Giant cell arteritis

Source: Wikipedia, the free encyclopedia.
Giant cell arteritis
Other namesTemporal arteritis, cranial arteritis,
bisphosphonates, proton-pump inhibitor[4]
PrognosisLife expectancy (typically normal)[4]
Frequency~ 1 in 15,000 people a year (> 50 years old)[2]

Giant cell arteritis (GCA), also called temporal arteritis, is an

blindness, as well as aortic dissection, and aortic aneurysm.[4] GCA is frequently associated with polymyalgia rheumatica.[4]
It can be confirmed by biopsy of the temporal artery in about 90% of people.

The cause is unknown.

biopsy of the temporal artery.[4] However, in about 10% of people the temporal artery is normal.[4]

Treatment is typical with high doses of

bisphosphonates to prevent bone loss and a proton-pump inhibitor to prevent stomach problems.[4]

It affects about 1 in 15,000 people over the age of 50 per year.

northern European descent are more commonly affected.[5] Life expectancy is typically normal.[4] The first description of the condition occurred in 1890.[1]

Signs and symptoms

Common symptoms of giant cell arteritis include:

The inflammation may affect blood supply to the

blindness may occur. In 76% of cases involving the eye, the ophthalmic artery is involved, causing arteritic anterior ischemic optic neuropathy.[13]

Giant cell arteritis may present with atypical or overlapping features.[14] Early and accurate diagnosis is important to prevent ischemic vision loss. Therefore, this condition is considered a medical emergency.[14]

While studies vary as to the exact relapse rate of giant cell arteritis, relapse of this condition can occur.[15] It most often happens at low doses of prednisone (<20 mg/day), during the first year of treatment, and the most common signs of relapse are headache and polymyalgia rheumatica.[15]

Associated conditions

The

varicella-zoster virus (VZV) antigen was found in 74% of temporal artery biopsies that were GCA-positive, suggesting that the VZV infection may trigger the inflammatory cascade.[16]

The disorder may co-exist (in about half of cases) with polymyalgia rheumatica (PMR),[12] which is characterized by sudden onset of pain and stiffness in muscles (pelvis, shoulder) of the body and is seen in the elderly. GCA and PMR are so closely linked that they are often considered to be different manifestations of the same disease process. PMR usually lacks the cranial symptoms, including headache, pain in the jaw while chewing, and vision symptoms, that are present in GCA.[17]

Giant cell arteritis can affect the aorta and lead to aortic aneurysm and aortic dissection.[18] Up to 67% of people with GCA having evidence of an inflamed aorta, which can increase the risk of aortic aneurysm and dissection.[18] There are arguments for the routine screening of each person with GCA for this possible life-threatening complication by imaging the aorta. Screening should be done on a case-by-case basis based on the signs and symptoms of people with GCA.[18]

Mechanism

The pathological mechanism is the result of an inflammatory cascade that is triggered by an as of yet undetermined cause resulting in

IL-6 inhibitors also play a suppressive role.[18]

Diagnosis

Physical exam

Intermediate magnification micrograph showing giant cell arteritis in a temporal artery biopsy. The arterial lumen is seen on the left. A giant cell is seen on the right at the interface between the thickened intima and mediaH&E stain

Laboratory tests

Biopsy

Histopathology of giant cell vasculitis in a cerebral artery. Elastica-stain.

The

negative result does not definitively rule out the diagnosis; since the blood vessels are involved in a patchy pattern, there may be unaffected areas on the vessel and the biopsy might have been taken from these parts. Unilateral biopsy of a 1.5–3 cm length is 85-90% sensitive (1 cm is the minimum).[23] Characterised as intimal hyperplasia and medial granulomatous inflammation with elastic lamina fragmentation with a CD 4+ predominant T cell infiltrate, currently biopsy is only considered confirmatory for the clinical diagnosis, or one of the diagnostic criteria.[11]

Medical imaging

Radiological examination of the temporal artery with ultrasound yields a halo sign. Contrast-enhanced brain MRI and CT are generally negative in this disorder. Recent studies have shown that 3T MRI using super high resolution imaging and contrast injection can non-invasively diagnose this disorder with high specificity and sensitivity.[24]

Early recognition

Women and men approximately 45 years old and who suffer from several complaints (at least 5 of the 16 symptoms)[25] listed below could have giant cell arteritis.

  • Fatigue and apathy
  • Stiffness in joints and/or muscles
  • Painful jaws when chewing
  • Sensitive scalp
  • Physical malaise and/or weakness
  • Bloated arteries of the temples
  • Headaches, migraine
  • Tongue problems
  • Bleakness, depression
  • Changed eyesight
  • Poor or lack of appetite
  • Reduced eyesight, blindness
  • Unusual loss of weight
  • A temperature
  • Unusual perspiration
  • Night sweats

Treatment

GCA is considered a

diabetes mellitus, osteoporosis, avascular necrosis, glaucoma, cataracts, cardiovascular disease, and an increased risk of infection.[30][31] It is unclear whether adding a small amount of aspirin is beneficial or not as it has not been studied.[32] Injections of tocilizumab may also be used.[33] Tocilizumab is a humanized antibody that targets the interleukin-6 receptor, which is a key cytokine involved in the progression of GCA.[34] Tocilizumab has been found to be effective at minimizing both recurrence, and flares of GCA when used both on its own and with corticosteroids.[34] Long term use of tocilizumab requires further investigation.[34][35] Tocilizumab may increase the risk of gastrointestinal perforation and infections, however it does not appear that there are more risks than using corticosteroids.[34][35]

Epidemiology

Giant cell arteritis typically only occurs in those over the age of 50;[4] particularly those in their 70s.[21] It affects about 1 in 15,000 people over the age of 50 per year.[2] It is more common in women than in men, by a ratio of 2:1,[4] and more common in those of Northern European descent, as well as in those residing further from the Equator.[5]

Disease impact

Giant cell arteritis and its treatment impact on people's lives because of symptoms, adverse effects of GCs and disruption to normal life.[36] People with GCA have previously ranked ‘losing sight in both eyes permanently’, ‘having intense or severe pain’ and ‘feeling weak, tired or exhausted’ as important quality of life domains.[37] Generic measures of disease impact such as SF36 may not always capture the disease specific aspects of GCA impact such as visual loss or systemic complications.[38] The Outcome Measures in Rheumatology (OMERACT) Large Vessel Vasculitis Working Group have identified the need for a disease-specific patient-reported outcome measure (PROM) for GCA.[39] Recently, a new disease specific measure of health-related quality of life in GCA has been developed.[40][41] The GCA-PRO has been shown to have robust validity and reliability in a cross-sectional study and can discriminate between different sub-groups of patients. This is likely going to help to capture the impact of disease and treatment in clinical trials and clinical practice.[41]

Terminology

The terms "giant cell arteritis" and "temporal arteritis" are sometimes used interchangeably, because of the frequent involvement of the temporal artery. However, other large vessels such as the aorta can be involved.[42] Giant-cell arteritis is also known as "cranial arteritis" and "Horton's disease".[43] The name (giant cell arteritis) reflects the type of inflammatory cell involved.[44]

References

  1. ^ from the original on 2017-10-22.
  2. ^ a b c d e "Orphanet: Giant cell arteritis". www.orpha.net. Archived from the original on 14 September 2017. Retrieved 14 September 2017.
  3. ^ a b "Giant Cell Arteritis". National Institute of Arthritis and Musculoskeletal and Skin Diseases. 13 April 2017. Archived from the original on 22 October 2017. Retrieved 21 October 2017.
  4. ^
    PMID 24988557
    .
  5. ^ from the original on 2017-10-22.
  6. from the original on 2017-10-22.
  7. .
  8. ^ "Giant Cell Arteritis". National Institute of Arthritis and Musculoskeletal and Skin Diseases. 13 April 2017. Archived from the original on 22 October 2017. Retrieved 21 October 2017.
  9. PMID 18711459
    .
  10. .
  11. ^ .
  12. ^ a b Hunder GG. "Polymyalgia rheumatica and giant cell (temporal) arteritis". uptodate.com. Wolters Kluwer. Archived from the original on 25 September 2015. Retrieved 23 September 2015.
  13. ^ Hayreh (April 3, 2003). "Ocular Manifestations of GCA". University of Iowa Health Care. Archived from the original on 2007-10-25. Retrieved 2007-10-15.
  14. ^
    PMID 25288850
    .
  15. ^ a b "UpToDate". www.uptodate.com. Retrieved 2019-11-25.
  16. PMID 25695965
    .
  17. ^ "Giant cell arteritis". BMJ Best Practice. Retrieved 2019-11-25.
  18. ^
    S2CID 3747982
    .
  19. .
  20. ^ a b c d e "Giant Cell Arteritis". The Lecturio Medical Concept Library. 26 November 2020. Retrieved 23 July 2021.
  21. ^
    PMID 31970405
    .
  22. .
  23. .
  24. .
  25. ^ "Symptomatix Home". www.symptomatrix-eng.eu. Retrieved 2023-05-07.
  26. PMID 16987903
    .
  27. ^ "BestBets: Steroids and Temporal Arteritis". Archived from the original on 2009-02-27.
  28. PMID 11520757
    .
  29. .
  30. .
  31. .
  32. .
  33. ^ "Press Announcements - FDA approves first drug to specifically treat giant cell arteritis". www.fda.gov. Retrieved 10 February 2018.
  34. ^
    S2CID 4227514
    .
  35. ^ .
  36. .
  37. .
  38. .
  39. .
  40. .
  41. ^ .
  42. .
  43. .
  44. ^ "giant cell arteritis" at Dorland's Medical Dictionary

External links

  • Mackie SL, Dejaco C, Appenzeller S, Camellino D, Duftner C, Gonzalez-Chiappe S, Mahr A, Mukhtyar C, Reynolds G, de Souza A, Brouwer E, Bukhari M, Buttgereit F, Byrne D, Cid MC, Cimmino M, Direskeneli H, Gilbert K, Kermani TA, Khan A, Lanyon P, Luqmani R, Mallen C, Mason JC, Matteson EL, Merkel PA, Mollan S, Neill L, Sullivan EO, Sandovici M, Schmidt WA, Watts R, Whitlock M, Yacyshyn E, Ytterberg S, Dasgupta B (1 March 2020). "British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis". Rheumatology. 59 (3). Oxford, England: e1–e23.
    PMID 31970405
    .