1. POLYMYALGIA RHEUMATICA AND
GIANT CELL ARTERITIS

2.  Polymyalgia rheumatica (PMR) and giant cell
arteritis (GCA) are two closely related inflammatory
syndromes.
 Occur in the same patient population, suggesting
common risk factors and pathogenic pathways.
 Both syndromes share laboratory abnormalities that
reflect a vigorous acute-phase response and are
critical for diagnosing and monitoring affected
patients.

3. GIANT CELL ARTERITIS
EPIDEMIOLOGY
 Incidence of GCA varies widely in different
populations, from less than 0.1 per 100,000 to 33
per 100,000 persons aged 50 years and older.
 Highest incidence figures found in Scandinavians
and in Americans of Scandinavian descent.
 The lowest incidence of GCA is reported in
Japanese, northern Indians, and African Americans.

4.  Women are affected about twice as often as men.
 Age is the greatest risk factor for developing either
condition.
 The incidence of GCA rises from 1.54 cases per
100,000 people in the sixth decade to 20.7 per
100,000 in the eighth decade.

5.  Experimental evidence supports a T-cell–mediated
immunopathology of GCA.
 B cells are not found within the arterial wall; no
pathognomic antibodies have been identifi ed; and
hypergammaglobulinemia is absent.
 Panarteritis of medium and large arteries is
combined with an intense systemic inflammatory
syndrome.
 Vasculitic lesions cause luminal occlusion and
tissue ischemia or aortic aneurysm.

6.  Preferentially targeted vascular beds include the
branches of the external carotid, subclavian,
common carotid, and vertebral arteries and aorta.

7. (1) MONONUCLEAR CELLS ENTER THE ADVENTITIA VIA THE VASA VASORUM, WHERE T CELLS
RECOGNIZE ANTIGENS AND PRODUCE
IFN-GAMMA.
(2) THE INFI TRATE ADVANCES TO THE MEDIA, WHERE MACROPHAGES AND GIANT CELLS
UNDERGO DIFFERENTIATION AND EXERT TISSUE-INJURIOUS EFFECTOR FUNCTIONS.
(3) THE ARTERY RESPONDS WITH NEOANGIOGENESIS AND INTIMAL HYPERPLASIA.

8. PATHOGENESIS OF GIANT CELL ARTERITIS.

9. SYMPTOMS AND SIGNS
GIANT CELL ARTERITIS
AMERICAN COLLEGE OF RHEUMATOLOGY
1990 CRITERIA FOR THE CLASSIFICATION OF
GIANT CELL ARTERITIS
1. Age at disease onset ≥ 50 years
2. Headache of new onset or new type
3. Tenderness or decreased pulsation of temporal
artery
4. Elevated erythrocyte sedimentation rate (≥50
mm/hr)
5. Histologic changes of arteritis (either
granulomatous lesions, usually with
multinucleated giant cells, or diffuse mononuclear
cell infiltration)

10. CLASSIC PRESENTING MANIFESTATIONS OF
GIANT CELL ARTERITIS
Symptoms Percentage of Cases
Headache 70
Jaw claudication 50
Constitutional symptoms 50
Polymyalgia rheumatica 40
Visual loss 20
Abnormal temporal artery 50
Anemia 80
Erythrocyte sedimentation rate >50 90
mm/h
Arthritis 15

11. ATYPICAL MANIFESTATIONS OF GCA ( PRESENT IN 40% OFlike SIADH
Fever of Respirator Neurologi Large Tumor CASES)
unknown y tract c artery lesions
origin symptoms symptoms involveme
nt
GCA causes Dry cough Mononeuriti Claudication Especially of
2% of all s multiplex in arms or the breasts
cases of fever legs and ovaries
of unknown
origin
accounts for Throat pain Stroke Unequal arm
16% of all blood
cases of fuo in pressures
patients over
the age of 65.
white blood Tongue pain Transient Thoracic
cell count is ischemic aortic
almost always attack aneurysm
normal
presenting Dementia
complaint in
1 of 25.

12. Giant cell arteritis presents with two major
symptomatic complexes,
A. Signs of vascular insufficiency resulting
from impaired blood flow .
B. Signs of systemic inflammation.
 Symptoms can wax and wane and resolve
temporarily, even in the absence of treatment.

13. Cranial Vasculature Extracranial Arteritis Systemic
Arteritis Inflammatory
Syndrome
Headaches Aortic Arch Syndrome Wasting
Syndrome
Ischemia of the Eye, Fever of Unknown
Brain Origin
Ischemia of the Malaise
Cranial nerves

14. CRANIAL ARTERITIS
SYMPTOMS
1. Headaches
2. Scalp tenderness
3. Jaw claudication
4. Tongue claudication
5. Visual loss
6. Transient ischemic attacks
7. Strokes

15. Headache : Diffuse or Localized, usually in the
temporal, occipital, or periorbital areas.
 severe, refractory to standard analgesics, and
interfere with sleep.
 Scalp tenderness : Localized over the temporal
and occipital arteries or diffuse

16. OCULAR COMPLICATIONS
 15% of patients experience ophthalmic
complications.
 Ischemia in the territory of the ophthalmic artery is
the leading cause of ocular problems.
Visual loss is
1. Pain free
2. Partial or complete
3. Unilateral or Bilateral
4. Irreversible.

17.  Most common cause is anterior ischemic optic
neuropathy resulting from occlusion of the posterior
ciliary arteries supplying the optic nerve.
 Ophthalmologic examination:pale disc edema
resulting from ischemic damage to the optic nerve
head

18. LARGE-VESSEL ARTERITIS AND AORTITIS
Stenotic lesions occur in
1) Distal subclavian arteries
2) Axillary arteries
3) Brachial arteries

19. AORTITIS
Aortic arch syndrome: Ischemia of the upper extremities
Pulseless disease.
 Ischemic pain during :Activities involving the arms, such
as brushing teeth, working overhead.
 Blood pressure readings are asymmetric, bilaterally
diminished, or absent.
 Raynaud’s phenomenon–like symptoms with
paleness, bluish discoloration, and dysesthesias
 Tissue gangrene, affecting the fingertips
 Bilateral, or unilateral involvement or asymmetric
patterning

20. SYSTEMIC INFLAMMATORY SYNDROME WITH
ARTERITIS
1. Fever. 15% of cases, fever of unknown origin is
the initial presentation
2. Malaise.
3. Fatigue.
4. Weakness.
5. Anorexia, Weight loss.
6. Depression.

21. CLINICAL SPECTRUM OF THE GIANT CELL ARTERITIS/
POLYMYALGIA RHEUMATICA SYNDROME.

22. LABORATORY FINDINGS
 The ESR averages about 100 mm/h in GCA.
 An ESR >30 mm/h is present in 96% of patients
with GCA, and an ESR of >50 mm/h is seen in 87%
of patients with GCA.
 The Creactive protein is also usually elevated and
may be more sensitive than the ESR in detecting
flares.

23.  The anemia, typically normochromic and
normocytic, is usually mild with a hematocrit often
in the 32–35 range.
 The platelet count, often elevated nonspecifically by
inflammatory disorders, is frequently increased in
GCA.

24.  Magnetic resonance angiography or computed
tomography angiography can provide noninvasive
assessment of larger artery disease.
 Positron emission tomography scanning can
demonstrate occult large-vessel inflammation.

25. IMAGING STUDIES
 Blood vessel imaging has gained importance as a
method for assessing the extent of vasculitis or
even for making a GCA diagnosis.
 In patients with the
subclavian, axillary, vertebral, carotid artery
involvement, imaging can establish the diagnosis.
 Detecting and monitoring aortic arch involvement
depend heavily on imaging procedures.

26.  Conventional radiographic angiography remains
superior for detailed assessment of vessel anatomy
and luminal status and is an absolute requirement
for preoperative evaluation.

27.  FDG-PET reportedly indicates inflammatory activity
in the vessel wall in GCA and PMR.
 Increased uptake of labeled glucose by
inflammatory cells provides the underlying
mechanisms of detection, but no properly designed
studies have been conducted to assess this
procedure’s specificity and sensitivity.
 Specifically, it is unknown whether subtle
inflammation in atherosclerotic lesions can be
distinguished from active arteritis in a patient
population expected to have widespread
atherosclerosis.

28. OCCLUSION OF THE AXILLARY-BRACHIAL JUNCTION. ANGIOGRAM SHOWING
IRREGULARITY OF THE RIGHT SUBCLAVIAN ARTERY WITH
OCCLUSION AT THE AXILLARY-BRACHIAL JUNCTION AND FORMATION OF COLLATERAL
VESSELS

29. DIGITAL SUBTRACTION IMAGE.

30. MAGNETIC RESONANCE ANGIOGRAM OF THE GREAT VESSELS
SHOWS NARROWING OF THE SUBCLAVIAN ARTERY DISTAL TO THE ORIGIN OF
THE VERTEBRAL ARTERY.

31. CONTRAST-ENHANCED CT IMAGE OF THE CHEST SHOWS PRONOUNCED ECTASIA OF
THE
ASCENDING AORTA, MINIMAL THICKENING, AND IRREGULARITY OF THE WALL.

32. MAKING A DIAGNOSIS
The diagnosis of GCA is suggested by the
1. Clinical picture
2. Elevated ESR
3. Proven by a positive temporal artery biopsy.
Patients with large artery involvement Subclavian
disease, are diagnosed by
A. Magnetic resonance imaging,
B. Computed tomography angiography, or
C. Conventional angiography showing long, smooth
arterial taperings uncharacteristic of
atherosclerosis

33. TEMPORAL ARTER BIOPSY SPECIMEN IS SHOWN.
CHARACTERISTIC CHANGES INCLUDE A PANMURAL MONONUCLEAR INFI LTRATE, DESTRUCTION
OF THE INTERNAL AND EXTERNAL ELASTIC LAMINAE, AND CONCENTRIC INTIMAL HYPERPLASIA.

34. TREATMENT
Corticosteroids
 Corticosteroids are highly effective in GCA
treatment.
 Initial doses of 60 mg prednisone or equivalent
have been recommended.
 Initial doses should be maintained until reversible
manifestations of the disease have responded and
the systemic inflammatory syndrome is suppressed.

35.  Under close monitoring for clinical signs of disease
reactivation, the dose of prednisone generally can
be tapered by 10% every 1 to 2 weeks.
 Aspirin is an important adjunctive treatment for
GCA patients without contraindications.

36. ADJUVANT THERAPY
 While on chronic corticosteroids, patients should be
monitored for bone mineral density, hypertension,
and diabetes mellitus.
 Measures to prevent osteoporosis include calcium
and vitamin D supplements, bone protective
therapy.

37. PROGNOSIS
 If diagnosed and treated promptly, progression of
the downstream effects of arterial wall
inflammation, in particular lumen occlusion with
tissue ischemia, can be prevented.
 In the majority of patients, GCA does not enter
remissions that are sustained indefi nitely after
discontinuation of glucocorticoids

39. POLYMYALGIA RHEUMATICA
 Polymyalgia rheumatica is a syndrome of pain and
stiffness, typically affecting proximal muscles of the
shoulder and pelvic girdle.
 PMR is frequently encountered in patients with
GCA in whom it may precede, follow, or accompany
manifestations of vasculitis.
 A small proportion (10%-20%) of patients with PMR
and no clinical evidence of vasculitis have frank
vascular inflammation on biopsy.

40. POLYMYALGIA RHEUMATICA:
DIAGNOSTIC CRITERIA
Chuang et al, 1982 Healey, 1984
1) Age at onset = 50 years or 1) Age at onset = 50 years or
older older
2) Erythrocyte sedimentation rate 2) Erythrocyte sedimentation rate
> 40 mm/hr > 40 mm/hr
3) Bilateral aching and stiffness 3) Pain persisting for ≥ 1 month
for ≥ 1 month and involving two and involving two of the
of the following areas: following areas: neck,
 Neck or Torso shoulders, and pelvic girdle
 Shoulders or Proximal regions 4) Absence of other diseases
of the arms capable of causing the
musculoskeletal symptoms
 Hips or Proximal aspects of
the thighs 5) Morning stiffness lasting more
than 1 hour
4) Exclusion of all other
diagnoses causing polymyalgia 6) Rapid response to prednisone
rheumatica–like symptoms (≤20 mg/day)

41.  PMR affects the same patient population as GCA,
but occurs approximately two to three times more
frequently.
 Women are affected more often than men, and the
diagnosis is extremely unlikely in individuals
younger than 50 years of age.

42.  High-risk populations: Scandinavians and other
peoples of Northern European descent.
 Annual incidence rates have been estimated at 20
to 53 per 100,000 persons over the age of 50
years.
 In low-risk populations, such as Italians, the annual
incidence rates for individuals aged 50 years and
older are only 10 cases per 100,000.

44. CLINICAL FEATURES
 Onset is abrupt
 Aching and pain in the muscles of the neck,
shoulders, lower back, hips, thighs, and
occasionally the trunk.
 Myalgias are Symmetrical.
 Nocturnal pain.
 Weight loss, anorexia, malaise, and depression are
common.

45.  Patients with PMR must be carefully evaluated for
possible GCA.
 A negative temporal artery biopsy does not exclude
the possibility of large vessel vasculitis targeting
primarily the subclavian and axillary arteries and
the aorta.

46.  Signs of vascular insufficiency, including
claudication in the extremities, bruits over
arteries, and discrepant blood pressure readings
should alert the physician to the possibility of GCA .
 MRA can be helpful in confirming the concomitant
diagnosis of large vessel vasculitis.

47.  Biceps tendonitis and glenohumeral synovitis may
also be present.
 Ultrasonography reveals fluid accumulation in the
bursae; T2-weighted MRI shows thickening and
edema.

48. DIFFERENTIAL DIAGNOSIS
1) Arthropathies
2) Shoulder disorders
3) Inflammatory myopathies
4) Hypothyroidism
5) Parkinson’sdisease
6) Malignancies
7) Infections.
8) Lack of the typical and impressive improvement upon
initiation of therapy can provide a clue towards
reevaluating the diagnosis of PMR

49. DISEASE ENTITIES WITH
POLYMYALGIAS
1) Rheumatoid arthritis
2) Rotator cuff syndrome
3) Osteoarthritis of shoulder and hip joints
4) Fibromyalgia
5) Polymyositis/dermatomyositis
6) Spondyloarthritis
7) Systemic lupus erythematosus
8) Vasculitides
9) Paraneoplastic myalgias
10) Infection-associated myalgias
11) Statin therapy
12) RS3PE (remitting seronegative symmetric synovitis and
pitting edema)
13) Parkinson’s disease
14) Hypothyroidism

50. TREATMENT
 Polymyalgia rheumatica is dramatically responsive
to glucocorticoid therapy.
 Two thirds of patients can be expected to respond
with remission of pain and stiffness when started on
20 mg/day or less prednisone.
 Some patients will need doses as high as 40
mg/day for complete clinical control.

51.  Patients initially controlled on 20 mg/day of
prednisone can usually taper the dose by 2.5 mg
every 10 to 14 days.
 In many patients, PMR can go into long-term
remission, and prednisone can be discontinued.

52. PROGNOSIS
 The prognosis of patients with PMR is good.
 In the majority of patients, the condition is self-
limited.
 A proportion of patients will eventually present with
typical symmetrical polyarthritis, fulfilling the criteria
for the diagnosis of seronegative rheumatoid
arthritis.
 Such patients may require disease-modifying
antirheumatic drug (DMARD) therapy.