Giant Cell Arteritis

The elusive enemy: Piecing together the diagnosis puzzle

The difficulty making the right diagnosis is to bring together individual symptoms, that can be very unspecific, and then to identify GCA from this symptom complex.

GCA is considered a little-known disease. Yet it affects at least 200 per 100,000 persons in the USA and even more in northern Europe.1,11,12 Those affected are almost exclusively over the age of 50 with peak incidence in the eighth decade of life.1 Women are also more often affected than men – three to one.1

A diagnosis is often delayed or incorrect because symptoms can be confused – by the patient and by doctors – with those of other common conditions, particularly those related to getting older. Patients can be treated for a host of different ailments from migraines and coughs, to allergies and even cancer.1 Some people suffer for weeks, months or even longer, often going back and forth to their doctor, before they are told they have GCA.1,13

Cheryl Stookey, from Charlestown, Massachusetts, USA, experienced exactly this situation. “After each step I would always go back to my primary care doctor who would lead me some place else... and weeks were going by. He finally sent me to a neurologist who in less than five minutes knew exactly what I had because she asked me the right questions.”

Hindsight is always great, you can look back and you can see all the mistakes that were made.
Cheryl Stookey Patient

Ensuring the public and doctors are aware of how to piece together the various signs and symptoms of GCA is the only way to get an early and accurate diagnosis. This could be the difference between someone going blind, suffering a devastating stroke or a dangerous aneurysm, or not.

“Early diagnosis is really the key determinant of outcome. Unfortunately, despite the fact that we may have treatment options, 15 to 20 percent of patients still have vision loss at the time they present and are diagnosed,” confirms Dr Stone.

The difficulty in making the right diagnosis

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Giant cell arteritis is a highly inflammatory disease.
It can present with many different symptoms.1

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The difficulty in making the right diagnosis is to bring together
individual symptoms
, that can be very unspecific.1

image

There is a clear need to improve communication
between the different specialties
involved to diagnose...

image

To get a formal diagnosis, two blood tests are taken to help assess levels of inflammation in the body. One is erythrocyte sedimentation rate (ESR) and the other C-reactive protein (CRP), the levels of which are both raised in GCA.10

A biopsy of the temporal artery in the side of the head can also be carried out.1,10 But this can be inaccurate because, depending on what part of the artery is examined, the ‘snapshot’ of their disease could be completely different at different times.1 Experts liken the biopsy to taking a photo of a butterfly at different stages of its development – you could be looking at a caterpillar, cocoon or butterfly – all very different things, albeit from the same origin.

A number of scans can also be carried out to look at arteries in the body to see if they are inflamed.2

Once diagnosed, a specialist works with the patient to agree a treatment plan to manage symptoms and limit the long-term damage that GCA can cause.

References

  • 1. Bhat S, et al. Giant cell arteritis. Midlife and Beyond, GM. Rheumatology. 2010; 071-079.
  • 2. Ponte C, et al. Giant cell arteritis: Current treatment and management. World J Clin Cases, 2015; 3(6): 484-494.
  • 3. Hunder G. History of giant cell arteritis (GCA) polymyalgia rheumatic (PMR). Rheumatology. 2005; 44(suppl 3):iii1.
  • 4. Hunder G et al. The American College of Rheumatology 1990 Criteria for the Classification of Giant Cell Arteritis. Arthritis & Rheum. 1990; 33(8):1122-1128.
  • 5. Salvarani C., et al. Laboratory investigations useful in giant cell arteritis and Takayasu’s arteritis. Clin Exp Rheumatol 2003; 21(Suppl. 32):S23-S28.
  • 6. Chatterjee S., et al. Clinical Diagnosis and Management of Large Vessel Vasculitis: Giant Cell Arteritis. Curr Cardiol Rep (2014) 16:498. DOI 10.1007/s11886-014- 0498-z.
  • 7. Mazlumzadeh M., et al. Treatment of giant cell arteritis using induction therapy with high-dose glucocorticoids: a double-blind, placebo-controlled, randomized prospective clinical trial. Arthritis Rheum. 2006;54(10):3310-8.
  • 8. Villiger P., et al. Tocilizumab for induction and maintenance of remission in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial. The Lancet. Published online 4 March 2016. http://dx.doi.org/10.1016/S0140-6736(16)00560-2.
  • 9. GCA pathophysiology. BMJ best practice. [Internet; cited 2017 Nov 7]. Available at: http://bestpractice.bmj.com/best-practice/monograph/177/basics/pathophysiology.html
  • 10. Giant cell arteritis. NICE CKS. [Internet; cited 2017 Nov 7]. Available at: https://cks.nice.org.uk/giant-cell-arteritis#!scenario.
  • 11. Lawrence C, et al. Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States. Arthritis & Rheum. 1998;41:778-799.
  • 12. Noltorp S, et al. High incidence of polymyalgia rheumatica and giant cell arteritis in a Swedish community. Clin Exp Rheumatol 1991;9:351-355.
  • 13. Prior JA, et al. Diagnostic delay for giant cell arteritis – a systematic review and meta-analysis. BMC Medicine. 2017; 15:120.