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Giant Cell Arteritis&Polymyalgia Rheumatica. Olabambo Ogunbambi Consultant Rheumatologist Hull Royal Infirmary. Epidemiology Pathogenesis Clinical Features Investigations Imaging Mimics Treatment. Giant cell arteritis. Primary systemic vasculitis medium/large vessels

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giant cell arteritis polymyalgia rheumatica

Giant Cell Arteritis&Polymyalgia Rheumatica

Olabambo Ogunbambi

Consultant Rheumatologist

Hull Royal Infirmary

slide2

Epidemiology

  • Pathogenesis
  • Clinical Features
  • Investigations
  • Imaging
  • Mimics
  • Treatment
giant cell arteritis
Giant cell arteritis
  • Primary systemic vasculitis

medium/large vessels

involves aorta & main branches

  • First described by Hutchinson 1890
  • Histological features described by Horton et al 1932
epidemiology
Epidemiology
  • Most common vasculitis Europe/N america
  • Incidence increases with age
  • Women affected 2-3 times more commonly
  • Incidence increases with latitude
  • 17/million in North American populations of Scandinavian descent (over age 50)
  • <12/million in South European populations
  • Rare in blacks and Asians
pathogenesis
Pathogenesis

Still much uncertainty

Factors implicated

  • Age
  • Genetic factors
  • Infection(?)

seasonal variation incidence

pathogenesis1
Pathogenesis

Genetic factors

  • HLA

Association with HLA DRB1*04

  • TNF microsatellite polymorphisms
  • Functional variant VEGF gene
  • Polymorphisms in genes for IL-13, NOS2, TLR-4
pathogenesis2
Pathogenesis
  • Both innate and adaptive immune factors

implicated

  • Possible viral/other trigger

stimulates monocyte activation

  • Activated monocytes infiltrate adventitia of large arteries and recruit further monocytes/lymphocytes
  • Macrophages migrate to media and produce cytokines and growth factors responsible for damage to elastic lamina and intimal hyperplasia
slide8

Figure 1 Pathogenetic mechanisms operating in GCA

Salvarani, C. et al. (2012)Clinical features of polymyalgia rheumatica and giant cell arteritis

Nat. Rev. Rheumatol. doi:10.1038/nrrheum.2012.97

pathology
Pathology
  • Affects extracranial branches of carotid artery
  • All layers of arterial wall involved
  • Inflammatory lesions contain

activated T cells

dendritic cells

macrophages

giant cell cells

clinical features
Clinical features
  • Classic features related to artery involvement

- extracranial branches of carotid artery

  • Headache

-Sudden, severe, predominantly temporal

-May affect occipital, parietal, frontal areas

-often severe enough to disturb sleep

clinical features1
Clinical features

Jaw claudication

  • Occurs in 40-50% patients
  • Highly specific
  • Needs to be distinguished from jaw pain , TMJ dysfunction and trismus
  • Occasionally patients have intermittent claudication affecting tongue, swallowing muscles
temporal artery abnormalities
Temporal artery abnormalities
  • Decreased or absent pulses
  • Tenderness
  • Thickening
  • Nodules
  • Redness
clinical features2
Clinical features
  • Scalp tenderness

-occurs in 30-50%

  • Worse with brushing/combing hair
  • Occasional patients develop scalp necrosis
slide17

Scalp necrosis in giant cell arteritis.

Mackie S L , and Pease C T Postgrad Med J 2013;89:284-292

clinic features
Clinic features
  • Constitutional symptoms

fever, night sweats, weakness, weight loss

  • Less commonly seen compared to pre-steroid era
  • Patients with constitutional symptoms and high infl markers may be less likely to develop ischemic manifestations
ophthalmic complications
Ophthalmic complications

Frequency of occurrence

  • Opthalmology studies:

50% of patients

  • Rheumatology studies:

20-30% of patients

ophthalmic complications1
Ophthalmic complications

Anterior ischemic optic neuritis

  • Most common cause visual loss
  • Due to interruption of flow in posterior ciliary arteries
  • Presents as sudden painless visual loss
  • May present as mist in VF progressing to blindness in 24-48 hrs
  • Unilat visual loss may initially be missed by patient
  • May progress to contralat eye in 1-10 days
ophthalmic complications2
Ophthalmic complications

Other causes of visual loss

  • Central retinal art occlusion
  • Ischaemic retrobulbar neuropathy
  • Occipital infarction
ophthalmic complications3
Ophthalmic complications
  • Amaurosis fugax

-2-30% patients

-Best clinical predictor of visual loss

  • Diplopia

-ischemia of oculomotor nerve

-occurs in 5-6% patient

slide24

Ness, T; Bley, T A; Schmidt, W A; Lamprecht, P

The Diagnosis and Treatment of Giant Cell Arteritis

Dtsch Arztebl Int 2013; 110(21): 376-86; DOI: 10.3238/arztebl.2013.0376

clinical features3
Clinical features

Large vessel involvement

  • Distal ischemia
  • Limb claudication
  • Vascular bruits
  • May present as PUO
  • Aortic involvement possibly more common than recognised

-risk of aortic rupture/dilatation

clinical features4
Clinical features

Neurological manifestations

  • CVA
  • Mononeuropathies/polyneuropathies(rare)
clinical features5
Clinical features

Resp tract symptoms

(often missed)

  • Cough
  • Sore throat
  • Hoarseness
clinical features6
Clinical features
  • Audiovestibular dysfunction
  • Facial pain
  • Facial swelling
  • Odontogenic pain
  • Glossitis
  • Carotidodynia
investigations
Investigations
  • Elevated ESR/CRP/PV
  • Inflammatory markers usually abnormal
  • Usual to check both CRP and ESR (or PV)
investigations1
Investigations
  • High fibrinogen/haptoglobin
  • Thrombocytosis
  • Anemia of chronic disease
  • Elevated alkaline phosphatase
  • Anticardiolipin antibodies
temporal artery biopsy
Temporal artery biopsy
  • Considered Gold Standard
  • Recommended length > 2 cm
  • False neg

-Sampling error

-missed areas of inflammation

-Skipped lesions

-Arteritis limited to great arteries

  • Biopsy should be done preferably before treatment

Or soon as possible after starting treatment if required

temporal artery biopsy1
Temporal artery biopsy
  • What is a positive biopsy?

-Transmural changes only

-What about adventitial changes only?

-“Healed” arteritis?

possible confusion with age related changes

  • Bilateral biopsies?
imaging
Imaging
  • High resolution colour doppler US
  • Can visualise both lumen and vessel wall
  • Vessel wall features of presumed inflammation
  • Seen as hypoechogenic mural thickening

-”halo”

  • Dependent on equipment, operator
  • NB “halo” reported in normal patient, PAN
imaging1
Imaging
  • Other features

stenoses, occlusions

  • Sensitivity 88%, Specificity 78%
  • Precise role still not clearly defined
slide38

Figure 3 Ultrasonographical findings for GCA

Salvarani, C. et al. (2012)Clinical features of polymyalgia rheumatica and giant cell arteritis

Nat. Rev. Rheumatol. doi:10.1038/nrrheum.2012.97

slide39

a & b = normal artery

c & d= temporal arteritis

slide40
MRI
  • Can demonstrate mural inflammatory enhancement
  • Role in diagnosis?

Temporal artery involvement

Small studies: Sens 89-94%, Specificity 92-100%

  • May be useful for assessing large vessels
  • Role in monitoring?
slide41

C+D= Biopsy proven Giant Cell arteritis

Bley et al AJNR October 2007 28: 1722-1727

slide42

A 62-yr-old female patient with histologically validated GCA. Transverse contrast-enhanced, fat-suppressed, T1-weighted SE image at initial presentation (A) and after 10 months of corticosteroid treatment (C).

Bley T A et al. Rheumatology 2008;47:65-67

pet ct
PET-CT
  • Useful modality for assessing extent of disease involvement
  • May demonstrate subclinical vasculitis of great vessels
  • May provide information about response to treatment
  • Can only evaluate large arteries
  • Clinical utility still unclear
mimics differentials
Herpes zoster

Migraine

Basal skull lesions

Infiltrative retro orbital lesions

TIA

Mimics/differentials
  • Cluster headache
  • Cervical spondylosis
  • Sinus disease
  • Temporomandibular joint pain
  • Ear problems
  • CTD
  • Other systemic vasculitides
classification criteria
Classification criteria
  • Age at onset>50yrs
  • New headache
  • Temporal artery abnormality
  • Elevated ESR >50 (Westergren method)
  • Abnormal artery biopsy

Three or more features yield

Sensitivity 93.5%

Specificity 91.2%

  • Limited applicability in daily practice
predictors of neuro ophthalmic complications positive tab biopsy
Predictors of neuro ophthalmic complications/positive TAB biopsy

History

  • Jaw claudication
  • Diplopia

Physical exam

  • TA beading
  • TA prominence
  • TA tenderness
treatment
Treatment
  • Recommended starting regimens
  • Uncomplicated GCA

-no visual symptoms

-no jaw claudication

Start Prednisolone 40-60mg

treatment1
Treatment
  • Complicated

evolving visual loss or hx amaurosis fugax

  • IV methylpred 500mg-1g daily for three days
  • Then Prednisolone 60mg daily
treatment2
Treatment

Other issues

  • Bone protection

Bisphosphonate/calcium/vitamin D supplementation

  • PPI
  • Aspirin 75mg daily
tapering
Tapering
  • 40-60mg prednisolone (not <0.75 mg/kg) continued

for 4 weeks

(until resolution of symptoms and laboratory abnormalities)

  • Then dose is reduced by 10mg every 2 weeks to

20 mg

  • Then by 2.5mg every 2- 4 weeks to 10 mg
  • Then by 1mg every 1-2 months provided there is no relapse
monitoring
Monitoring

Frequency: Suggested review at Weeks 0, 1, 3, 6 then months 3, 6, 9, 12 in the first year

Features:

  • Headaches
  • Jaw and tongue claudication
  • Visual symptoms.
  • Vascular claudication of limbs, bruits, pulses
  • Blood pressure
  • Proximal pain and morning stiffness.
  • Disability related to GCA.
monitoring1
Monitoring
  • Full blood count, ESR/CRP, urea and electrolytes, glucose
  • Every two yrs-CXR(?)
  • Bone mineral density
management of relapse
Management of relapse
  • Headache: treat with the previous higher glucocorticosteroid dosage
  • Headache and jaw claudication: treat with 60mg prednisolone
  • Eye symptoms: treat with either 60mg prednisolone or IV methylprednisolone
steroid sparing agents
Steroid sparing agents

Limited evidence

Consider if recurrent relapses or difficulty reducing steroid dose

  • Methotrexate
  • Tocilizumab

small case series/case reports of efficacy

  • Cyclophosphamide
complications prognosis
Complications/prognosis
  • Generally runs self limited course
  • Overall survival similar to general population
  • Permanent partial/complete loss of vision in 15-20%
  • Inc risk CV events inc MI, CVA & PVD
  • Risk aortic dilatation/aneurysmal rupture
polymyalgia rheumatica
Polymyalgia rheumatica
  • Highest incidence in Northern Europeans & people of Scandinavian ancestry
  • 2-3 times more common than GCA
  • Occurs in 50% patients with GCA
  • 5-30 % of patients with PMR may develop GCA
  • Some pathogenetic similarity to GCA
polymyalgia rheumatica1
Polymyalgia rheumatica
  • Presentation with pain and stiffness of neck. Shoulder girdle and pelvic girdle usually at least 4 weeks duration
  • May be abrupt in onset
  • Symptoms and signs of systemic inflammation
  • Malaise, weight loss, low grade fever, swats
  • Elevated CRP/ESR.
  • Up to 20% may have normal ESR
clinical features7
Clinical features
  • Up to 50% distal MSK features
  • Mild distal synovitis, bursitis
  • Occasionally swelling/pitting edema of hands, wrists
  • Carpal tunnel syndrome
  • Subjective weakness
  • Constitutional symptoms
investigations2
Investigations
  • Elevated CRP &/or ESR(PV)
  • Nonspecific abnormalities in other tests
  • Anemia, elevated alkaline phosphatase
  • US & MRI can demonstrate bursitis and synovitis
  • PET CT may demonstrate subclinical vasculitis
differentials
Differentials
  • Rheumatoid arthritis
  • Remitting Seronegative Symmetrical Synovitis with Pitting Edema
  • Multifocal MSK problems
  • Bone disease
  • Inflammatory myositis
  • Fibromyalgia
  • Hypothyroidism
  • Parkinson’s disease
pmr treatment
PMR treatment
  • Dramatically responsive to steroids
  • Most response to Prednisolone <20mg/day
  • Dose gradually tapered
  • Tapering an art not science!
  • Monitor for relapse, features of GCA ,side-effects of GC
steroid sparing
Steroid sparing
  • Mostly conflicting and inconclusive data

Options tried include

  • Methotrexate
  • Biologics (anti-TNF agents)
  • Azathioprine
summary
Summary
  • GCA & PMR are closely related disorders affecting middle aged/older people
  • Unknown cause but genetic and enviromental factors influence pathogenesis
  • GCA primarily affects aorta and extracranial branches
  • In GCA biopsy is important in confirming diagnosis
  • GC are cornerstone of treatment
  • Significant associated morbidity
  • Some patients have chronic course and require GC for several yrs
question
Question

Jaw claudication

  • A. is pathognomonic of GCA
  • B. is defined by pain on chewing
  • C. signifies extensive involvement of branches of the external

carotid artery

  • D. is classified as an ischaemic feature of GCA
  • E. is never due to atherosclerosis alone

S. Mackie and C Pease. Postgrad Med J 2013;89:284-292

question1
Question

In the diagnosis of GCA

  • A. The American College of Rheumatology criteria are

useful diagnostic criteria in clinical practice.

  • B. Ophthalmological evaluation is necessary in the presence

of visual manifestations

  • C. Pain on opening the mouth is one of the typical ischaemic

manifestations of GCA

  • D. Jaw claudication is never caused by atherosclerosis
  • E. Aortic imaging should be routinely performed

S. Mackie and C Pease. Postgrad Med J 2013;89:284-292

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