2020 Vol. 11, No. 3

Editorial
Abstract:
Belimumab is a human monoclonal antibody that inhibits B-cell activating factor of the tumor necrosis factor family(BAFF). With the approval of belimumab for treating patients with systemic lupus erythematosus (SLE), targeted biologics has generally ushered a new era in the treatment of SLE. The pathogenesis of SLE involves a general breakdown in both B cell and T cell tolerance. In this review, we focused on the new promising therapeutic targets and several ongoing clinical trials for SLE. The approaches of these biologic therapeutic agents included targeting B cell selective cell surface molecules (CD20 or CD19), inhibiting B cell survival by targeting cytokines and signaling molecules (BAFF or a proliferation-inducing ligand), interfering with B cell antigen presentation by targeting co-stimulatory molecules (CD40-CD40 ligand interactions or ICOS-ICOS ligand interactions), blocking the signal pathways (rigerimod, interferon-α, or JAK/STAT), et al. Biologic target therapies for SLE have made some progress and bringing successful new biologic therapies into the clinical practice for SLE remains challenging but promising in the future.
Specialist Forum
Abstract:
There have been exciting advances in the treatment for systemic lupus erythematosus(SLE) with a better understanding of the pathogenesis. Since the long term use of traditional medicines such as glucocorticoids may have negative impacts on the outcome, novel treatments have increasingly specific targets that result in fewer adverse side effects. Medicines newly approved or undergoing clinical trials, such as rituximab, belimumab, anifrolumab, and baricitinib, mainly target at lymphocytes, cytokines, or their intracellular signaling pathways. A combinatory treatment that simultaneously blocks multiple targets may bring better efficacy. Development of the pharmacological treatment is a process evolving from chemical agents to biologics and back to small molecule chemical inhibitors. The modality of treatment is involved in a spiral manner from non-specific to specific and then to the combination of multi-target therapy.
Abstract:
Human papilloma virus(HPV)can cause pre-malignant cervical abnormalities and cervical cancer as well as benign conditions such as anogenital warts, especially serotype 16 and 18, which were closely associated with cervical cancer. The prevalence of HPV infection and multiple HPV infections in patients with systemic lupus erythematosus(SLE) is higher than general population, and the risk of abnormal Pap smears and cervical high grade intraepithelial lesions are significantly increased. It is very important to prevent HPV infection in patients with SLE. Vaccine is one of the most effective tools to prevent the infectious diseases. Three non-live protein subunit vaccines for HPV were approved in the last decade:bivalent(HPV 16, 18), quadri-valent(HPV 6, 11, 16, 18), and 9 valent(HPV 6, 11, 16, 18, 31, 33, 45, 52, 58) vaccines. These vaccines were developed to prevent pre-malignant cervical and cervical cancer. qHPV and 9 valent vaccines also prevent benign conditions caused by HPV, such as anogenital warts. Multiple prospective studies showed HPV vaccines were safe and efficacious in patients with SLE, and protective immune response could be produced. In 2019 update of EULAR recommendations for vaccination in adult patients with autoimmune inflammatory rheumatic diseases(AⅡRD), it is recommended that patients with AⅡRD, in particular patients with SLE, should receive vaccinations against HPV in accordance with recommendations for the general population.
Abstract:
Systemic lupus erythematosus (SLE) is a common autoimmune disease characterized by chronic inflammation and multi-organ damage and mainly affects women of childbearing age. SLE can only be controlled but not cured currently. The occurrence and development of SLE were reported to be associated with genetic factors as well as environmental triggers. In recent years, mounting evidence showed that there were compositional and functional changes in the gut microbiota of SLE patients. Also, the disturbed gut microbiota was suggested to partake in SLE pathogenesis by damaging the gut barrier, as well as inducing or aggravating chronic inflammation and autoimmunity. This review summarized the progress of research on the gut microbiota in SLE, discussed the clinical value of gut microbiota-related interventions, and envisions the future direction of research related to gut microbiota in lupus.
Abstract:
Neuropsychiatric systemic lupus erythematosus (NPSLE) is a group of severe complications with a poor prognosis and high mortality caused by systemic lupus erythematosus (SLE). Its lesions involve the nervous system and cause neurological and/or psychiatric symptoms. Unique autoantibodies detected in the serum or cerebrospinal fluid of patients with NPSLE are associated with the presence of neuropsychiatric disorders. The prevalence of NPSLE ranges from 17% to 75% in SLE. This article reviewed the latest progress and focused on the current state of knowledge and the potential clinical value of anti-ribosomal P antibody, antiphospholipid antibody, anti-neuronal antibody, and other related antibodies in NPSLE with central nervous involvement.
Abstract:
Systemic lupus erythematosus(SLE) is a disease with a high incidence in women of childbearing age, especially 20-40 years old. Most patients at this age will have the need for pregnancy. However, when SLE combines with pregnancy, pregnant women are facing not only the risk of relapse and aggravation of lupus, but also the increased risk of adverse pregnancies. Therefore, it is very important to do well in the comprehensive management of pregnancy in SLE patients. This paper focused on the interaction between SLE and pregnancy, as well as the management of SLE in pregnant patients, aiming to better understand and improve the pregnancy outcome of SLE patients.
Abstract:
Childhood-onset systemic lupus erythematosus (cSLE), which refers to SLE before the age of 18, is the second systemic connective tissue disease in children. Compared with adults, cSLE has its own characteristics in epidemiology, clinical presentation, and treatment. In recent years, with the rapid development of human genomics and molecular biology, the understanding of the disease has been updated. This paper reviewed recent progress in the etiology, manifestation, and treatment of cSLE, so as to better understand this disease and guide clinical practice.
Guideline Interpretation
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2019 update of the EULAR recommendations for the management of systemic lupus erythematosus is the second edition that EULAR has developed for the general management of systemic lupus erythematosus (SLE) since 2007. The treatment strategy, validated goals of treatment, alternative regimens of glucocorticoids, "multi-targeted" therapy in lupus nephritis, and biological therapy for SLE were updated in the recommendations, which indicated the direction for the treatment of SLE. A detailed interpretation of the updated guidelines is provided in this article, aiming to provide references for the clinical diagnosis and treatment of SLE.
Abstract:
  Objective  This study aimed to evaluate the diagnostic value of percutaneous ultrasound-guided (US-guided) biopsy for pancreatic neoplasms, and to compare the evaluation of fine needle aspiration(FNA) and core needle biopsy (CNB).  Methods  Data of consecutive patients who had percutaneous US-guided biopsies of pancreatic masses for the diagnosis of suspiciously malignant solid pancreatic masses in Peking Union Medical College Hospital between January 2014 and April 2017 were retrospectively analyzed. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were evaluated based on the biopsy methods (FNA vs. CNB). The final diagnosis was determined according to a combination of follow-up (no shorter than 6 months) imaging, clinical course evaluation, repeated biopsy and/or surgical pathology.  Results  A total of 160 patients who met the inclusion and exclusion criteria were enrolled in this study, including 95 in the FNA group and 65 in the CNB group. The overall sensitivity, specificity, PPV, NPV, and accuracy of percutaneous US-guided biopsy were 93.8%, 100%, 100%, 64.0%, and 94.4%, respectively. The sensitivity, NPV, and accuracy of the CNB group were higher than those of FNA group (94.7% vs. 93.1%,72.7% vs. 57.1%,95.4% vs. 93.7%), but there was no statistical difference between the two groups. Two cases with major complications (1.3%, 2/160) were observed, including one acute pancreatitis and one tumor seeding.  Conclusions  Percutaneous US-guided biopsy is an effective and safe diagnostic method for pancreatic neoplasms. The current data do not suggest significant differences in the diagnostic efficacy of FNA and CNB.
Original Contributions
Abstract:
  Objective  The aim of this study was to compare the efficacy and prognosis of different therapeutic regimens in treating idiopathic membranous nephropathy (IMN) in patients with type 2 diabetes.  Methods  The clinical data were retrospectively collected and analyzed in patients with IMN and type 2 diabetes admitted in the Peking Union Medical College Hospital from January 2004 to April 2015. The patients were classified into three groups and designated as cyclophosphamide (CTX), calcineurin inhibitors (CNIs)and other immunosuppressants groups according to the initial therapeutic regimens. Kaplan-Meier analysis and multivariable Cox regression were used to assess the influence of therapeutic regimens on total remission (TR), complete remission (CR), risks of developing ≥ 30% decline in estimated glomerular filtration rate (eGFR) or end-stage renal disease (ESRD), and risks of achieving fast blood glucose >8 mmol/L.  Results  Totally, 89 patients meeting the inclusive and exclusive criteria were included in this study. The multivariable Cox analysis showed that patients initially treated with CNIs would be significantly higher to develop ≥ 30% eGFR decline or ESRD (HR=13.505, 95% CI:1.512-120.665, P=0.020) than those treated with CTX. However, the likeliness to reach CR(HR=0.793, 95% CI:0.315-1.999, P=0.623), TR(HR=0.647, 95% CI:0.334-1.252, P=0.196), or fast blood glucose >8 mmol/L (HR=1.709, 95% CI:0.669-4.369, P=0.263) were similar between the two groups.  Conclusions  In patients with type 2 diabetes, CNIs as the initial treatment for IMN might have comparable remission rate of proteinuria with CTX, but an extra risk of renal function decline. We would better be cautious in choosing the therapeutic regimens to treat IMN in patients with type 2 diabetes.
Reviews
Abstract:
Documenting the seizure attack accurately is curial for evaluating the medical treatment for epileptic patients and acquiring reliable data from clinical trials. However, nearly half of all seizures captured during the long-term video-electroencephalography monitoring were not reported by patients. Therefore, innovative and feasible tools for automatic seizure detection are in great demand. This article reviewed the most recent advances in the technique of seizure detection.
Abstract:
Multiple myeloma has unique manifestations of bone disease, mainly presenting as osteolytic lesions and diffuse osteoporosis as a result of over-activation of osteoclasts and suppression of osteoblasts. The severity of myeloma bone disease is negatively correlated with quality of life and survival. Plain X ray has low sensitivity to contour destructive bone lesions. Whole body low dose CT does not differentiate active foci from tranquil ones. Functional imaging including diffuse weighted imaging-magnetic resonance imaging (DWI-MRI) and positron emission tomography/computed tomography (PET/CT) are more sensitive in evaluation of comprehensive tumor load and detection of early disease. Moreover, minimal residue disease detected by DWI-MRI or PET/CT is a complement of flow cytometry and predicts progressive disease in patients achieving complete response. In this article, we summarized the role of imaging methods in evaluating myeloma bone disease and tumor load.
Complicated and Rare Disease
Abstract:
Hydatidiform mole is a benign gestational trophoblastic disease. Twin pregnancy with hydatidi-form mole and a coexistent fetus is extremely rare. The diagnosis is usually delayed and the pregnancy is often terminated due to severe vaginal bleeding or other complications. There are few guidelines for termination methods. Here we report a case of successful labor induction and vaginal delivery of twin pregnancy with hydatidiform mole and a coexistent fetus at 16 weeks of gestation. The diagnosis, treatment principles, and termination methods are discussed.
Clinical Research and Evidence Based Medicine
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Meta-analysis is an important method of systematic review. With the development of evidence-based medicine, there are more and more meta-analysis published. Reading and interpreting meta-analysis becomes one of the important methods for obtaining cutting-edge information. Mastering meta-analysis is essential for physicians in clinical work and research. We explained the forest plot, chart of bias evaluation, and funnel plot in detail through examples in this article. Moreover, we introduced the evaluation method of heterogeneity, robustness, and sample size to assess whether the results of the meta-analysis are reliable and can be applied to the clinical practice, in order to help physicians to correctly grasp the interpretation methods of meta-analysis.
Clinical Practice Guidelines
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In recent years, the number of clinical practice guidelines has been growing rapidly. An increasing number of identical or similar guidelines on the same topic have been developed and published for diseases. There are certain differences or conflicts in methodological quality and contents of recommendation. Clinical staffs face a lot of confusion when using the guidelines. A systematic review of guidelines can present more comprehensively and objectively the current status and differences in guidelines on the same topic, and can better guide clinical practice and improve the quality of healthcare service. Thus, we discussed the definition, methods, and challenges in systematic review of guidelines, and put forward some suggestions on how to develop a systematic review of guidelines, so as to provide references for guideline developers, users, and researchers.
Drug Therapy for Tumors
Abstract:
Curative resection remains the most effective and the only potentially curative therapy for biliary tract cancer(BTC). Most of the patients with BTC present with an advanced (inoperable or metastatic) disease, and the relapse rate is high in those undergoing curative resection. In Meta-analysis, compared with surgery only, there was a significant improvement in overall survival with any adjuvant therapy after surgery. Although the BILCAP study failed to meet its primary endpoint by intention to treat analysis, a survival benefit was seen in a preplanned sensitivity analysis. Gemcitabine combined with cisplatin is still the first-line chemotherapy for patients with advanced disease. Gemcitabine with TS-1, or gemcitabine with cisplatin and TS-1 become an alternative to the first-line chemotherapy. IDH1 mutations and FGFR2 fusions have been positioned as the two main driving alterations in intrahepatic cholangiocarcinoma, and are the hot spots for targeted therapies. Immunotherapy alone or combined with other therapy is still on the way in the treatment of advanced BTC. This review discussed the past, present and exciting future of the medical treatments of cholangiocarcinoma.
In Memory of Acadimician SHI Yi-fan
Abstract:
Since its establishment in 1979, the multidisciplinary pituitary team (MPT) of Peking Union Medical College Hospital(PUMCH) has gone through more than 40 years. It is the first pituitary diagnosis and treatment center in China. Through the inheritance and improvement of generations in PUMCH, it has formed a mature operation mode with a complete process and distinct characteristics, which provides a systematic scheme for the diagnosis and treatment for refractory and complex pituitary tumors and plays an important role in clinical treatment, medical education, and scientific research related to pituitary diseases. This paper summarized the development history of MPT, the mode and flow path of diagnosis and treatment, typical cases, further thought and enlightenment, so as to provide experiences and references for the promotion of MPT in China.
Abstract:
There are a large number of patients in China, but the distribution of medical resources is unbalanced. The diagnosis and treatment level of pituitary diseases in medical institutions is uneven, which makes this kind of disease easily misdiagnosed and improperly treated. With the development of hospital information technology and internet technology, a large number of clinical medical data can build and improve the database of pituitary diseases after data cleaning. With artificial intelligence technology and real world research methods, data are deeply mined, summarized, and analyzed. In view of problems encountered in clinical practice, we should carry out relevant clinical research, build a more efficient and consistent auxiliary solution to clinical diagnosis and treatment, guide clinicians to formulate more reasonable diagnosis and treatment strategies, and realize individualized diagnosis and treatment for pituitary diseases.
Abstract:
Establishing correct clinical thinking is the core and foundation of improving doctors' clinical ability as well as the key and difficult point of medical education. Different from common diseases, clinical thinking about pituitary diseases has its unique characteristics as a rare disease. Taking a case of Hypothalamic and pituitary mass as an example, this paper summarized the characteristics of diagnosis thinking of rare diseases from the aspects of data collection, logical reasoning, evidence-based teaching, multiple disciplinary teams, and basic medical research, to improve the efficiency of physicians and provide experience for the training of clinical thinking about pituitary diseases.
MDT from PUMCH
Abstract:
Intracardiac thrombus is a rare complication of Antiphospholipid syndrome secondary to systemic lupus erythematosus (SLE). Its treatment is extremely tough and requires a multidisciplinary approach. We reported a case of SLE with intracardiac thrombus and pulmonary embolism. The patient was complicated with hemoptysis and kidney injury, and had a high risk of sudden death from incidental thrombus shedding off. In the state of thrombosis and hemorrhage, the management of anticoagulation and the evaluation and treatment of the primary disease complicated with infection are challenging. The multidisciplinary collaboration helpedus to create a more favorable surgical condition while controlling SLE aggressively. After surgical treatment at the proper time, the outcome of the patient was satisfying.
Teaching and Research
Abstract:
  Objective  The purpose of this study was to explore the value of short-term training in the standardized training and examining system of thyroid nodule ultrasound.  Methods  We prospectively recruited trainees who were trained in the Department of Ultrasound, Peking Union Medical College Hospital in December 2017, and then assessed using the standardized training and examining system of thyroid nodule ultrasound. The ultrasonic characteristics and malignant risk classification of thyroid nodules were investigated. The scores were automatically recorded by the system. The training part of the system was then used for corresponding training. After twelve hours and one month, the system was used again to evaluate all participants, and the differences in scores before and after training were compared.  Results  A total of 12 participants were enrolled in this study, and all had received professional training in the ultrasonic diagnosis of thyroid diseases. The results of thyroid nodule reading were significantly improved after 12 hours and one month(80.7±7.3 vs. 69.9±4.0, P < 0.01;78.0±9.2 vs. 69.9±4.0, P < 0.05). The diagnostic ability of recognition of ultrasonic features and thyroid node classification was improved (P < 0.05). Compared with the results 12 hours after training, the diagnostic level of thyroid nodule ultrasound did not drop significantly one month later (P> 0.05).  Conclusions  The standardized training and examining system of thyroid nodule ultrasound can improve trainees' ability to diagnose thyroid nodules with ultrasound in a short time, and at the same time, it provides new teaching methods for the standardization process of thyroid ultrasound in the future.
2020, 11(3): 358-360. doi: 10.3969/j.issn.1674-9081.20190066
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