Abstract
Background: Although there are multiple ways to manage immunoglobulin G4–related disease (IgG4-RD), including treatment with glucocorticoids, “steroid-sparing” immunosuppressive drugs, or biologic agents, few clinical trials on IgG4-RD have been conducted. This study aimed to compare the efficacy and safety of glucocorticoids (GCs) combined with cyclophosphamide (CYC) or mycophenolate mofetil (MMF) in IgG4-RD patients. This cohort study was registered at ClinicalTrials.gov (ID: NCT01670695).Methods: This retrospective study included 155 IgG4-RD patients who received GCs with CYC or MMF at the Department of Rheumatology at Peking Union Medical College Hospital between January 2012 and July 2018. Propensity score matching (PSM) was conducted to match two groups of patients based on their baseline clinical characteristics. Treatment response, relapse rate, and drug safety were analyzed. The treatment response was evaluated based on complete response (CR), partial response (PR), and no change (NC), and the cumulative relapse rate and adverse events in each treatment group were compared using Kaplan–Meier curves and log-rank test, respectively.Results: Of the 155 IgG4-RD patients, 90 were treated with GCs plus CYC (group I) and 65 with GCs plus MMF (group II). After propensity score–matched (PSM) analysis, 108 patients were selected (54 in each group), 49 of whom had “definite” IgG4-RD, 8 “probable” IgG4-RD, and 51 “possible” IgG4-RD. At the last follow-up, the total response in groups I and II was 98.15 and 96.3%, respectively, and within 12 months, the cumulative relapse rate in group II was significantly higher than that in group I (14.8 vs. 3.7%, P = 0.046). Recurrence occurred at the paranasal sinus, lacrimal glands, skin, lung, pancreas, and bile ducts, and the relapsed patients achieved remission after switching immunosuppressants or/and increasing the GC dose.Conclusions: In IgG4-RD patients with internal organ involvement, GCs plus CYC or MMF are both effective with similar effects in disease response, while GCs plus CYC reduced the relapse rate better than GCs plus MMF.
Highlights
Immunoglobulin G4–related disease (IgG4-RD) is a systemic fibroinflammatory condition that affects multiple organs including the pancreas, bile duct, lacrimal gland, salivary gland, thyroid, lung, liver, gastrointestinal tract, kidney, and retroperitoneum [1]
From January 2012 to July 2018, 155 IgG4-RD patients who conformed to the inclusion criteria were selected
To minimize the influence of confounders on the results of the study, we used 1:1 propensity score-matched (PSM) analysis to match group I patients with group II patients based on gender, age, involvement organs, and other involvement and constitutional symptoms not attributable to the involvement of a particular organ
Summary
Immunoglobulin G4–related disease (IgG4-RD) is a systemic fibroinflammatory condition that affects multiple organs including the pancreas, bile duct, lacrimal gland, salivary gland, thyroid, lung, liver, gastrointestinal tract, kidney, and retroperitoneum [1]. It is characterized by tissue infiltration by IgG4-positive cells, and causes prominent pathological changes in most of the affected organs, including lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis, as well as tumor-like lesions and even depletion [2]. This study aimed to compare the efficacy and safety of glucocorticoids (GCs) combined with cyclophosphamide (CYC) or mycophenolate mofetil (MMF) in IgG4-RD patients. This cohort study was registered at ClinicalTrials.gov (ID: NCT01670695)
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
