Öz
We aimed to investigate the clinical, demographic, and laboratory characteristics, sites of disease onset, medical treatments, and factors associated with relapse in patients with immunoglobulin G4-related disease (IgG4-RD). Thirty patients with IgG4-RD treated at the tertiary rheumatology clinic between August 2013 and August 2023 were retrospectively reviewed. The mean age of the patients was 49.5±13.2 years and the majority (n=16, 53.3%) were male. The mean follow-up time was 25 months. Erythrocyte sedimentation rate was elevated in 73.3% (n=22) and C-reactive protein in 66.7% (n=20) of patients. Elevated immunoglobulin G4 (IgG4) levels were detected in only 10 (33.3%) patients. Retroperitoneal involvement was the most common site (n=12, 40%) and lacrimal or salivary gland involvement (n=11, 36.7%) was the second most common site. Glucocorticoids (GC) were used in all 29 (96.7%) patients, except for one patient with testicular involvement who was followed up without treatment. The most commonly used immunosuppressive agent was azathioprine (n=13, 43.3%) and rituximab (n=10, 33.3%) was the most commonly used biologic disease-modifying antirheumatic drug. Treatment was changed in eleven (36.7%) patients due to relapse. Prolongation of follow-up (odds ratio=1.040; 95% confidence interval=1.006-1.075; p<0.05) was associated with relapse. Diagnosis of IgG4-RD can still be difficult because serum IgG4 levels may be normal, there are no disease-specific laboratory markers, and pathologic results cannot always be obtained by biopsy of the affected organ. Further prospective randomised studies are needed to determine more precisely the factors that influence disease recurrence.
Anahtar Kelimeler
glucocorticoid immunoglobulin G4-related disease immunosuppressive treatment rituximab
Kaynakça
- 1. Lanzillotta M, Mancuso G, Della-Torre E. Advances in the diagnosis and management of IgG4 related disease. BMJ. 2020;369:m1067. Published 2020 Jun 16. doi:10.1136/bmj.m1067
- 2. Wallace ZS, Naden RP, Chari S, et al. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. Arthritis Rheumatol. 2020;72(1):7-19. doi:10.1002/art.41120
- 3. Umehara H, Okazaki K, Masaki Y, et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2012;22(1):1-14. doi:10.1007/s10165-011-0508-6
- 4. Umehara H, Okazaki K, Kawa S, et al. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Mod Rheumatol. 2021;31(3):529-533. doi:10.1080/14397595.2020.1859710
- 5. Carruthers MN, Stone JH, Deshpande V, Khosroshahi A. Development of an IgG4-RD Responder Index. Int J Rheumatol. 2012;2012:259408. doi:10.1155/2012/259408
- 6. Wang Y, Zhao Z, Gao D, et al. Additive effect of leflunomide and glucocorticoids compared with glucocorticoids monotherapy in preventing relapse of IgG4-related disease: A randomized clinical trial. Semin Arthritis Rheum. 2020;50(6):1513-1520. doi:10.1016/j.semarthrit.2020.01.010
- 7. Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol. 2010;34(12):1812-1819. doi:10.1097/PAS.0b013e3181f7266b
- 8. Yamada K, Yamamoto M, Saeki T, et al. New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases. Arthritis Res Ther. 2017;19(1):262. Published 2017 Dec 1. doi:10.1186/s13075-017-1467-x
- 9. Wallace ZS, Deshpande V, Mattoo H, et al. IgG4-Related Disease: Clinical and Laboratory Features in One Hundred Twenty-Five Patients. Arthritis Rheumatol. 2015;67(9):2466-2475. doi:10.1002/art.39205
- 10. Ebbo M, Daniel L, Pavic M, et al. IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry. Medicine (Baltimore). 2012;91(1):49-56. doi:10.1097/MD.0b013e3182433d77
- 11. Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21-30. doi:10.1007/s10165-011-0571-z
- 12. Hao M, Liu M, Fan G, Yang X, Li J. Diagnostic Value of Serum IgG4 for IgG4-Related Disease: A PRISMA-compliant Systematic Review and Meta-analysis. Medicine (Baltimore). 2016;95(21):e3785. doi:10.1097/MD.0000000000003785
- 13.Wei B, Guo Y, Ou X, et al. Clinical Significance of SerumIgG4 in the Diagnosis and Treatment Response of IgG4-Related Disease in Adults of Southwest China: A Retrospective Study. Ann Lab Med. 2023;43(5):461-469. doi:10.3343/alm.2023.43.5.461
- 14.Xia CS, Fan CH, Liu YY. Diagnostic performances of serumIgG4 concentration and IgG4/IgG ratio in IgG4-related disease. Clin Rheumatol. 2017;36(12):2769-2774. doi:10.1007/s10067-017-3685-7
- 15.Della Torre E, Mattoo H, Mahajan VS, Carruthers M, Pillai S,Stone JH. Prevalence of atopy, eosinophilia, and IgE elevation in IgG4-related disease. Allergy. 2014;69(2):269-272. doi:10.1111/all.12320
- 16.Culver EL, Sadler R, Bateman AC, et al. Increases in IgE,Eosinophils, and Mast Cells Can be Used in Diagnosis and to Predict Relapse of IgG4-Related Disease. Clin Gastroenterol Hepatol. 2017;15(9):1444-1452.e6. doi:10.1016/j.cgh.2017.02.007
- 17.Wallace ZS, Zhang Y, Perugino CA, et al. Clinical phenotypesof IgG4-related disease: an analysis of two international cross-sectional cohorts. Ann Rheum Dis. 2019;78(3):406-412. doi:10.1136/annrheumdis-2018-214603
- 18.Khosroshahi A, Wallace ZS, Crowe JL, et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015;67(7):1688-1699. doi:10.1002/art.39132
- 19.Wang L, Zhang P, Wang M, et al. Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients. Arthritis Res Ther. 2018;20(1):65. Published 2018 Apr 10. doi:10.1186/s13075-018-1567-2
- 20.Yunyun F, Yu P, Panpan Z, et al. Efficacy and safety of low dose Mycophenolate mofetil treatment for immunoglobulin G4-related disease: a randomized clinical trial. Rheumatology (Oxford). 2019;58(1):52-60. doi:10.1093/rheumatology/key227
- 21.Khosroshahi A, Carruthers MN, Deshpande V, Unizony S,Bloch DB, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore). 2012;91(1):57-66. doi:10.1097/MD.0b013e3182431ef6
- 22.Sasaki T, Akiyama M, Kaneko Y, et al. Risk factors of relapse following glucocorticoid tapering in IgG4-related disease. Clin Exp Rheumatol. 2018;36 Suppl 112(3):186-189.
- 23.Aydın Tufan M., Kaşkari D. İmmünglobülin G4 ilişkili hastalıkta nüksü etkileyen faktörler: retrospektif bir analiz. J Med Palliat Care / JOMPAC / jompac. 2022; 3(4): 306-310.
Öz
İmmünglobülin G4 ilişkili hastalık (IgG4-İH) tanısıyla takip ettiğimiz hastaların klinik, demografik ve laboratuvar özelliklerini, tutulum yerlerini, medikal tedavileri ve nüksle ilişkili faktörleri değerlendirmeyi planladık. Üçüncü basamak romatoloji kliniğinde Ağustos 2013-Ağustos 2023 tarihleri arasında IgG4-İH tanısıyla takip edilen, 30 hasta restrospektif olarak tarandı. Hastaların yaş ortalaması 49,5±13,2 olup, çoğunluğunu (n=16, %53,3) erkek hastalar oluşturmaktaydı. Takip süresi ortalama 25 aydı. Eritrosit sedimentasyon hızı hastaların %73,3 (n=22)’ünde, C-reaktif protein ise %66,7 (n=20)’sinde yüksekti. İmmünglobülin G4 (IgG4) düzeyleri sadece 10 (%33,3) hastada yüksek olarak saptandı. En sık retroperitoneal tutulum (n=12,%40) olup, lakrimal veya tükürük bezi tutulumu (n=11,% 36,7) ise ikinci en sık tutulan bölgeydi. Testis tutulumu olup tedavisiz takip edilen bir hasta dışında diğer 29 (%96,7) hastanın tamamında glukokortikoid (GK) kullanımı mevcuttu. En sık kullanılan immünsupresif tedavi ajanı azatiyoprin (n=13, %43,3) olup, rituksimab (n=10, %33,3) ise en sık kullanılan biyolojik hastalık modifiye edici antiromatizmal ilaçtı. On bir (%36,7) hastamızda nüks nedeniyle tedavi değişikliği yapılmıştı. Takip süresinin (Odds oranı=1,040; %95 güven aralığı=1,006-1,075; p<0,05) artmasının nüksle ilişkili olduğu saptandı. Serum IgG4 düzeylerinin normal olabilmesi, hastalığa özgü laboratuvar belirteçlerinin olmaması ve ilgili organ tutulumundan her zaman biyopsi ile patoloji sonuçlarının elde edilememesi gibi nedenlerle hala IgG4-İH tanısında zorluklar yaşanabilmektedir. Nüksü etkileyen faktörlerin daha net olarak belirlenebilmesi için ise daha fazla prospektif randomize çalışmalara ihtiyaç vardır.
Anahtar Kelimeler
glukokortikoid immünglobülin G4 ilişkili hastalık immünsupresif tedavi rituksimab
Kaynakça
- 1. Lanzillotta M, Mancuso G, Della-Torre E. Advances in the diagnosis and management of IgG4 related disease. BMJ. 2020;369:m1067. Published 2020 Jun 16. doi:10.1136/bmj.m1067
- 2. Wallace ZS, Naden RP, Chari S, et al. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. Arthritis Rheumatol. 2020;72(1):7-19. doi:10.1002/art.41120
- 3. Umehara H, Okazaki K, Masaki Y, et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2012;22(1):1-14. doi:10.1007/s10165-011-0508-6
- 4. Umehara H, Okazaki K, Kawa S, et al. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Mod Rheumatol. 2021;31(3):529-533. doi:10.1080/14397595.2020.1859710
- 5. Carruthers MN, Stone JH, Deshpande V, Khosroshahi A. Development of an IgG4-RD Responder Index. Int J Rheumatol. 2012;2012:259408. doi:10.1155/2012/259408
- 6. Wang Y, Zhao Z, Gao D, et al. Additive effect of leflunomide and glucocorticoids compared with glucocorticoids monotherapy in preventing relapse of IgG4-related disease: A randomized clinical trial. Semin Arthritis Rheum. 2020;50(6):1513-1520. doi:10.1016/j.semarthrit.2020.01.010
- 7. Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol. 2010;34(12):1812-1819. doi:10.1097/PAS.0b013e3181f7266b
- 8. Yamada K, Yamamoto M, Saeki T, et al. New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases. Arthritis Res Ther. 2017;19(1):262. Published 2017 Dec 1. doi:10.1186/s13075-017-1467-x
- 9. Wallace ZS, Deshpande V, Mattoo H, et al. IgG4-Related Disease: Clinical and Laboratory Features in One Hundred Twenty-Five Patients. Arthritis Rheumatol. 2015;67(9):2466-2475. doi:10.1002/art.39205
- 10. Ebbo M, Daniel L, Pavic M, et al. IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry. Medicine (Baltimore). 2012;91(1):49-56. doi:10.1097/MD.0b013e3182433d77
- 11. Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21-30. doi:10.1007/s10165-011-0571-z
- 12. Hao M, Liu M, Fan G, Yang X, Li J. Diagnostic Value of Serum IgG4 for IgG4-Related Disease: A PRISMA-compliant Systematic Review and Meta-analysis. Medicine (Baltimore). 2016;95(21):e3785. doi:10.1097/MD.0000000000003785
- 13.Wei B, Guo Y, Ou X, et al. Clinical Significance of SerumIgG4 in the Diagnosis and Treatment Response of IgG4-Related Disease in Adults of Southwest China: A Retrospective Study. Ann Lab Med. 2023;43(5):461-469. doi:10.3343/alm.2023.43.5.461
- 14.Xia CS, Fan CH, Liu YY. Diagnostic performances of serumIgG4 concentration and IgG4/IgG ratio in IgG4-related disease. Clin Rheumatol. 2017;36(12):2769-2774. doi:10.1007/s10067-017-3685-7
- 15.Della Torre E, Mattoo H, Mahajan VS, Carruthers M, Pillai S,Stone JH. Prevalence of atopy, eosinophilia, and IgE elevation in IgG4-related disease. Allergy. 2014;69(2):269-272. doi:10.1111/all.12320
- 16.Culver EL, Sadler R, Bateman AC, et al. Increases in IgE,Eosinophils, and Mast Cells Can be Used in Diagnosis and to Predict Relapse of IgG4-Related Disease. Clin Gastroenterol Hepatol. 2017;15(9):1444-1452.e6. doi:10.1016/j.cgh.2017.02.007
- 17.Wallace ZS, Zhang Y, Perugino CA, et al. Clinical phenotypesof IgG4-related disease: an analysis of two international cross-sectional cohorts. Ann Rheum Dis. 2019;78(3):406-412. doi:10.1136/annrheumdis-2018-214603
- 18.Khosroshahi A, Wallace ZS, Crowe JL, et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015;67(7):1688-1699. doi:10.1002/art.39132
- 19.Wang L, Zhang P, Wang M, et al. Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients. Arthritis Res Ther. 2018;20(1):65. Published 2018 Apr 10. doi:10.1186/s13075-018-1567-2
- 20.Yunyun F, Yu P, Panpan Z, et al. Efficacy and safety of low dose Mycophenolate mofetil treatment for immunoglobulin G4-related disease: a randomized clinical trial. Rheumatology (Oxford). 2019;58(1):52-60. doi:10.1093/rheumatology/key227
- 21.Khosroshahi A, Carruthers MN, Deshpande V, Unizony S,Bloch DB, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore). 2012;91(1):57-66. doi:10.1097/MD.0b013e3182431ef6
- 22.Sasaki T, Akiyama M, Kaneko Y, et al. Risk factors of relapse following glucocorticoid tapering in IgG4-related disease. Clin Exp Rheumatol. 2018;36 Suppl 112(3):186-189.
- 23.Aydın Tufan M., Kaşkari D. İmmünglobülin G4 ilişkili hastalıkta nüksü etkileyen faktörler: retrospektif bir analiz. J Med Palliat Care / JOMPAC / jompac. 2022; 3(4): 306-310.
Ayrıntılar
| Birincil Dil | Türkçe |
|---|---|
| Konular | Romatoloji ve Artrit |
| Bölüm | Özgün Araştırma Makaleleri |
| Yazarlar | |
| Yayımlanma Tarihi | 31 Aralık 2023 |
| Gönderilme Tarihi | 3 Kasım 2023 |
| Kabul Tarihi | 5 Aralık 2023 |
| Yayımlandığı Sayı | Yıl 2023 Cilt: 49 Sayı: 3 |
Kaynak Göster
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