The treatment outcomes in IgG4-related disease

A F Karim, R D Bansie, S M Rombach, D Paridaens, R M Verdijk, P M van Hagen, J A M Van Laar

Research output: Contribution to journalArticleResearchpeer-review


INTRODUCTION: IgG4-related disease (IgG4-RD) is an emerging systemic inflammatory disease involving nearly all organs eventually leading to fibrosis. Prompt and adequate treatment to prevent irreversible organ damage is therefore pivotal. To evaluate the treatment outcomes, we studied a well-defined cohort of patients with IgG4-RD.

METHOD: 32 patients with histologically confirmed IgG4-RD diagnosed between 1999 and April 2017 were included and reviewed for demographic and clinical characteristics. The response to treatment with glucocorticoids, disease modifying antirheumatic drugs, rituximab and other therapeutic interventions were evaluated.

RESULTS: Glucocorticoids as well as rituximab appeared successful therapeutic drugs leading to clinical remission (complete or partial remission) in all patients. Recurrences, however, were frequently seen (62% versus 100%, respectively). Diseases modifying antirheumatic drugs (DMARDs), including azathioprine, methotrexate and mycophenolate mofetil were effective in less than half of the cases. A minority of patients was treated with alternative treatments including hydroxychloroquine, thalidomide and infliximab which all appeared effective. Surgical intervention and radiotherapy in local disease seemed to induce clinical remission and were associated with low recurrence rates.

CONCLUSION: Glucocorticoids and rituximab induce substantial responses as well as primary surgical intervention and radiotherapy, while the efficacy of DMARDs is limited. Based on the few data available, hydroxychloroquine, infliximab and thalidomide may be promising treatment options for second or third line strategies.

Original languageEnglish
Pages (from-to)275-285
Number of pages11
JournalThe Netherlands journal of medicine
Issue number6
Publication statusPublished - Aug 2018


  • Adrenal Cortex Hormones/therapeutic use
  • Adult
  • Aged
  • Antirheumatic Agents/therapeutic use
  • Autoimmune Diseases/drug therapy
  • Cohort Studies
  • Cyclophosphamide/therapeutic use
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulin G/drug effects
  • Immunoglobulin G4-Related Disease/drug therapy
  • Male
  • Methotrexate/therapeutic use
  • Middle Aged
  • Netherlands
  • Recurrence
  • Retrospective Studies
  • Risk Assessment
  • Rituximab/therapeutic use
  • Severity of Illness Index
  • Time Factors
  • Treatment Outcome
  • Young Adult


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