the diagnosis because of possible skip lesions.
Temporal artery biopsy samples
Positive in 60-80% of patients with giant cell arteritis
Positive in only 15-20% of patients with PMR
Temporal artery biopsy for GCA may show severe intima thickening with reduction in the vessel lumen. However, a normal biopsy does not completely exclude the diagnosis of GCA. Additionally, while temporal artery biopsy may be positive in only 60-80% of patients with GCA, it may also be positive in up to 15-20% of patients with PMR.
Source:
Epperly, TD, Moore KE, Harrover JD. Polymyalgia Rheumatica and Temporal Arteritis. Am Fam Physician 2000;62:789-96,801. (

23. Diagnostic Criteria Diagnosis of GCA (ACR) Three or more of:
Age 50 years of age or older
New localized headache
Temporal artery abnormal
tenderness or decreased temporal artery pulse
ESR 50 mm/hr or greater
Abnormal temporal artery biopsy
Sensitivity: 93.5%
Specificity: 91.2%
Diagnosis of PMR
Age 50 years or older
1 month aching & morning stiffness in at least two of :
Shoulders & upper arms
Hips & thighs
Neck & torso
ESR 40 mm/hr or greater
Exclusion of other disease
Sources:
Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990;33: (see
Evans JM, Hunder GG. Polymyalgia rheumatica and giant cell arteritis. Clin Geriatr Med 1998;14:455-73

24. Management Corticosteroids are primary therapy To prevent bone loss
For GCA steroids should not be delayed to wait for biopsy
Temporal artery biopsy in GCA will remain abnormal for up to four weeks after starting steroids.
To prevent bone loss
Calcium w/ Vitamin D
Bisphosphonates
Corticosteroids are the primary therapy for PMR and GCA. For suspect, GCA steroids should not be delayed to wait for biopsy, since temporal artery biopsy specimens in GCA will remain abnormal for up to four weeks after starting steroids. To prevent bone loss from long-term steroids, patients should be advised to use Calcium w/ Vitamin D as well as bisphosphonates.
Source:
Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: (

25. Management - GCA Start with Prednisone 40-60 mg daily
If visual symptoms, give Solu-medrol 250 mg Q 6hrs for 3-5 days, then oral steroids
Once symptoms resolve and ESR normal (usually 2-4 weeks) begin slow taper
Reduce dose 10 % of daily dose every 2 weeks
Monitor symptoms and ESR for relapse
If relapse, hold taper until Sx/ESR normalize then resume slower taper.
Taper to 5 mg daily, then SLOW taper (18-24 mo)
For treatment of GCA, start prednisone mg daily. If the patient has visual symptoms, give Solu-medrol 250 mg Q 6hrs for 3-5 days, then change to oral steroids. Once symptoms resolve and the ESR is normal (usually 2-4 weeks) begin slow taper of steroids.
Sources:
Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: (
Meskimen S, Cook TD, Blake RL. Management of Giant Cell Arteritis and Polymyalgia Rheumatica. Am Fam Physician 2000;61:2061-8, (
Epperly, TD, Moore KE, Harrover JD. Polymyalgia Rheumatica and Temporal Arteritis. Am Fam Physician 2000;62:789-96,801. (

26. Management - PMR Corticosteroids usually provide immediate relief
Start Prednisone mg daily
Do not use higher doses
Absence of response suggests alternate diagnosis
Taper once Sx & ESR are stable 2-4 weeks
Then taper by 2.5 mg every 2-4 weeks to 10 mg daily
Then taper by 1 mg every 2-4 weeks to 5 mg daily
Then SLOW taper till off (18-24 mo)
Corticosteroids usually provide immediate relief of PMR. Start Prednisone at a dose of mg daily. Do not use higher doses of steroids, as absence of response to low-dose prednisone suggests an alternate diagnosis. Begin tapering prednisone once symtoms and ESR are stable for 2-4 weeks.
Sources:
Michet CJ, Matteson EL. Polymyalgia rheumatica. BMJ Apr 5;336(7647): PMID: (
Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: (
Meskimen S, Cook TD, Blake RL. Management of Giant Cell Arteritis and Polymyalgia Rheumatica. Am Fam Physician 2000;61:2061-8, (
Epperly, TD, Moore KE, Harrover JD. Polymyalgia Rheumatica and Temporal Arteritis. Am Fam Physician 2000;62:789-96,801. (

27. Long-Term Prognosis GCA & PMR tend to be self-limited GCA PMR Steroids
Relapse rates up to 25%
GCA
Blindness – 10% of untreated GCA
Increased risk of macrovascular disease - aortic aneurysms & dissection, stroke
PMR
Generally no long-term effects
Steroids
DM, Osteoporosis, Vertebral and Hip Fractures
GCA and PMR tend to be self-limited, though relapse rates up are up to 25%. Patients with GCA are at risk for blindness (rates as high as 10% if GCA is untreated). Patients with GCA are also at increased risk of macrovascular disease, including aortic aneurysms and dissection, and stroke. PMR generally has no long-term effects. Steroid therapy for GCA and PMR places patients at increased risk of diabetes, osteoporosis, and vertebral and hip fractures.
Sources:
Michet CJ, Matteson EL. Polymyalgia rheumatica. BMJ Apr 5;336(7647): PMID: (
Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: (
Meskimen S, Cook TD, Blake RL. Management of Giant Cell Arteritis and Polymyalgia Rheumatica. Am Fam Physician 2000;61:2061-8, (
Epperly, TD, Moore KE, Harrover JD. Polymyalgia Rheumatica and Temporal Arteritis. Am Fam Physician 2000;62:789-96,801. (

28. Follow-up – Diane Z Diagnosis of PMR
Age 50 years or older YES
Aching & stiffness
Shoulders and upper arms YES
Hips and thighs YES
Neck and torso
ESR > NO
Exclusion of other disease YES
Did not meet all dx criteria, but did have good response to Prednisone started at 15 mg daily and slowly tapered.

29. Follow-up – Jane T Diagnosis of TA Diagnosed as no TA
Three or more of the following:
Age 50 years of age or older NO
New onset of localized headache YES?
Temporal artery tenderness YES
ESR > 50 mm/hr NO
Abnormal temporal artery biopsy NO
Diagnosed as no TA
Tried Amitryptiline for chronic daily headache

30. Patient education FamilyDoctor.org
/common/pain/disorders/567.html
Patient UK

31. Selected References and Resources
Michet CJ, Matteson EL. Polymyalgia rheumatica. BMJ Apr 5;336(7647): PMID: ( Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: ( Weyand CM, Goronzy JJ. Giant-cell arteritis and polymyalgia rheumatica. Ann Intern Med Sep 16;139(6): PMID: ( Puttick MP. Rheumatology: 11. Evaluation of the patient with pain all over. CMAJ Jan 23;164(2): PMID: ( Smetana GW, Shmerling RH. Does this patient have temporal arteritis? JAMA Jan 2;287(1): PMID: ( assn.org/cgi/content/full/287/1/92)
Resources:
Michet CJ, Matteson EL. Polymyalgia rheumatica. BMJ Apr 5;336(7647): PMID: (
Unwin B, Williams CM, Gilliland W. Polymyalgia rheumatica and giant cell arteritis. Am Fam Physician Nov 1;74(9): PMID: (
Weyand CM, Goronzy JJ. Giant-cell arteritis and polymyalgia rheumatica. Ann Intern Med Sep 16;139(6): PMID: (
Puttick MP. Rheumatology: 11. Evaluation of the patient with pain all over. CMAJ Jan 23;164(2): PMID: (
Smetana GW, Shmerling RH. Does this patient have temporal arteritis? JAMA Jan 2;287(1): PMID: (
Meskimen S, Cook TD, Blake RL. Management of Giant Cell Arteritis and Polymyalgia Rheumatica. Am Fam Physician 2000;61:2061-8, (
Epperly, TD, Moore KE, Harrover JD. Polymyalgia Rheumatica and Temporal Arteritis. Am Fam Physician 2000;62:789-96,801. (
Swannell AJ. Polymyalgia rheumatica and temporal arteritis: diagnosis and management. BMJ May 3;314(7090): PMID: (