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BackgroundOral corticosteroid remains the first-line treatment of IgG4-related ophthalmic disease, but steroid-dependence is common and serious. Factors associated with steroid dependence and relapse have to be further explored.Study populationA city-wide, biopsy-proven, Chinese cohort.MethodsRetrospective, masked review of medical records, orbital images and histopathology reports.ResultsThere were 101 patients with at least 24-month follow-up. Up to 82% (82/101) received oral corticosteroid as first-line treatments, and 7 of them received also concomitant steroid-sparing agents (SSA)/biological agents as primary treatment. There was 61% (50/82) of patients required long-term corticosteroid (alone=23, with SSA=27) after 1.9±0.7 (range 1–5) relapses. When compared with the 21% (17/82) of patients who tapered corticosteroid successfully for 24 months, steroid dependence was associated with elevated baseline serum IgG4 level (94% vs 65%, p<0.01) and Mikulicz syndrome (46% vs 18%, p<0.05). Up to 13% (11/82) of patients tolerated residual disease after tapering off corticosteroid. There was 17% (17/101) of patients did not require any medications after biopsies. They were more likely to have debulking surgeries (71% vs 40%, p<0.05), discrete orbital lesions (65% vs 26%, p<0.05), normal baseline serum IgG4 level (24% vs 6%, p<0.05) and no Mikulicz syndrome (94% vs 61%, p<0.05).ConclusionIn this cohort, 60% of patients required long-term maintenance oral corticosteroid. Elevated pretreatment serum IgG4 level and Mikulicz syndrome were associated with steroid dependence. Debulking surgery is an alternative for a subgroup of patients with discrete orbital lesions, normal baseline IgG4 level and no Mikulicz syndrome.

Purpose Clinical phenotypes in Immunoglobulin G4-related disease (IgG4-RD) according to the patterns of affecting organs have different risks of malignancies. We attempt to determine the association of malignancies with IgG4-related ophthalmic disease (IgG4-ROD).Design Retrospective cohort study.Methods Review of medical records, orbital images and histopathology reports in a territory-wide cohort of biopsy proven IgG4-ROD patients from 2005–2019.Findings Among 122 patients who had biopsies taken from adnexal lesions including lacrimal glands (n = 108), orbital mass (n = 30), infiltrated orbital fat (n = 10), conjunctiva (n = 2) or extraocular muscles (n = 3), 13% (16/122) developed malignancies over 73 ± 48months’ follow-up. There were 9 cases of ocular adnexal lymphoma (OAL) and 7 extra-orbital malignancies. Compared with the general population, the incidence of OAL was significantly higher (standardized incidence ratios, SIRs = 10.0, 95%CI = 4.5–17.6) while that of extra-orbital malignancies was similar. The SIRs was highest within the first year (SIR = 46.7, 95%CI = 18.5–87.6) when 7 OAL were concomitantly diagnosed. Patients who developed OAL or extra-orbital malignancies were older than other patients at IgG4-ROD diagnosis (64.9 ± 7.1, 68.3 ± 8.5 versus 55.2 ± 15.0 years, P < 0.05). Asymmetric lacrimal gland enlargement (78% versus 13%), lack of frontal (0% versus 12%) or infraorbital nerve enlargement (0% versus 36%) were associated with OAL (all P < 0.05). Pre-treatment serum IgG4 level or extra-orbital IgG4-RD involvement was similar among patients with or without malignancies.Conclusion In this biopsy-proven IgG4-ROD cohort, 7% developed OAL which was 10 times higher than the general population. Patients with asymmetric lacrimal gland enlargement or without trigeminal nerves involvement radiologically were associated with OAL.

Emerging evidence suggest that antibiotic prophylaxis may be omitted in early cirrhosis patients with upper gastrointestinal bleeding (UGIB), which question the benefits of antibiotic prophylaxis on rebleeding, mortality related to ongoing bleeding, and the need for salvage therapy. As the management of UGIB has improved over time since the last review a decade ago, we performed an updated meta‐analysis to review the benefits of antibiotic prophylaxis in cirrhosis patients with UGIB. Six electronic databases including PubMed/MEDLINE, EMBASE, Scopus, Web of Science, Cochrane library, and ClinicalTrial.govwere systematically searched up to December 1, 2021. The primary outcome was 6 weeks mortality. Secondary outcomes include the risk of infection, rebleeding at 7 days and 6 weeks, mortality related to ongoing bleeding, need for salvage therapy, and infection‐related mortality. Eighteen studies (12 randomized controlled trials [RCT], 6 non‐RCT) from 3180 subjects were identified among 2129 citations. Antibiotic prophylaxis reduces mortality at 6 weeks, risk of infection, and infection‐related mortality (pooled relative risk: 0.72, 0.39, and 0.41, respectively). Although antibiotics reduce the risk of rebleeding and the amount of blood transfusion, they did not reduce the risk of mortality from ongoing bleeding nor the need for salvage therapy. Antibiotic prophylaxis may shorten the length of stay in the intensive care unit. Antibiotic prophylaxis reduces rebleeding, 6‐week mortality, and infection‐related mortality. Due to the low risk of infection and death, dedicated studies are warranted to evaluate the benefit of antibiotic prophylaxis in early cirrhosis with UGIB. Our updated meta‐analysis reaffirms the benefit of antibiotic prophylaxis in reducing the mortality at 6 weeks, risk of infection, and infection‐related mortality.Although antibiotics reduce the risk of rebleeding and the amount of blood transfusion, they did not reduce the risk of mortality from ongoing bleeding nor the need for salvage therapy.Antibiotic prophylaxis may reduce the length of stay in the intensive care unit.Dedicated studies are required to study the benefit of prophylactic antibiotic among early cirrhosis patients with upper gastrointestinal bleeding. Our updated meta‐analysis reaffirms the benefit of antibiotic prophylaxis in reducing the mortality at 6 weeks, risk of infection, and infection‐related mortality. Although antibiotics reduce the risk of rebleeding and the amount of blood transfusion, they did not reduce the risk of mortality from ongoing bleeding nor the need for salvage therapy. Antibiotic prophylaxis may reduce the length of stay in the intensive care unit. Dedicated studies are required to study the benefit of prophylactic antibiotic among early cirrhosis patients with upper gastrointestinal bleeding.