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Giant Cell Arteritis ( GCA )

Giant cell arteritis GCA ( also known by other terms as Temporal arteritis , Horton or granulomatous arteritis ) is a large – and medium sized blood vessel systemic vasculitis characterized by the granulomatous involvement of the aorta and its main branches ( Salvarani C et al , Gonzalez-Gay MA et al )

 

Background –GCA is the most common primary vasculitides First references can be found in the 10th century when an oculist in Baghdad called Ali Ibn Isa noted a link between inflamed arteries and visual signs and symptoms reported in1890 as ‘ arteritis of the aged ‘ Affects large and medium sized vessels with a predisposition for cranial branches of carotid artery – in more than half of cases the aorta and its major branches are also involved GCA is an acute cause of blindness – hence it is a medical emergency The blindness is irreversible ( ischaemic optic neuropathy ) and GCA remains the prime medical emergency in ophthalmology RCP paper ( 2010 ) quotes that blindness is seen in up to 1/5th of patients and this may be due to late recognition There is considerable overlap with PMR ( Polymyalgia rheumatica ) and PMR with GCA is one of the most common reason for long term steroid treatment Features of PMR may be present before , simultaneously or after the diagnosis of GCA A fifth of PMR patients will actually have silent biopsy proven GCA and about half of GCA patients will have PMR symptoms ( Gonzalez-Gay MA et al ) Diagnosis and management of GCA remains a challenging task and the condition can be missed easily.

 

How common – Most common vasculitis in Europe and N America particularly in people > 70 It is not very well known how this condition affects people from other parts of the world as the prevalence is poorly reported globally Affects mostly whites and rarely seen before age 50 After 50 the incidence increases with age Peak incidence in the 7th and 8th decades Seen more commonly in women ( 2 : 1 ) and more commonly in people from Scandinavia CKS quotes annual incidence in UK of about 20 per 100.000 people A full time GP may expect to see 1 new case every 1-2 yrs Genetic predisposition has been reported.

 

What happens- Etiology is complex, studied widely and remains unclear Seasonal variations are known to occur and environmental causes as infections have been implicated Clinical manifestations are related to either tissue ischaemia from vascular lesions or systemic inflammation Histologically GCA shows granulomatous inflammation with lymphocytes , macrophages and giant cells ( fused macrophages ) in the vascular wall Our understanding of the pathogenesis is based on findings of temporal artery biopsy ( TAB ) samples Based on epidemiology it can be concluded that GCA pathogenesis is driven by multiple factors , including genetic substrate , sex and alterations of the immune and arterial systems related to ageing Role of immune mediated processes is thought to play a firm role in the pathogenesis via a systemic inflammatory response and maladaptive antigen-specific immune response

 

Presentation – Headache which starts abruptly
- most papers report that the headache is 
unilateral , localised and in the temporal region
- it can also be diffuse or bilateral
- it is mostly constant
- it responds poorly to analgesia Scalp pain Jaw and tongue claudication
- due to ischaemia of the chewing muscles
- jaw claudication may be intermittent and 
may happen in upto 1/2 of people with GCA Visual symptoms as
- amaurosis fugax
- blurring
- diplopia Temporal artery abnormalities as
- pain 
- nodules
- absent pulse Systemic symptoms as
- fever , weight loss , night sweats , tiredness , depression Claudication of extremities ( if aorta and braches of aorta are involved ) PMR features ( can be present in up to 50 % of cases ) Patients may also present with symptoms of TIA and stroke.

 

Examination – Palpate the temporal artery– this may show
- tenderness
- nodules
- swollen and thickened or beaded artery
- decreased or absent pulses
- erythema of the overlying skin Enquire about visual symptoms as
- transient visual loss 
- loss of vision which can be partial or complete in one or both eyes
- diplopia ( ischaemia of ocular muscles , nerves or brainstem)
, visual field defects , change to colour vision
- check VA , funduscopy , relative affarent papillary defect Auscultate ( for bruits ) arteries including the subclavian and axillary arteries , check peripheral pulses Check BP in both arms Look for upper cranial nerve palsies.

 

Differentials – Other causes of headache as migraine , cluster headache Transient ischaemic attack Herpes zoster Connective tissue disease Diseases of the cervical spine as
- spondylosis
- radiculopathy ( cervicogenic headache ) Other causes of acute visual loss as central retinal vein occlusion , non ischaemic optic neuropathy Glaucoma Serious underlying intracranial pathologies ENT conditions as
- temporomandibular

jt disorder
- sinusitis

 

Complications – eye

GCA can cause irreversible blindness and this is the most dreaded acute complication Loss of visual field or loss of vision which is painless , sudden and profound Papers quote that up to 70 % of the patients will suffer with eye involvement and if one eye is involved and not treated they will go blind in the contra-lateral eye in 60 % of cases Transient unilateral visual loss ( amaurosis fugax ) due to posterior circulation ischaemia is more common than b/l transient visual loss Without treatment the patient would suffer blindness in the other eye within 1-14 days Among the causes of eye manifestations the commonest is anterior ischaemic optic neuropathy ( AION ) due to inflammatory occlusion of the posterior ciliary arteries Diplopia tends to improve with treatment but loss of vision is permanent.

 

Other systems – Vasculitis of the coronary arteries 
-myocardial infarction
- congestive heart failure
- stroke
- peripheral arterial disease Aortic aneurysm rupture or aortic dissection Large artery stenosis Ischaemic brain damage Necrotic bowel segments.

 

Miscellaneous – Peripheral neuropathy Depression Confusion and encephalopathy Deafness Complications related to treatment with steroids.

 

Diagnosis – Diagnosis is challenging and would be established by experts for e.g rheumatologists – a high index of clinical suspicion and prompt referral to specialists is vital in primary care Most NHS trusts would have referral pathways for primary care , BSR guidance says that the patients should be evaluated by a specialist on the same working day if possible and within 3 working days in all cases A combination of history , physical examination and laboratory tests aid in the diagnosis but they are neither highly sensitive nor specific for GCA No single diagnostic test for GCA and no universally agreed diagnostic criteria- the ACR criteria are for study purpose and not diagnostic.

 

ESR ( often > 50 ) and CRP LFT FBC CKS recommends not to wait for the result of these tests before making a decision to refer or not as these may even be normal.

 

Temporal artery biopsy – remains the gold standard for diagnosis usually done under LA in OP settings well tolerated with SEs / complications not being problematic sensitivity of TAB is difficult to calculate false negative result is possible if adequate length is not taken treatment should not be delayed while a TAB is being organised- a positive biopsy is proof of GCA but a negative test does not rule out GCA.

 

Other imaging studies – Role of imaging has been studied extensively to aid in diagnosis and treatment. Access to these will vary based on availability , cost , expertise and local protocols , these can include
 colour coded duplex sonography high resolution MRI Positrion emission tomography ( PET )

 

Treatment – PeoPeople with suspected GCA should be immediately treated with oral prednisolone ( CKS )ple with suspected GCA should be immediately.

 

Do not wait for the result of urgent lab tests if strong clinical suspicion of GCA The aim of the treatment is to contain the inflammatory process Document diagnostically relevant symptoms and signs carefully and bloods should be taken before or immediately after commencing steroids The recommended dose advised by CKS is 
- people with visual symptoms 60 to 100 mg as a one off dose and an ophthalmologist review on the same day– people without visual symptoms give 40 to 60 mg but ensure that the dose is not less than 0.75 mg / kgtreated

 

Arrange a specialist review on the same day – BSR guidance says specialist review same day if possible or within 3 working days – discuss with Rheumatology / ophthalmology for advice and make a clear plan if same day assessment is not possible and document with oral prednisolone ( CKS )

References

  1. Diagnosis and management of giant cell arteritis Concise guidance to good practice series Royal College of Physicians, 2010
  2. Gonzalez‐Gay, M.A., Vazquez‐Rodriguez, T.R., Lopez‐Diaz, M.J., Miranda‐Filloy, J.A., Gonzalez‐Juanatey, C., Martin, J. and Llorca, J. (2009), Epidemiology of giant cell arteritis and polymyalgia rheumatica. Arthritis & Rheumatism, 61: 1454-1461. doi:10.1002/art.24459
  3. The prevalence of giant cell arteritis and polymyalgia rheumatica in a UK primary
    care population Max Yates1,2* , Karly Graham2 , Richard Arthur Watts2 and Alexander James MacGregor Yates et al. BMC Musculoskeletal Disorders (2016) 17:285
    DOI 10.1186/s12891-016-1127-3
  4. CKS Giant cell arteritis https://cks.nice.org.uk/topics/giant-cell-arteritis/
  5. Tracey, G. and Kevin, S. (2020), Investigation and management of giant cell arteritis. Prescriber, 31: 28-32. doi:10.1002/psb.1825
  6. Nekane Terrades-Garcia, Maria C Cid, Pathogenesis of giant-cell arteritis: how targeted therapies are influencing our understanding of the mechanisms involved, Rheumatology, Volume 57, Issue suppl_2, 1 February 2018, Pages ii51–ii62, https://doi.org/10.1093/rheumatology/kex423
  7. Sarah L Mackie, Christian Dejaco, Simone Appenzeller, Dario Camellino, Christina Duftner, Solange Gonzalez-Chiappe, Alfred Mahr, Chetan Mukhtyar, Gary Reynolds, Alexandre Wagner S de Souza, Elisabeth Brouwer, Marwan Bukhari, Frank Buttgereit, Dorothy Byrne, Maria C Cid, Marco Cimmino, Haner Direskeneli, Kate Gilbert, Tanaz A Kermani, Asad Khan, Peter Lanyon, Raashid Luqmani, Christian Mallen, Justin C Mason, Eric L Matteson, Peter A Merkel, Susan Mollan, Lorna Neill, Eoin O’ Sullivan, Maria Sandovici, Wolfgang A Schmidt, Richard Watts, Madeline Whitlock, Elaine Yacyshyn, Steven Ytterberg, Bhaskar Dasgupta, British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis: executive summary, Rheumatology, Volume 59, Issue 3, March 2020, Pages 487–494, https://doi.org/10.1093/rheumatology/kez664
  8. Giant cell arteritis: Always keep it in your head  Best Practice Journal > 2013 > BPJ: 53

  9. Giant cell arteritis: Current treatment and management
    Cristina Ponte, Ana Filipa Rodrigues, Lorraine O’Neill, Raashid Ahmed Luqmani World J Clin Cases 2015 June 16; 3(6): 484-494
    ISSN 2307-8960 (online) https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4468893/pdf/WJCC-3-484.pdf
  10. Giant cell arteritis
    S.S.L. Chew, N.M. Kerr, H.V. Danesh-Meyer *
    Department of Ophthalmology, Faculty of Medical and Health Sciences, The University of Auckland, Private Bag 92019, Auckland, New Zealand Journal of Clinical Neuroscience 16 (2009) 1263–1268
  11. Ameer MA, Peterfy RJ, Bansal P, et al. Temporal (Giant Cell) Arteritis. [Updated 2020 Sep 15]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459376/
  12. Giant Cell Arteritis by Stacy L Pineless, MD et al https://eyewiki.aao.org/Giant_Cell_Arteritis

  13. CONTINUING MEDICAL EDUCATIONThe Diagnosis and Treatment of Giant Cell ArteritisThomas Ness, Thorsten A. Bley, Wolfgang A. Schmidt, Peter Lamprecht Deutsches Ärzteblatt International | Dtsch Arztebl Int 2013; 110(21): 376−86 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3679627/pdf/Dtsch_Arztebl_Int-110-0376.pdf
  14. Guideline on diagnosis and treatment of giant cell arteritis By British Society for Rheumatology 24 July 2020

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