Different factors underlie recurrent and de novo organ involvement in immunoglobulin G4–related disease

Mizushima, Ichiro; Tsuge, Shunsuke; Fujisawa, Yuhei; Hara, Satoshi; Suzuki, Fae; Ito, Kiyoaki; Fujii, Hiroshi; Yamada, Kazunori; Kawano, Mitsuhiro

doi:10.1093/rheumatology/kez321

Cited by 4 publications

(7 citation statements)

References 19 publications

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“…Mizushima et al. [10] only included serum IgG4 levels at baseline in the regression analysis, neither at follow‐up nor the relapse/baseline ratio. Interestingly, the ratio of relapse/baseline IgG4 ≥ third quartile (≥74.03%) was an independent risk factor and correlated with the highest weight in the prediction model.…”

Section: Discussionmentioning

confidence: 99%

“…The baseline characteristics of the two groups are shown in Table 1. Compared with patients with NOI, the frequency of allergy history, 2019 ACR/EULAR classification diagnostic score, and IgG4-RD RI were higher in those without NOI (47 [58.02%] vs. 17 [38.64%], 30 [25][26][27][28][29][30][31][32][33][34][35][36][37][38][39] vs. 25 [19][20][21][22][23][24][25][26][27][28][29], and 8 [6][7][8][9][10][11][12] vs. 6 [4][5][6][7][8]; P = 0.0383, P = 0.0001, and P = 0.0005, respectively). No significant differences were found in age, male-to-female ratio, or disease duration.…”

Section: Demographic Features and Assessment Of Relapsed Patientsmentioning

confidence: 99%

“…Clinically, there are two forms of disease recurrence in IgG4-RD patients: new organ involvement (NOI) and recurrent organ involvement (ROI). Recently, a study including 86 patients (20 patients with ROI and 15 with NOI) from Japan found that risk factors for recurrence and de novo organ involvement are different in IgG4-RD, and they emphasized that de novo organ involvement after glucocorticoid (GC) cessation should be the focus [10]. Except for the ROI alone and NOI alone, some patients experienced multiple recurrences with both ROI and NOI during long-term follow-up.…”

Section: Introductionmentioning

confidence: 99%

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Prediction of new organ onset in recurrent immunoglobulin G4–related disease during 10 years of follow‐up

Liu

Peng

et al. 2022

J Intern Med

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Objective. Frequent relapse is a prominent challenge in managing immunoglobulin G4-related disease (IgG4-RD). According to the types of organs involved in relapse, relapse patterns were divided into recurrent organ involvement (ROI) and new organ involvement (NOI). We aimed to investigate the discrepancy in clinical relapse patterns and establish an effective prognostic nomogram for NOI.Methods. We retrospectively enrolled 125 IgG4-RD patients who experienced relapse during the follow-up period. Patients were classified into two groups: those with NOI (including NOI and NOI + ROI) and without NOI (ROI). Logistic regression analyses were used to assess the risk factors for NOI. The results were externally validated by a separate prospective cohort of 39 patients with relapse.Results. There were 81 (64.8%) and 44 (35.2%) patients without NOI and with NOI, respectively. Patients without NOI showed higher baseline disease activity. The most common ROIs were the lacrimal gland and submandibular gland, while the lung and urinary system were the most involved in NOI. Re-elevation of serum IgG4 level to 74.31% of baseline was associated with NOI. Multiple relapses, organ involvement type at baseline, glucocorticoids combined with immunosuppressive drugs (IM) or IM alone during the maintenance period, and relapse IgG4/baseline IgG4 ratio were included in the nomogram. Both internal and external validations showed good agreement and discrimination.Conclusions. About one third of IgG4-RD patients with relapse suffer from NOI. We developed a risk stratification model that can effectively predict the future risk of NOI. Glucocorticoid and IM combined therapy during maintenance is also recommended.

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Section: Discussionmentioning

confidence: 99%

Section: Demographic Features and Assessment Of Relapsed Patientsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Prediction of new organ onset in recurrent immunoglobulin G4–related disease during 10 years of follow‐up

Liu

Peng

et al. 2022

J Intern Med

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“…Risk factors of recurrent organ involvement were younger age and ANA positivity, whereas those of de novo organ involvement were elevation of peripheral blood eosinophil counts and discontinuation of glucocorticoids/observation without glucocorticoids. 78 …”

Section: Prognosismentioning

confidence: 99%

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Mizushima¹,

Kawano²

2021

IJNRD

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Retroperitoneal fibrosis (RPF) is a rare disorder consisting of idiopathic and various secondary forms and characterized by chronic inflammatory infiltrates and marked fibrosis in the retroperitoneal space. In idiopathic RPF (IRPF), 35–60% of cases have been reported to be IgG4-related RPF, the retroperitoneal lesions of IgG4-related disease (IgG4-RD). IRPF can frequently lead to renal insufficiency mediated by urinary tract obstruction and hydronephrosis irrespective of being IgG4-related or not. Clinical pictures, laboratory and imaging findings, and location of the urinary tract obstruction are generally similar in IgG4-related and non-IgG4-related IRPF although multiple organ involvement and serum IgG4 elevation may be characteristic of the IgG4-related forms. Periaortic/periarterial lesions are the most frequent cause of renal insufficiency. Although the response to glucocorticoids is generally good, relapse does occur in a considerable proportion of patients, and may require an additional immunosuppressive agent and/or urological intervention in cases with multiple relapses or refractory obstructive uropathy. In general, the prognosis of patients with IRPF is good, but careful attention needs to be paid to chronic kidney disease as a major complication and rupture of the affected aorta/artery as a life-threatening one. Further studies are necessary to better understand the pathogenesis of the disease and to establish the optimal diagnostic and therapeutic strategies for it.

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“…However, there were no differences in the adverse events (odds ratio 0.96; 0.08, 12.09) [12], which suggests that achieving drug-free remission is very difficult with glucocorticoids alone and that maintenance therapy with glucocorticoids alone might involve a certain risk of relapse. In cases with a risk of relapse, such as male patients, young patients [13], history of allergy, multi-organ involvements [14], higher levels of serum IgG4 [15], peripheral counts of eosinophils [14,16] and patients with repeated relapses, the use of immunosuppressants may be considered during the course of treatment.…”

Section: Introductionmentioning

confidence: 99%

B cell targeted therapy for immunoglobulin G4-related disease

Yamamoto

2021

Immunological Medicine

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Glucocorticoids are the first-line drug for the remission induction therapy of immunoglobulin (Ig) G4-related disease. Achieving drug-free remission using glucocorticoids alone is difficult, however, and many patients require maintenance therapy with glucocorticoids and immunosuppressants. Studies have recently found that the number of peripheral memory B cells and plasmablasts is increased in IgG4-related disease and have indicated the efficacy of rituximab, which, in remission induction therapy, rapidly reduces serum IgG4 levels and has the tapering effect of glucocorticoids. Rituximab has been shown to reduce the risk of relapse more than oral immunosuppressants such as azathioprine. However, maintaining drug-free remission is difficult with a single course of rituximab alone, and many cases require maintenance therapy with rituximab. This article outlines the potential of B-cell targeted therapy, focusing on the efficacy, and safety of rituximab for IgG4-related disease.

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Different factors underlie recurrent and de novo organ involvement in immunoglobulin G4–related disease

Cited by 4 publications

References 19 publications

Prediction of new organ onset in recurrent immunoglobulin G4–related disease during 10 years of follow‐up

Prediction of new organ onset in recurrent immunoglobulin G4–related disease during 10 years of follow‐up

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

B cell targeted therapy for immunoglobulin G4-related disease

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