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Background Neuropsychiatric involvement is one of the major concerns in systemic lupus erythematosus (SLE). The therapeutic effect of intrathecal treatment of methotrexate and dexamethasone has been investigated in some exploratory studies, but its influence on the long-term prognosis of neuropsychiatric SLE (NPSLE) remains unknown. Methods This was a propensity score-matched retrospective study. Outcomes at discharge and time free from NPSLE relapse or death were evaluated by multivariate logistic regression, survival analysis, and Cox regression as appropriate. Results Among 386 hospitalized patients with NPSLE, the median [IQR] age was 30.0 [23.0–40.0] years, and 342 patients (88.4%) were female. Of those, 194 patients received intrathecal treatment. Patients in the intrathecal treatment group had higher Systemic Lupus Erythematosus Disease Activity Index 2000 scores (median 17 vs. 14 points, IQR 12–22 vs. 10–19 points, P P < 0.001) than those who did not receive intrathecal therapy. Intrathecal treatment was associated with a higher probability of survival and being free from NPSLE relapse than control treatment among the 386 unmatched patients (P =0.042 by log-rank test) and within 147 propensity score-matched pairs (P =0.032 by log-rank test). In the subgroup of NPSLE patients with increased levels of protein in cerebrospinal fluid, intrathecal treatment had a positive influence on their prognosis (P < 0.001). Conclusions Intrathecal treatment of methotrexate and dexamethasone was associated with a more favorable prognosis of NPSLE and may serve as a valuable additional therapy for NPSLE patients, especially for those with elevated levels of protein in cerebrospinal fluid.

Objective: Autoimmune comorbidities may be associated with IgG4-Related Disease (IgG4-RD), here we aimed to determine the correlation of AID family history and IgG4-RD in a Chinese cohort. Methods: This case-control studyidentified 628 cases of IgG4-RD in Peking Union Medical College Hospital. Patients were classified into two groups, with AID family history group (AID-pos) and without AID family history group (AID-neg). We viewed the potential value of AID family history on IgG4-RD by comparing the differences between the two groups. In addition, Cox regression analysis estimated confidence intervals (CI) and hazard ratio (HR) for IgG4-RD risk. Results: 93 (14.8%) IgG4-RD patients had AID family history. Compared with AID-neg group, baseline data analysis revealed that AID-pos group patients had an earlier age of IgG4-RD onset (50.4 ± 14.8 vs. 54.2 ± 12.6, p=0.014*), a higher percentage of antinuclear antibody (ANA) positivity (38.9% vs 22.7%, p=0.0277*) and IgG4-related thyroiditis (10.9% vs 2.4%, p=0.001*), were prone to comorbid with other AID (16.1% vs 6.2%, p=0.0238*). Cox analysis found that younger age (HR 0.97 [95%CI 0.94-0.99], p=0.0384*) and higher proportions of baseline peripheral eosinophils (HR 1.1 [95%CI 1.02-1.2], p=0.0199*) increased the risk of unfavorable prognosis for AID-pos IgG4-RD patients. Conclusions: 14.8% of IgG4-RD patients had AID family history, with younger age of disease onset age and higher frequency of ANA positivity, indicating that IgG4-RD may share genetic background with other AID.

Objective To figure out the functions of thymic stromal lymphopoietin (TSLP) in IgG4-related disease (IgG4-RD). Methods Plasma TSLP levels were tested by Elisa, and its receptors were detected by flow cytometry. Expressions of TSLP and TSLPR in involved tissues were stained by immunohistochemistry and immunofluorescence. Proliferation, apoptosis, and B subsets of TSLP stimulated-B cells were analyzed by flow cytometry. TSLP-stimulated B cells were co-cultured with CD4+ Naïve T cells. Signaling pathway was identified by RNA-sequencing and western blot. Anti-TSLP therapy was adapted in LatY136F knock-in mice (Lat, IgG4-RD mouse model). Results Plasma TSLP level was increased in IgG4-RD patients and was positively correlated with serum IgG4 level and responder index (RI). TSLPR was co-localized with CD19+ B cells in the submandibular glands (SMGs) of IgG4-RD. TSLP promoted B cell proliferation, and TSLP-activated B cells polarized CD4+ naive T cells into follicular helper T (Tfh) cells through OX40L. RNA-sequencing identified JAK-STAT signaling pathway in TSLP-activated B cells and it was verified by western blot. Anti-TSLP therapy alleviated the inflammation of lung in Lat mice. Conclusion Elevated TSLP in IgG4-RD promoted B cells proliferation and polarized Tfh cells and might be served as a potential therapeutic target.

A 56-year-old Chinese woman with previous disseminated mycobacterium avium complex infection and recurrent cervical abscesses from Burkholderia cepacia complex visited our hospital. She was diagnosed with adult-onset immunodeficiency (AOID) and tested positive for interferon-γ–neutralizing autoantibody. Ceftazidime was administered as the initial antimicrobial treatment, which was later combined with sulfamethoxazole-trimethoprim (SMZ-TMP). She developed drug rash with eosinophilia and systemic symptoms (DRESS) syndrome after SMZ-TMP administration and improved after withdrawal of the culprit antibiotic and systemic glucocorticoids treatment. Her cervical infection was eventually cured after combined therapy of long-term antibiotics and anti–IFN-γ autoantibodies (AIGA) titer-lowering treatments including glucocorticoids, rituximab, and plasmapheresis. This is the first case of DRESS syndrome in the setting of AIGA-induced AOID and is worthy of notice.

Systemic lupus erythematosus (SLE) is a prototypical autoimmune disease with high heterogeneity but the common characterization of numerous autoantibodies and systemic inflammation which lead to the damage of multiple organs. Aberrance of B cells plays a pivotal role in the immunopathogenesis of SLE via both antibody-dependent and antibody-independent manners. Escape of autoreactive B cells from the central and peripheral tolerance checkpoints, over-activation of B cells and their excessive cytokines release which drive T cells and dendritic cells stimulation, and dysregulated surface molecules, as well as intracellular signal pathways involved in B cell biology, are all contributing to B cell aberrance and participating in the pathogenesis of SLE. Based on that rationale, targeting aberrance of B cells and relevant molecules and pathways is expected to be a promising strategy for lupus control. Multiple approaches targeting B cells through different mechanisms have been attempted, including B-cell depletion via monoclonal antibodies against B-cell-specific molecules, blockade of B-cell survival and activation factors, suppressing T-B crosstalk by interrupting costimulatory molecules and inhibiting intracellular activation signaling cascade by targeting pathway molecules in B cells. Though most attempts ended in failure, the efficacy of B-cell targeting has been encouraged by the FDA approval of belimumab that blocks B cell-activating factor (BAFF) and the recommended use of anti-CD20 as a remedial therapy in refractory lupus. Still, quantities of clinical trials targeting B cells or relevant molecules are ongoing and some of them have displayed promising preliminary results. Additionally, advances in multi-omics studies help deepen our understandings of B cell biology in lupus and may promote the discovery of novel potential therapeutic targets. The combination of real-world data with basic research achievements may pave the road to conquering lupus.

ObjectiveAutoimmune comorbidities may be associated with IgG4-related disease (IgG4-RD), here we aimed to determine the correlation of autoimmune diseases (AID) family history and IgG4-RD in a Chinese cohort.MethodsThis retrospective cohort study identified 628 cases of IgG4-RD in Peking Union Medical College Hospital. Patients were classified into two groups, with AID family history group (AID-positive) and without AID family history group (AID-negative). We viewed the potential value of AID family history on IgG4-RD by comparing the differences between the two groups. In addition, Cox regression analysis estimated CIs and HR for IgG4-RD risk.Results93 (14.8%) IgG4-RD patients had AID family history. Compared with AID-negative group, baseline data analysis revealed that AID-positive group patients had an earlier age of IgG4-RD onset (50.4±14.8 vs 54.2±12.6, p=0.014*), a higher percentage of antinuclear antibody (ANA) positivity (38.9% vs 22.7%, p=0.0277*) and Riedel thyroiditis (10.9% vs 2.4%, p=0.001*), were prone to comorbid with other AID (16.1% vs 6.2%, p=0.0238*). Cox analysis found that younger age (HR 0.97 (95% CI 0.94 to 0.99), p=0.0384*) and higher proportions of baseline peripheral eosinophils (HR 1.1 (95% CI 1.02 to 1.2), p=0.0199*) increased the risk of unfavourable prognosis for AID-positive IgG4-RD patients.Conclusions14.8% of IgG4-RD patients had AID family history, with younger age of disease onset age and higher frequency of ANA positivity in AID-positive group, indicating that IgG4-RD may share genetic background with other AID.

Objective To describe clinical features and long-term prognosis in patients with Good syndrome (GS). Methods We retrospectively reviewed medical records of GS patients at Peking Union Medical College Hospital from January 2001 to May 2019. Data regarding clinical manifestations and treatments were collected. Patients were routinely followed-up via clinical and telephone interviews, and survival analysis was performed with Kaplan-Meier analysis. Results Twenty-four patients were identified, including eight males and 16 females, with a median age at diagnosis of 58 years (interquartile range [IQR], 52-62 years). Twelve patients (50%) had autoimmune manifestations. Multi-organ involvements included musculoskeletal (37.5%), respiratory (33.3%), gastrointestinal (29.2%), hematologic (29.2%) systems, et.al. Infections were detected in 23 (95.8%) patients, mostly located in lung (69.6%), blood (26.1%), and gastrointestinal tract (21.7%). Thymectomy was performed in 23 patients, with the most common histology of type AB (10, 47.6%). Twenty-one patients were consecutively followed-up with a median follow-up of 84 (IQR, 48-116) months and 11 (52.4%) died, mainly due to infection (8/11, 72.7%). The 5- and 10-year survival rates were 90% (95% confidence interval [CI], 77.8-100%) and 38.5% (95% CI, 19.6-75.5%), respectively. Conclusion GS patients tended to present with various infections and autoimmune manifestations. The 10-year survival rate from the Chinese population was poor, mainly due to infections.

Background Choledocholithiasis is an endemic condition in the world. Although rare, foreign body migration with biliary complications needs to be considered in the differential diagnosis for patients presenting with typical symptoms even many years after cholecystectomy, EPCP, war-wound, foreign body ingestion or any other particular history before. It is of great clinical value as the present review may offer some help when dealing with choledocholithiasis caused by foreign bodies. Case presentation We reported a case of choledocholithiasis caused by fishbone from choledochoduodenal anastomosis regurgitation. Moreover, we showed up all the instances of choledocholithiasis caused by foreign bodies published until June 2018 and wrote the world’s first literature review of foreign bodies in the bile duct of 144 cases. The findings from this case suggest that the migration of fishbone can cause various consequences, one of these, as we reported here, is as a core of gallstone and a cause of choledocholithiasis. Conclusion The literature review declared the choledocholithiasis caused by foreign bodies prefer the wrinkly and mainly comes from three parts: postoperative complications, foreign body ingestion, and post-war complications such as bullet injury and shrapnel wound. The Jonckheere-Terpstra test indicated the ERCP was currently the treatment of choice. It is a very singular case of choledocholithiasis caused by fishbone, and the present review is the first one concerning choledocholithiasis caused by foreign bodies all over the world.

Additional file 1. Timeline of the case.