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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


Giant cell arteritis polymyalgia rheumatica

  • 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


Giant cell arteritis polymyalgia rheumatica

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


Giant cell arteritis polymyalgia rheumatica

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


Giant cell arteritis polymyalgia rheumatica

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


Giant cell arteritis polymyalgia rheumatica

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


Giant cell arteritis polymyalgia rheumatica

a & b = normal artery

c & d= temporal arteritis


Giant cell arteritis polymyalgia rheumatica
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?


Giant cell arteritis polymyalgia rheumatica

C+D= Biopsy proven Giant Cell arteritis

Bley et al AJNR October 2007 28: 1722-1727


Giant cell arteritis polymyalgia rheumatica

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 GCA. Transverse contrast-enhanced, fat-suppressed, T1-weighted SE image at initial presentation (A) and after 10 months of corticosteroid treatment (C).

  • 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


Giant cell arteritis polymyalgia rheumatica

Patient presenting with PUO GCA. Transverse contrast-enhanced, fat-suppressed, T1-weighted SE image at initial presentation (A) and after 10 months of corticosteroid treatment (C).


Mimics differentials

Herpes zoster GCA. Transverse contrast-enhanced, fat-suppressed, T1-weighted SE image at initial presentation (A) and after 10 months of corticosteroid treatment (C).

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 GCA. Transverse contrast-enhanced, fat-suppressed, T1-weighted SE image at initial presentation (A) and after 10 months of corticosteroid treatment (C).

  • 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 biopsy

  • Recommended starting regimens

  • Uncomplicated GCA

    -no visual symptoms

    -no jaw claudication

    Start Prednisolone 40-60mg


Treatment1
Treatment biopsy

  • Complicated

    evolving visual loss or hx amaurosis fugax

  • IV methylpred 500mg-1g daily for three days

  • Then Prednisolone 60mg daily


Treatment2
Treatment biopsy

Other issues

  • Bone protection

    Bisphosphonate/calcium/vitamin D supplementation

  • PPI

  • Aspirin 75mg daily


Tapering
Tapering biopsy

  • 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 biopsy

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 biopsy

  • Full blood count, ESR/CRP, urea and electrolytes, glucose

  • Every two yrs-CXR(?)

  • Bone mineral density


Management of relapse
Management of relapse biopsy

  • 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 biopsy

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 biopsy

  • 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 biopsy

  • 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 biopsy

  • 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 biopsy

  • 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 biopsy

  • 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 biopsy

  • 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 biopsy

  • 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 biopsy

  • Mostly conflicting and inconclusive data

    Options tried include

  • Methotrexate

  • Biologics (anti-TNF agents)

  • Azathioprine


Summary
Summary biopsy

  • 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 biopsy

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 biopsy

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


Thank you

Thank you biopsy