Clinical features and relapse risks of IgG4-related ophthalmic disease: a single-center experience in China

Zhao, Zhen; Mou, Dapeng; Wang, Ziqiao; Zeng, Qiaozhu; Wang, Zhenfan; Xue, Jimeng; Ren, Limin; Li, Yanying; Su, Yin

doi:10.1186/s13075-021-02489-9

Cited by 11 publications

(11 citation statements)

References 41 publications

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“…Zhao et al. concluded that initial treatment with GC + IM was a protective factor against IgG4‐ROD relapse [15]. Consistently, through logistic regression analysis, we found that GC + IM maintenance was a protective factor for NOI.…”

Section: Discussionsupporting

confidence: 88%

“…ROI is the most common type of relapse. Several risk factors for relapse have been reported for IgG4‐related ophthalmic disease (IgG4‐ROD) and IgG4‐related dacryoadenitis and sialadenitis (IgG4‐DS) at baseline, including higher serum IgG4 levels and IgE levels, and increased eosinophil count [14, 15]. Consistently, relapse with ROI usually manifested as IgG4‐ROD and IgG4‐DS and with higher levels of serum IgG4 and IgG at baseline.…”

Section: Discussionmentioning

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: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

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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“…As a result, glucocorticoid sparing treatments have been used including azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide, and tacrolimus, but studies have not been conducted to compare efficacies of these agents [ 39 ]. Glucocorticoids with immunosuppressants have been shown to have a higher relapse-free rate than glucocorticoids alone [ 16 ]. In patients with refractory disease, rituximab can decrease serum IgG4 subclass concentrations.…”

Section: Discussionmentioning

confidence: 99%

“…Salivary and lacrimal gland involvement can cause facial and orbital swelling and can present similarly to Sjogren's syndrome with sclerosing sialadenitis [1,14]. Orbital pseudotumors involve the lacrimal gland in 69% of cases but can also occur in extraocular muscles, palpebrae, optic nerve, and orbital bone, as well causing exophthalmos and restriction of ocular movements [1,[15][16][17]. ese orbital manifestations tend to have higher relapse rates on steroids and overall have higher cumulative doses of corticosteroids [10].…”

Section: Introductionmentioning

confidence: 99%

Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency

Olson

Olson²

2021

Case Reports in Immunology

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We report a case of IgG4-RD in a patient with high IgG4 levels, low functional antibodies, and low IgM levels. He presented with bilateral orbital pseudotumors and, after initial improvement on corticosteroids, relapsed with recurrent pleural effusion and pelvic pseudotumor. He had a grossly elevated serum IgG (1905 mg/dl) with elevations in all IgG subclasses but marked elevation in IgG4 (412 mg/dl), low IgM, and low pneumococcal antibodies. Orbital mass biopsy showed polyclonal lymphocytic infiltration and increased IgG4 plasma cells. The patient was started on prednisone and tried several immunosuppressive medications including mycophenolate mofetil, methotrexate, hydroxychloroquine, and azathioprine with decrease in size of the orbital pseudotumor. During a period when the patient stopped his medications, the pseudotumor enlarged with new development of recurrent pleural effusions. He was also found to have a pelvic mass that was biopsy positive for IgG4 proliferation. This case with progression to multiorgan involvement highlights the importance of identifying patients with IgG4-related disease. In contrast to previous cases with normal-to-high IgM, the IgM was low with impaired functional antibodies.

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“…Larger cohorts of IgG4-ROD are needed to better understand its treatment outcomes and to provide a higher level of evidence around management of patients. The only study on a large cohort of IgG4-ROD was published recently (4). However, it did not report details of therapy and the exact relapse rate.…”

Section: Introductionmentioning

confidence: 99%

Long-Term Outcomes of IgG4-Related Ophthalmic Disease in a Chinese IgG4-Related Disease Cohort

et al. 2021

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Purpose: To define the treatment response and long-term outcomes of a large IgG4-related ophthalmic disease (IgG4-ROD) cohort.Methods: A total of 132 patients with a minimum follow-up of 1 year were included in this study. Demographic, clinical, and laboratory data were collected. Treatment response was assessed by the IgG4-RD responder index (IgG4-RD RI). Risk factors for relapse were analyzed with the multivariate Cox regression analysis.Results: The median follow-up time was 39 months. Lacrimal gland involvement was detected in 87.9% of cases. Extraocular muscles, the trigeminal nerve, and other soft tissue were affected in 25.8, 6.1, and 18.2% of patients. The relapse rate of watchful waiting, glucocorticoid monotherapy, immunosuppressant monotherapy, and combination therapy was 50.0, 51.7, 50.0, and 26.7% (p = 0.038), respectively. The combination therapy group exhibited shorter glucocorticoids therapy duration (36 vs. 48 months, p = 0.009) and maintenance period (24 vs. 42 months, p = 0.003). At the 6th month, the median IgG4-RD RI declined from 12 to 1 and 105 (79.5%) patients achieved complete response (CR). Relapse occurred in 49 (37.1%) patients. The multivariate Cox regression analysis exhibited that CR at the 6th month was an independent protective factor for relapse. Patients with multiple ocular lesions suffered from a higher risk of relapse. No patient had severe adverse reactions to the treatment in this study.Conclusion: Relapse was common in patients with IgG4-ROD. Patients receiving combination therapy showed a lower relapse rate and a shorter glucocorticoids therapy period. The presence of multiple ocular lesions was associated with a higher risk of relapse. CR at the 6th month might be a predictor for a better prognosis in IgG4-ROD. Thus, a more aggressive regimen should be prescribed for patients with a poor initial response.

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Clinical features and relapse risks of IgG4-related ophthalmic disease: a single-center experience in China

Cited by 11 publications

References 41 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

Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency

Long-Term Outcomes of IgG4-Related Ophthalmic Disease in a Chinese IgG4-Related Disease Cohort

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