Immunoglobulin G4-Related Disease: A Single Centre Experience of 30 Cases

Year 2023, Volume: 49 Issue: 3, 389 - 395, 31.12.2023

Salim Mısırcı , Ali Ekin , Belkıs Nihan Coşkun , Burcu Yağız , Hüseyin Ediz Dalkılıç , Yavuz Pehlivan

https://doi.org/10.32708/uutfd.1385438

Abstract

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.

Keywords

glucocorticoid, immunoglobulin G4-related disease, immunosuppressive treatment, rituximab

İmmünglobülin G4 İlişkili Hastalık: 30 Vakalık Tek Merkez Deneyimi

Abstract

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.

Keywords

glukokortikoid, immünglobülin G4 ilişkili hastalık, immünsupresif tedavi, rituksimab

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