İmmünglobülin G4 ilişkili hastalıkta nüksü etkileyen faktörler: retrospektif bir analiz

Amaç: Bu çalışmada immünglobülin G4 ilişkili hastalıkta (IgG4-İH) klinik tutulum özelliklerinin, nüks oranlarının ve nüks ile ilişkili faktörlerin belirlenmesi amaçlanmıştır. Gereç ve Yöntem: Çalışmamıza IgG4-İH tanısıyla 36 hasta dahil edildi. Bu hastaların demografik, klinik, laboratuvar, radyolojik bulguları, steroid, immünsüpresif tedavi rejimleri ve nüks oranları retrospektif olarak kaydedildi. Bulgular: Hastaların %36’sı kadındı ve ortalama yaş 58,5±13,2 yıldı. Remisyon oranı %56, nüks oranı ise %19 olarak tespit edildi. İlk tanı anında pankreatit ve retroperitoneal fibrozisi olan hastalarda nüks oranı daha yüksekti (p=0.001). Tek değişkenli analize göre, nüks ile ilişkili bulunan faktörler şu şekildeydi: Hastalık süresi (p=0.001), alerji öyküsü (p=0.018), sigara kullanımı (p=0.027), eozinofili (p=0.001), total IgE (p=0.005) ve kreatinin yüksekliği (p=0.001). Çok değişkenli analizde ise hastalık süresi (Odds oranı (OO) [%95 güven aralığı (GA)]=1.1. [1.01-1.20]; p=0.016) nüks ile ilişkili bağımsız risk faktörü olarak bulundu. Sonuç: IgG4-İH’li hastalıkta nüks riski, tanı anında pankreas ve retroperitoneal tutulumu olması, alerji öyküsü, sigara kullanımı, eozinofili, total IgE ve kreatinin yüksekliği ile ilişkiliydi. Bu risk faktörlerini taşıyan hastalarda kombine immünsüpresif tedavilerin tercihi ve steroid dozunun kontrollü azaltılması nüksü engellemekte yararlı olabilir.

Anahtar Kelimeler:

IgG4 ilişkili hastalık, Nüks, Retroperitoneal fibrozis

Factors affecting relapse in immunoglobulin G4-related disease: a retrospective analysis

Introduction: In this study, it was aimed to determine the clinical features of involvement, relapse rates and relapse-related factors in immunoglobulin-G4-related disease (IgG4-RD). Materials and Method: Thirty-six patients with IgG4-RD were included in our study. Demographic, clinical, laboratory, radiological findings, immunosuppressive treatment regimens and recurrence rates of these patients were recorded retrospectively. Results: 36% of the patients were female and the mean age was 58.5±13.2 years. The remission rate and relapse rate were 56% and 19%, respectively. Recurrence was detected more frequently in the coexistence of pancreatitis and retroperitoneal fibrosis at the time of diagnosis (p=0.001). Based on univariate analysis, factors associated with relapse were attributed as follows: disease duration (p=0.001), history of allergy (p=0.018), smoking (p=0.027), eosinophilia (p=0.001), total IgE (p=0.005) and elevated creatinine (p=0.001). In multivariate analysis, disease duration (odds ratio (OR) [95% confidence interval (CI)]=1.1. [1.01-1.20]; p=0.016) was found to be an independent risk factor for relapse. Conclusion: The risk of recurrence in disease with IgG4-RD was associated with pancreatic and retroperitoneal involvement at the time of diagnosis, history of allergy, smoking, eosinophilia, high total IgE and creatinine. Combined immunosuppressive therapy and slow steroid dose reduction are important to prevent relapse in high-risk patients.

Keywords:

IgG4-related disease, relapse, retroperitoneal fibrosis,

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Perugino CA, Wallace ZS, Meyersohn N, Oliveira G, Stone JR, Stone JH. Large vessel involvement by IgG4-related disease. Medicine (Baltimore) 2016; 95: 3344.
Peng L, Zhang P, Li J, et al. IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease. Arthritis Res Ther 2020; 22: 103.
Lian L, Wang C, Tian JL. IgG4-related retroperitoneal fibrosis: a newly characterized disease. Int J Rheum Dis 2016; 19: 1049- 1055.
Li PH, Ko KL, Ho CT, et al. immunoglobulin G4-related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease. Hong Kong Med J 2017; 23: 446- 53.
Karadeniz H, Vaglio A. IgG4-related disease: a contemporary review. Turk J Med Sci 2020; 50: 1616- 31.
Kuruma S, Kamisawa T, Tabata T, et al. Allergen-specific IgE antibody serologic assays in patients with autoimmune pancreatitis. Intern Med 2014; 53: 541- 3.
Rogger TM, De Marchi G, Brozzi L, et al. Immunoglobulin G4-Related Disease Responder Index Correlates with the Risk of 1-Year Relapse in Type 1 Autoimmune Pancreatitis. Pancreas 2021; 50: 879- 881.
Zongfei J, Lingying M, Lijuan Z, et al. Prognostic factors in IgG4-related disease: a long-term monocentric Chinese cohort study. Clin Rheumatol 2021; 40: 2293- 2300.
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012; 366: 539- 51.
Lanzillotta M, Campochiaro C, Mancuso G, et al. Clinical phenotypes of IgG4-related disease reflect different prognostic outcomes. Rheumatology (Oxford) 2020; 59: 2435- 42.
Brito-Zerón P, Kostov B, Bosch X, Acar-Denizli N, Ramos-Casals M, Stone JH.
Therapeutic approach to IgG4-related disease: A systematic review. Medicine (Baltimore) 2016; 95: 4002.
Liu Y, Zeng Q, Zhu L, et al. Relapse predictors and serologically unstable condition of IgG4-related disease: a large Chinese cohort. Rheumatology (Oxford) 2020; 59: 2115- 23.
Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 2012; 22: 21- 30.
Wallace ZS, Naden RP, Chari S, et al; American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. Arthritis Rheumatol 2020; 72: 7- 19.
Peng Y, Li JQ, Zhang PP, et al. Clinical outcomes and predictive relapse factors of IgG4-related disease following treatment: a long -term cohort study. J Intern Med 2019; 286: 542- 552.
Ebbo M, Grados A, Samson M, et al. Long-term efficacy and safety of rituximab in IgG4-related disease: Data from a French nationwide study of thirty-three patients. PLoS One 2017; 12: 0183844.
Culver EL, Sadler R, Simpson D, et al. Elevated Serum IgG4 Levels in Diagnosis, Treatment Response, Organ Involvement, and Relapse in a Prospective IgG4-Related Disease UK Cohort. Am J Gastroenterol 2016; 111: 733- 43.
Wallace ZS, Mattoo H, Mahajan VS, et al. Predictors of disease relapse in IgG4-related disease following rituximab. Rheumatology (Oxford) 2016; 55: 1000- 8.
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: 1444- 52.
Zhang W, Xue F, Wang C, Shao L. Clinical features and prognostic factors of ten patients with renal failure caused by IgG4-related retroperitoneal fibrosis. Oncotarget 2017; 9: 2858- 65.
Wallwork R, Perugino CA, Fu X, et al. The association of smoking with immunoglobulin G4-related disease: a case-control study. Rheumatology (Oxford) 2021; 60: 5310- 17.
Shirakashi M, Yoshifuji H, Kodama Y, et al. Factors in glucocorticoid regimens associated with treatment response and relapses of IgG4-related disease: a multicentre study. Sci Rep 2018; 8: 10262.