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Overall survival of systemic lupus erythematosus (SLE) patients significantly increased in recent decades, however, the relative risk of mortality is still high. Long-term survival outcome of pediatric SLE remains unclear. This study aims to explore the long-term survival rate and its predictors in patients with systemic lupus erythematosus (SLE).A retrospective, hospital-based cohort study was performed between 2004 and 2018 in a tertiary referral medical center in Taiwan. Data on comorbidities, medications, and causes of admission were collected for risk factor analysis using time-dependent multivariate Cox proportional hazards models.A total of 2392 adults and 115 pediatric SLE patients were enrolled (female, n = 2157 and 95, respectively). The 10-year survival rates were 93.2%, 90.2%, 98.9%, and 100% in adult women, adult men, girls, and boys with SLE, respectively. The overall mortality rate was 2.09 case/100 patient-years (PY) for male SLE and 1.39 case/100 PY for female SLE patients. Male SLE patients did not have a statistically significantly higher mortality rate than female SLE patients in each age stratification. Infectious disease (n = 119), heart failure (n = 21), and cerebrovascular accident (n = 14) were the leading causes of death in adult SLE patients. Advanced age (hazard ratio [HR]: 1.04, 95% confidence interval [CI]: 1.03-1.05), treatment with mean dosage of systemic glucocorticoid equivalent to10 mg/d of prednisolone (HR: 1.71, 95% CI: 1.14-2.57), comorbidities with malignancy (HR: 1.94, 95% CI: 1.22-3.09), chronic kidney disease (HR: 1.86, 95% CI: 1.25-2.77), hypertension (HR: 1.42, 95% CI: 1.01-1.98), and admission due to bacterial pneumonia (HR: 1.92, 95% CI: 1.12-3.31) and sepsis (HR: 2.78, 95% CI: 1.51-5.13) were independent risk factors for mortality in SLE patients.SLE patients with advanced age, malignancy, chronic kidney disease, hypertension, treated with a higher average dosage of glucocorticoids, and admission due to bacterial pneumonia and sepsis have an increased risk of mortality.

The real-world outcomes of golimumab (GLM) use have been rarely studied in Asian patients with rheumatoid arthritis (RA). This study assessed the real-world effectiveness and safety of GLM in a Taiwanese cohort.One hundred and eight GLM-treated RA patients were enrolled. Predictors of a good European League Against Rheumatism (EULAR) response at 24 months and drug retention were identified through multivariate analyses.After 24 months of GLM treatment, the mean Disease Activity Score using 28 joint counts with the erythrocyte sedimentation rate (DAS28-ESR) decreased from 6.7 to 3.1 (p0.001). Up to 58.9% of patients achieved a good EULAR response at 24 months. Multivariate logistic regression analysis revealed that after adjustment for other variables, a higher baseline C-reactive protein was an independent negative predictor of good EULAR responses (odds ratio, 0.82; 95% confidence interval [CI], 0.67-0.99; p = 0.043). During the mean follow-up period of 38.3 months, 15 (13.9%) patients discontinued GLM due to treatment failure. In multivariate analysis, high baseline ESR level, high DAS28-ESR, and the experience of biologic therapy were independent risk factors for GLM discontinuation (adjusted hazard ratio [HR], 1.03; 95% CI, 1.01-1.05; p = 0.003; adjusted HR, 2.93; 95% CI, 1.42-6.08; p = 0.004; and adjusted HR, 5.00; 95% CI, 1.75-14.26; p = 0.003, respectively). In receiver operator characteristic curve analysis, the optimal cutoff values of baseline ESR and DAS28-ESR for predicting drug survival were 52 mm/h (sensitivity: 60.0% and specificity: 77.4%) and 7.7 (sensitivity: 46.7% and specificity: 94.3%), respectively. During the follow-up period, 22 patients (20.4%) developed adverse events. The safety profile of GLM in this study was comparable with that in previous clinical trials.GLM was effective and safe for the real-life management of Taiwanese RA patients and showed a high retention rate in biologic-naive patients compared with biologic-experienced patients.

Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized heterogeneous, subacute, and usually silent autoimmune disease involving many organs with protean manifestations. However, high IgG4 in serum is not necessarily indicating an IgG4-RD. The aims of this study were to investigate the clinical relevance of high serum IgG4 level in IgG4-RD or non IgG4-RD patients, and to see if IgG4-RD in Taiwan differs from that in other parts of the world.Eight hundred forty-five patients with high IgG4 were retrospectively reviewed from January 2002 to May 2020 in Taipei Veteran General Hospital. Two hundred sixty-seven patients fulfilled IgG4-RD criteria and were categorized into pancreato-hepato-biliary disease, retroperitoneal fibrosis and/or aortitis, head/neck-limited disease, classic Mikulicz syndrome with systemic involvement, CNS-limited disease, sclerosing vasculitis, skin-limited disease, and sensorineural hearing disease. These manifestations were correlated to smoking, atopy, hyper-IgE/eosinophilia, aging, malignancies, and hypocomplementemia. Five hundred seventy-eight patients were not fulfilling the criteria but were also analyzed for the prevalence of allergy, malignancy, connective tissue diseases, lung diseases, and infections.In IgG4-RD patients, 124 (46.4%) smoked. Top 4 clinical subtypes included Mikulicz syndrome with systemic involvement (33.3%), pancreato-hepatobiliary disease (31.4%), head/neck disease (19.4%), and retroperitoneal fibrosis/aortitis (12.7%). Top 4 co-morbid conditions included high serum IgE/eosinophilia (46.2%), hypocomplementemia (34%), malignancies (13.4%), and allergy (13.4%). Pancreato-biliary disease was associated with high IgE/eosinophilia (rSmoking may precipitate IgG4-RD. IgG4-RD with pancreato-hepatobiliary disease is closely related to allergy and neoplasm, and those with Mikulicz syndrome may result from atopy. Elderly IgG4-RD patients tend to develop CNS pathology parallel to advancing of age. The disease may probably be originated from an unknown mechanism that may sporadically evolve into malignancies.

Background Our aim was to characterize etiologic diagnoses obtained from bronchoalveolar lavage fluid (BALF) and blood specimens, and to identify risk factors for mortality in systemic lupus erythematosus (SLE) patients with pneumonia and respiratory failure. Methods We conducted a retrospective analysis of SLE patients with pneumonia and respiratory failure. Clinical characteristics, laboratory profiles, and microbiology in BALF and blood samples were evaluated. We performed univariable analyses to identify mortality risk factors. Results All 24 patients (F:M = 21:3, median age 46.5 years; disease duration 11 years) received mechanical ventilation (median duration: 11 days). Pathogens identified in BALF included Pneumocystis jiroveci (12 patients [50%]), cytomegalovirus (CMV, 7 patients [29.2%]), and bacteria (11 patients [45.8%]). Thirteen patients (54.2%) yielded pathogens in blood (CMV in 8 patients [33.3%] and Escherichia coli in 5 patients [20.8%]). Eight developed septic shock, and 9 died within 30 days. Univariable analysis identified thrombocytopenia (odds ratio [OR]: 8.0, 95% confidence interval [CI]: 1.23–52.25), bacteremia within 30 days before or after endotracheal intubation (OR: 8.0, 95% CI: 1.23–52.5), and P. jiroveci pneumonia (PJP, OR: 7.0, 95% CI: 1.04–46.95) as risk factors for 30-day mortality. Kaplan–Meier analysis confirmed an increased risk of 30-day mortality with thrombocytopenia and bacteremia. Conclusion There are high prevalence rates of PJP and CMV infections as evidenced by BALF analyses in SLE patients with pneumonia and respiratory failure. BALF analysis can facilitate rescue therapy per pathogen. Thrombocytopenia, bacteremia, and PJP in SLE patients can increase their 30-day mortality, so warrant early and aggressive treatments.

Objective.To evaluate the role of cytomegaloviral or Pneumocystis jiroveci pneumonia (CMV/PJP) in systemic lupus erythematosus (SLE) patients with pulmonary hemorrhage (PH).Methods.We retrospectively examined hospital records for 27 SLE patients with PH who received bronchoalveolar lavage fluid (BALF) analyses. Clinical profile and mortality rates were compared between groups with and without CMV/PJP. Risk factors for PH-related mortality were analyzed.Results.Among 27 SLE patients with PH, 15 had pathogens from BALF samples, and 8 had CMV/PJP. Although CMV/PJP was treated, the RR for 90- and 180-day mortality rates of SLE patients with CMV/PJP were higher than those without these infections (5.94, 95% CI 1.44–24.48; 7.13, 95% CI 1.81–28.06, respectively). Risk factors for 90- and 180-day mortality were presence of CMV/PJP (OR 14.2, 95% CI 1.83–109.9; OR 25.5, 95% CI 2.91–223.3, respectively) and use of pulse methylprednisolone for PH treatment (OR 12.0, 95% CI 1.48–97.2; OR 8.5, 95% CI 1.13–63.9, respectively). Factors increasing the 90-day mortality rate were duration of mechanical ventilation exceeding 14 days (OR 11.1, 95% CI 1.11–112.0) and use of aggressive immunosuppression close to PH onset (OR 7.56, 95% CI 1.09–52.4). Three of the 7 patients receiving aggressive immunosuppression died with the presence of CMV/PJP.Conclusion.Owing to the high prevalence of CMV/PJP and its association with mortality, routine BALF analysis is recommended for all suitable SLE patients with PH. Use of aggressive immunosuppression does not benefit SLE patients with opportunistic infections during PH attack.

Objective The objective of this paper is to identify the risk of complications of real-time ultrasound-guided renal biopsy in adult and pediatric patients with systemic lupus erythematosus (SLE). Materials and methods This retrospective study examined outcomes of 296 renal biopsy procedures in 275 SLE patients. Imaging-confirmed symptomatic hematoma was regarded as a major complication when intervention (blood transfusion, angiographic embolization, or surgery) was required or as a minor complication otherwise. Clinical and laboratory data were compared between groups with or without complications after initial or subsequent renal biopsy. Binary logistic regressions were used to evaluate complication risk of initial renal biopsy. Results Overall complication rate of initial renal biopsy was 8.7% (major: 2.9%, minor: 5.8%). Three patients expired from pulmonary hemorrhage, thrombotic microangiopathy, and pneumonia. Pediatric SLE patients tended to have a higher rate of major complications (12.5%) than adult patients (2.3%). According to multivariable analysis results, elevated serum creatinine (SCr) level (OR 1.45; 95% CI 1.17-1.81 per mg/dl), prolonged prothrombin time (PT) (OR 2.2; 95% CI 1.05-4.62 per second), and thrombocytopenia (OR 4.3; 95% CI 1.56-11.9) increased overall complication risk of initial renal biopsy. Age 18 years (OR 8.43; 95% CI 1.21-58.8), thrombocytopenia (OR 16.4; 95% CI 2.44-110.5), and elevated SCr level (OR 1.97; 95% CI 1.36-2.86 per md/dl) increased risk of major complications. Thrombocytopenia, prolonged PT, and elevated SCr level were associated with complications after subsequent renal biopsy (all p = 0.01). Conclusions SLE patients, particularly patients under 18 years old or with elevated SCr level, prolonged PT, or thrombocytopenia, have an increased risk of complications after initial or subsequent renal biopsy.

To evaluate the role of cytomegaloviral orWe retrospectively examined hospital records for 27 SLE patients with PH who received bronchoalveolar lavage fluid (BALF) analyses. Clinical profile and mortality rates were compared between groups with and without CMV/PJP. Risk factors for PH-related mortality were analyzed.Among 27 SLE patients with PH, 15 had pathogens from BALF samples, and 8 had CMV/PJP. Although CMV/PJP was treated, the RR for 90- and 180-day mortality rates of SLE patients with CMV/PJP were higher than those without these infections (5.94, 95% CI 1.44-24.48; 7.13, 95% CI 1.81-28.06, respectively). Risk factors for 90- and 180-day mortality were presence of CMV/PJP (OR 14.2, 95% CI 1.83-109.9; OR 25.5, 95% CI 2.91-223.3, respectively) and use of pulse methylprednisolone for PH treatment (OR 12.0, 95% CI 1.48-97.2; OR 8.5, 95% CI 1.13-63.9, respectively). Factors increasing the 90-day mortality rate were duration of mechanical ventilation exceeding 14 days (OR 11.1, 95% CI 1.11-112.0) and use of aggressive immunosuppression close to PH onset (OR 7.56, 95% CI 1.09-52.4). Three of the 7 patients receiving aggressive immunosuppression died with the presence of CMV/PJP.Owing to the high prevalence of CMV/PJP and its association with mortality, routine BALF analysis is recommended for all suitable SLE patients with PH. Use of aggressive immunosuppression does not benefit SLE patients with opportunistic infections during PH attack.