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J Rheum Dis 2024; 31(4): 191-192

Published online October 1, 2024

© Korean College of Rheumatology

Giant cell arteritis in South Korea

Jin Kyun Park , M.D., Ph.D.

Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea

Correspondence to : Jin Kyun Park, https://orcid.org/0000-0003-2167-9393
Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, 101 Daehakro, Jongno-gu, Seoul 03080, Korea. E-mail: jinkyunpark@snu.ac.kr

Received: July 26, 2024; Revised: September 5, 2024; Accepted: September 9, 2024

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Giant cell arteritis (GCA) is a systemic vasculitis that primarily affects large and medium-sized arteries, especially the cranial arteries, resulting in classic symptoms such as acute onset headache, visual disturbances, jaw claudication, and stroke [1]. Despite a prevalence of 7.3 per 100,000 in Northern Europe, GCA is the most common vasculitis among individuals over 50 years old [2]. It predominantly affects elderly Caucasians in Northern Europe, whereas Takayasu arteritis, another type of large vessel vasculitis, mainly affects young Asian women. GCA is extremely rare in Black and Asian populations. In Japan, the prevalence of GCA is estimated at 1.47 per 100,000 among individuals over 50 years old, and its prevalence in South Korea has not been accurately estimated to date [3].

Given the geographic and ethnic variations in GCA prevalence, it is unclear whether clinical features, treatment responses, and prognoses observed in Western populations can be generalized to Korean patients with GCA. Therefore, the study by Lee et al. [4] in this issue is a significant contribution, as it describes the clinical characteristics of a relatively large retrospective multi-center cohort of 27 Korean patients with GCA.

The clinical characteristics of Korean GCA patients are similar to those reported in studies from Western countries from countries like France and the USA (Table 1) [5-7]. As a mean age at diagnosis was 75 years, the incidence of GCA is expected to rise in Korea’s aging population [8]. Concomitant polymyalgia rheumatica (PMR) was present in 50% of the Korean patients, compared to 34.5% and 44.3% in Western studies. PMR is notably rare in Korea, with a prevalence of 0.0082% (8.21 per 100,000), compared to 0.6% in a previous study [2,7], suggesting that both GCA and PMR, which share similar pathophysiology, are very rare in South Korea, possibly due to genetic factors.

Table 1 . Characteristics of Korean and Western GCA patients [5-7]

Korea (n=27)Western (n=693)Western (n=286)
Age at diagnosis (yr)75.0 [71.0~79.0]75 [48~94]75.0±7.6
Sex, female17 (63.0)486 (70.1)213 (74.5)
Duration of symptoms (wk)5.5 [1.0~8.0]8.4 [3.6~17.6]
Fever at onset12 (44.4)229 (33.0)57 (20.4)
Positive TAB15/16 (93.8)632 (91.2)
Visual ischemia complication9 (33.3)215 (31.0)36 (12.7)
Concurrent PMR14 (51.9)239 (34.5)36 (12.6)
ESR (mm/hr)98.0 [74.5~111.0]66.0 [42.5~93.5]
CRP (mg/dL)9.09 [5.91~15.86]8.3 [0.1~53.5]5.47 [2.30~10.05]
Large vessel involvement12 (44.4)173/310 (55.8)
Relapse4 (14.8)354 (51.1)213 (74.5)
Median follow-up duration (mo)33.849.961.2
Factors associated with relapseAbsence of fever at onset
Vision loss at onset
Established HTN/DM at diagnosis
Female
Permanent vision loss4 (14.8)16 (5.7)
Initial GC dose60.0 [50.0~60.0] mg/d*0.73 [0.25~1.6] mg/kg/d50.8±13.1 mg/d*
Use of GC-sparing agents22 (81.5)124 (17.9)

Values are presented as median [interquartile ranges], mean±standard deviation, or number (%). GCA: giant cell arteritis, TAB: temporal artery biopsy, PMR: polymyalgia rheumatica, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, HTN: hypertension, DM: diabetes mellitus, GC: glucocorticoids. *Prednisolone-equivalent dose. Reused from the article of de Boysson et al. (Clin Exp Rheumatol 2019;37 Suppl 117:57-60) [5], Labarca et al. (Rheumatology (Oxford) 2016;55:347-56) [6], Kim et al. (J Rheum Dis 2014;21:297-302) [7].



The most serious complication of GCA is vision loss to anterior ischemic optic neuropathy. Visual ischemic complications were quite common, occurring in 33.3% of Korean GCA patients at baseline, comparable to the previously reports [9]. However, the rate of permanent vision loss in Korean GCA patients was high at 14.8%, compared to 5.7% in Western population. This underscores the importance of early diagnosis and treatment to prevent blindness.

The treatment response in Korean GCA patients was favorable, with a relapse rate of only 14.8%, compared to 51.1% and 74.5% in Western studies. This lower relapse rate may be attributed to the higher use of glucocorticoid-sparing agents in Korean patients.

While the study by Lee et al. [4] provides valuable insights into Korean patients with GCA, further large-scale epidemiologic studies are needed to accurately estimate the incidence and prevalence, and clinical characteristics of both GCA and PMR in South Korea. Additionally, it is necessary to assess the therapeutic efficacy, including the novel treatment options such as interleukin-6 inhibitors, and long-term outcome in Korean patients with GCA.

In conclusion, although GCA is rare in South Korea, it can lead to severe complications, including permanent vision loss. Therefore, elderly patients presenting with headaches and elevated inflammatory markers should be promptly evaluated for GCA.

J.K.P. has been an editorial board member since June 2020, but has no role in the decision to publish this article.

  1. Salvarani C, Cantini F, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. Lancet 2008;372:234-45.
    Pubmed CrossRef
  2. Salvarani C, Gabriel SE, O'Fallon WM, Hunder GG. Epidemiology of polymyalgia rheumatica in Olmsted County, Minnesota, 1970-1991. Arthritis Rheum 1995;38:369-73.
    Pubmed CrossRef
  3. Kobayashi S, Yano T, Matsumoto Y, Numano F, Nakajima N, Yasuda K, et al. Clinical and epidemiologic analysis of giant cell (temporal) arteritis from a nationwide survey in 1998 in Japan: the first government-supported nationwide survey. Arthritis Rheum 2003;49:594-8.
    Pubmed CrossRef
  4. Lee JI, Park JW, Jung Y, Shin K, Choi SR, Kang EH, et al. Clinical characteristics and courses of Korean patients with giant cell arteritis: a multi-center retrospective study. J Rheum Dis 2024;31:160-70.
    Pubmed KoreaMed CrossRef
  5. de Boysson H, Liozon E, Ly KH, Dumont A, Delmas C, Aouba A. The different clinical patterns of giant cell arteritis. Clin Exp Rheumatol 2019;37 Suppl 117:57-60.
    Pubmed CrossRef
  6. Labarca C, Koster MJ, Crowson CS, Makol A, Ytterberg SR, Matteson EL, et al. Predictors of relapse and treatment outcomes in biopsy-proven giant cell arteritis: a retrospective cohort study. Rheumatology (Oxford) 2016;55:347-56.
    Pubmed KoreaMed CrossRef
  7. Kim IY, Seo GH, Lee S, Jeong H, Kim H, Lee J, et al. Epidemiology of polymyalgia rheumatica in Korea. J Rheum Dis 2014;21:297-302.
    CrossRef
  8. Kermani TA, Schäfer VS, Crowson CS, Hunder GG, Gabriel SE, Matteson EL, et al. Increase in age at onset of giant cell arteritis: a population-based study. Ann Rheum Dis 2010;69:780-1.
    Pubmed CrossRef
  9. Margolin E, Donaldson L. Homonymous hemianopia as the presenting sign of posterior cortical atrophy. Pract Neurol 2022;22:160-1.
    Pubmed CrossRef

Article

Editorial

J Rheum Dis 2024; 31(4): 191-192

Published online October 1, 2024 https://doi.org/10.4078/jrd.2024.0091

Copyright © Korean College of Rheumatology.

Giant cell arteritis in South Korea

Jin Kyun Park , M.D., Ph.D.

Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea

Correspondence to:Jin Kyun Park, https://orcid.org/0000-0003-2167-9393
Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, 101 Daehakro, Jongno-gu, Seoul 03080, Korea. E-mail: jinkyunpark@snu.ac.kr

Received: July 26, 2024; Revised: September 5, 2024; Accepted: September 9, 2024

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Body

Giant cell arteritis (GCA) is a systemic vasculitis that primarily affects large and medium-sized arteries, especially the cranial arteries, resulting in classic symptoms such as acute onset headache, visual disturbances, jaw claudication, and stroke [1]. Despite a prevalence of 7.3 per 100,000 in Northern Europe, GCA is the most common vasculitis among individuals over 50 years old [2]. It predominantly affects elderly Caucasians in Northern Europe, whereas Takayasu arteritis, another type of large vessel vasculitis, mainly affects young Asian women. GCA is extremely rare in Black and Asian populations. In Japan, the prevalence of GCA is estimated at 1.47 per 100,000 among individuals over 50 years old, and its prevalence in South Korea has not been accurately estimated to date [3].

Given the geographic and ethnic variations in GCA prevalence, it is unclear whether clinical features, treatment responses, and prognoses observed in Western populations can be generalized to Korean patients with GCA. Therefore, the study by Lee et al. [4] in this issue is a significant contribution, as it describes the clinical characteristics of a relatively large retrospective multi-center cohort of 27 Korean patients with GCA.

The clinical characteristics of Korean GCA patients are similar to those reported in studies from Western countries from countries like France and the USA (Table 1) [5-7]. As a mean age at diagnosis was 75 years, the incidence of GCA is expected to rise in Korea’s aging population [8]. Concomitant polymyalgia rheumatica (PMR) was present in 50% of the Korean patients, compared to 34.5% and 44.3% in Western studies. PMR is notably rare in Korea, with a prevalence of 0.0082% (8.21 per 100,000), compared to 0.6% in a previous study [2,7], suggesting that both GCA and PMR, which share similar pathophysiology, are very rare in South Korea, possibly due to genetic factors.

Table 1 . Characteristics of Korean and Western GCA patients [5-7].

Korea (n=27)Western (n=693)Western (n=286)
Age at diagnosis (yr)75.0 [71.0~79.0]75 [48~94]75.0±7.6
Sex, female17 (63.0)486 (70.1)213 (74.5)
Duration of symptoms (wk)5.5 [1.0~8.0]8.4 [3.6~17.6]
Fever at onset12 (44.4)229 (33.0)57 (20.4)
Positive TAB15/16 (93.8)632 (91.2)
Visual ischemia complication9 (33.3)215 (31.0)36 (12.7)
Concurrent PMR14 (51.9)239 (34.5)36 (12.6)
ESR (mm/hr)98.0 [74.5~111.0]66.0 [42.5~93.5]
CRP (mg/dL)9.09 [5.91~15.86]8.3 [0.1~53.5]5.47 [2.30~10.05]
Large vessel involvement12 (44.4)173/310 (55.8)
Relapse4 (14.8)354 (51.1)213 (74.5)
Median follow-up duration (mo)33.849.961.2
Factors associated with relapseAbsence of fever at onset
Vision loss at onset
Established HTN/DM at diagnosis
Female
Permanent vision loss4 (14.8)16 (5.7)
Initial GC dose60.0 [50.0~60.0] mg/d*0.73 [0.25~1.6] mg/kg/d50.8±13.1 mg/d*
Use of GC-sparing agents22 (81.5)124 (17.9)

Values are presented as median [interquartile ranges], mean±standard deviation, or number (%). GCA: giant cell arteritis, TAB: temporal artery biopsy, PMR: polymyalgia rheumatica, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, HTN: hypertension, DM: diabetes mellitus, GC: glucocorticoids. *Prednisolone-equivalent dose. Reused from the article of de Boysson et al. (Clin Exp Rheumatol 2019;37 Suppl 117:57-60) [5], Labarca et al. (Rheumatology (Oxford) 2016;55:347-56) [6], Kim et al. (J Rheum Dis 2014;21:297-302) [7]..



The most serious complication of GCA is vision loss to anterior ischemic optic neuropathy. Visual ischemic complications were quite common, occurring in 33.3% of Korean GCA patients at baseline, comparable to the previously reports [9]. However, the rate of permanent vision loss in Korean GCA patients was high at 14.8%, compared to 5.7% in Western population. This underscores the importance of early diagnosis and treatment to prevent blindness.

The treatment response in Korean GCA patients was favorable, with a relapse rate of only 14.8%, compared to 51.1% and 74.5% in Western studies. This lower relapse rate may be attributed to the higher use of glucocorticoid-sparing agents in Korean patients.

While the study by Lee et al. [4] provides valuable insights into Korean patients with GCA, further large-scale epidemiologic studies are needed to accurately estimate the incidence and prevalence, and clinical characteristics of both GCA and PMR in South Korea. Additionally, it is necessary to assess the therapeutic efficacy, including the novel treatment options such as interleukin-6 inhibitors, and long-term outcome in Korean patients with GCA.

In conclusion, although GCA is rare in South Korea, it can lead to severe complications, including permanent vision loss. Therefore, elderly patients presenting with headaches and elevated inflammatory markers should be promptly evaluated for GCA.

FUNDING

None.

ACKNOWLEDGMENTS

None.

CONFLICT OF INTEREST

J.K.P. has been an editorial board member since June 2020, but has no role in the decision to publish this article.

Table 1 . Characteristics of Korean and Western GCA patients [5-7].

Korea (n=27)Western (n=693)Western (n=286)
Age at diagnosis (yr)75.0 [71.0~79.0]75 [48~94]75.0±7.6
Sex, female17 (63.0)486 (70.1)213 (74.5)
Duration of symptoms (wk)5.5 [1.0~8.0]8.4 [3.6~17.6]
Fever at onset12 (44.4)229 (33.0)57 (20.4)
Positive TAB15/16 (93.8)632 (91.2)
Visual ischemia complication9 (33.3)215 (31.0)36 (12.7)
Concurrent PMR14 (51.9)239 (34.5)36 (12.6)
ESR (mm/hr)98.0 [74.5~111.0]66.0 [42.5~93.5]
CRP (mg/dL)9.09 [5.91~15.86]8.3 [0.1~53.5]5.47 [2.30~10.05]
Large vessel involvement12 (44.4)173/310 (55.8)
Relapse4 (14.8)354 (51.1)213 (74.5)
Median follow-up duration (mo)33.849.961.2
Factors associated with relapseAbsence of fever at onset
Vision loss at onset
Established HTN/DM at diagnosis
Female
Permanent vision loss4 (14.8)16 (5.7)
Initial GC dose60.0 [50.0~60.0] mg/d*0.73 [0.25~1.6] mg/kg/d50.8±13.1 mg/d*
Use of GC-sparing agents22 (81.5)124 (17.9)

Values are presented as median [interquartile ranges], mean±standard deviation, or number (%). GCA: giant cell arteritis, TAB: temporal artery biopsy, PMR: polymyalgia rheumatica, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, HTN: hypertension, DM: diabetes mellitus, GC: glucocorticoids. *Prednisolone-equivalent dose. Reused from the article of de Boysson et al. (Clin Exp Rheumatol 2019;37 Suppl 117:57-60) [5], Labarca et al. (Rheumatology (Oxford) 2016;55:347-56) [6], Kim et al. (J Rheum Dis 2014;21:297-302) [7]..


References

  1. Salvarani C, Cantini F, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. Lancet 2008;372:234-45.
    Pubmed CrossRef
  2. Salvarani C, Gabriel SE, O'Fallon WM, Hunder GG. Epidemiology of polymyalgia rheumatica in Olmsted County, Minnesota, 1970-1991. Arthritis Rheum 1995;38:369-73.
    Pubmed CrossRef
  3. Kobayashi S, Yano T, Matsumoto Y, Numano F, Nakajima N, Yasuda K, et al. Clinical and epidemiologic analysis of giant cell (temporal) arteritis from a nationwide survey in 1998 in Japan: the first government-supported nationwide survey. Arthritis Rheum 2003;49:594-8.
    Pubmed CrossRef
  4. Lee JI, Park JW, Jung Y, Shin K, Choi SR, Kang EH, et al. Clinical characteristics and courses of Korean patients with giant cell arteritis: a multi-center retrospective study. J Rheum Dis 2024;31:160-70.
    Pubmed KoreaMed CrossRef
  5. de Boysson H, Liozon E, Ly KH, Dumont A, Delmas C, Aouba A. The different clinical patterns of giant cell arteritis. Clin Exp Rheumatol 2019;37 Suppl 117:57-60.
    Pubmed CrossRef
  6. Labarca C, Koster MJ, Crowson CS, Makol A, Ytterberg SR, Matteson EL, et al. Predictors of relapse and treatment outcomes in biopsy-proven giant cell arteritis: a retrospective cohort study. Rheumatology (Oxford) 2016;55:347-56.
    Pubmed KoreaMed CrossRef
  7. Kim IY, Seo GH, Lee S, Jeong H, Kim H, Lee J, et al. Epidemiology of polymyalgia rheumatica in Korea. J Rheum Dis 2014;21:297-302.
    CrossRef
  8. Kermani TA, Schäfer VS, Crowson CS, Hunder GG, Gabriel SE, Matteson EL, et al. Increase in age at onset of giant cell arteritis: a population-based study. Ann Rheum Dis 2010;69:780-1.
    Pubmed CrossRef
  9. Margolin E, Donaldson L. Homonymous hemianopia as the presenting sign of posterior cortical atrophy. Pract Neurol 2022;22:160-1.
    Pubmed CrossRef
JRD
Oct 01, 2024 Vol.31 No.4, pp. 191~263
COVER PICTURE
Ancestry-driven pathways for SLE-risk SNP-associated genes. The ancestry-driven key signaling pathways in Asians, Europeans, and African Americans were analyzed by enrichr (https://maayanlab.cloud/Enrichr/#libraries) using non-HLA SNP-associated genes. SLE: systemic lupus erythematosus, SNP: single-nucleotide polymorphism, JAK–STAT: janus kinase–signal transducers and activators of transcription, IFN: interferon gamma. (J Rheum Dis 2024;31:200-211)

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