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

Abstract

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

İmmünglobülin G4 ilişkili hastalıkta nüksü etkileyen faktörler: retrospektif bir analiz

Abstract

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.

Keywords

• IgG4 ilişkili hastalık
• Nüks
• Retroperitoneal fibrozis

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