北京大学学报（医学版）最新文献

This article reports the diagnosis and treatment process of a 52-year-old female patient who was finally diagnosed with POEMS syndrome. Her main clinical manifestations included Raynaud phenomenon of both hands, skin pigmentation, swelling of both hands and feet, and numbness of both feet. The patient was admitted to the Department of Rheumatology and Immunology, Peking University People's Hospital on April 8, 2024, due to "purplish red skin on the neck and chest for 1.5 years, swelling of both hands for 1 year, and numbness of both feet for 8 months". One and a half years ago, she was diagnosed with systemic sclerosis (SSc) in another hospital, based on evidence from a series of clinical manifestations. Her main symptoms were Raynaud phenomenon of both hands, skin pigmentation, swelling of both hands and feet, and numbness of both feet, which met the 2013 classification criteria for SSc by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). During the disease course, the patient received regular treatment with glucocorticoids and disease-modifying antirheumatic drugs (DMARDs), but her condition still progressed continuously. Eight months ago, she developed new-onset numbness in both her feet accompanied by pinprick-like pain, indicating the complexity of the condition and the need for further investigation of the etiology. Differential diagnosis should consider possibilities, such as mixed connective tissue disease, eosinophilic fasciitis, and malignant tumors. Auxiliary examinations showed that the patient's serum antinuclear antibody, anti-topoisomerase Ⅰ(Scl-70) antibody, anti-U1 ribonucleoprotein (RNP) antibody, and antineutrophil cytoplasmic antibody were all negative. Imaging examinations revealed no pulmonary arterial hypertension or pulmonary interstitial fibrosis. In addition, the patient had multiple endocrine abnormalities and responded poorly to treatment with glucocorticoids and DMARDs, suggesting the need to be alert to the possibility of lymphoproliferative diseases. Further examinations including vascular endothelial growth factor detection, whole-body bone scan, and bone marrow aspiration and biopsy were performed, and the final diagnosis of POEMS syndrome was confirmed. The patient was then transferred to the Department of Hemato-logy and received treatment with the pomalidomide combined with dexamethasone regimen, and her clinical symptoms gradually relieved. This case suggests that POEMS syndrome is similar to rheumatological and immunological diseases such as SSc in terms of clinical manifestations. Clinicians should be more vigilant during diagnosis and treatment, and pay attention to differentiation, so as to reduce missed diagnoses and misdiagnoses, thereby formulating more accurate and effective treatment plans for patients.

Objective: To evaluate the use of aspirin during pregnancy in patients with systemic lupus erythematosus (SLE) and to assess its effects on pregnancy outcomes.

Methods: We consecutively enrolled SLE patients discharged from the Department of Obstetrics at Peking University Third Hospital between 2010 and 2024. Collected data included general patient characteristics, such as age, histories of adverse pregnancy, thrombosis, hypertension and renal disease. SLE related organ involvement, antiphospholipid antibodies (aPLs), SLE disease activity index (SLEDAI) score, medication regimens during pregnancy, and pregnancy outcomes were all documented. Differences in clinical characteristics between the aspirin user group and the non-user group were compared. Logistic regression analysis was used to assess the impact of aspirin on pregnancy outcomes.

Results: A total of 171 SLE patients were included in this study. The mean age was (31±4) years, and 46 patients had a history of adverse pregnancy. The most commonly involved organs were skin and joints, accounting for 68.4% and 45.6% respectively. In the study, 52 cases had renal involvement, accounting for 30.4%. SLEDAI scores during pregnancy of the 87.1% patients were less than 4 scores. Aspirin use during pregnancy accounted for 48.5%. Among them, 19 patients (11.1%) used between 2010 and 2017, while 64 patients (37.4%) used after 2017, demonstrating an increasing trend. Regarding pregnancy outcomes, the rates of fetal loss, preterm birth, preeclampsia/eclampsia, and early-onset preeclampsia were 14.0%, 23.4%, 22.8%, and 10.5%, respectively. After adjusting for covariates such as age, adverse pregnancy history, SLEDAI score, and aPLs, aspirin use was a protective factor for live birth (OR=2.34, 95%CI: 1.18－4.65, P=0.015) and reduced the incidence of preeclampsia/eclampsia and early-onset preeclampsia (OR=0.42, 95%CI: 0.19－0.91, P=0.028; OR=0.31, 95%CI: 0.11－0.89, P=0.029, respectively) for the total 171 SLE patients. Among the SLE pregnant patients without high-risk factors for preeclampsia/eclampsia, aspirin use was a protective factor for live birth (OR=8.22, 95%CI: 1.61－42.16, P=0.012) and might help reduce the incidence of early-onset preeclampsia/eclampsia (OR=0.26, 95%CI: 0.06－1.10, P=0.067).

Conclusion: Aspirin can reduce the incidence of preeclampsia/eclampsia, early-onset preeclampsia, and stillbirth in pregnant SLE patients. Even for those without high-risk factors for preeclampsia/eclampsia, aspirin should be taken under physician evaluation and recommendation. Current clinical practice in managing SLE during pregnancy deviates from guideline recommendations, underscoring the need for greater standardization.

The disease "Sjögren syndrome" has been widely known as a "syndrome" for a long time. However, this nomenclature and its classification as "primary" and "secondary" have raised debates in recent years. The word "syndrome" denotes an aggregate of symptoms and signs that are associated with a morbid process, independent of pathogenesis. It is now well recognized that "Sjögren syndrome" is an independent systemic autoimmune disease with specific clinical manifestations, serological markers and associated underlying pathogenesis mechanisms. Thus, the use of "syndrome" to designate this disease is not accurate. Furthermore, the traditional distinction between "primary" and "secondary" forms fails to account for the complex interplay between overlapping autoimmune diseases. Based on this background, the International Task Force on Nomenclature of Sjögren disease was established with the aim to develop a rational consensus on the nomenclature of Sjögren syndrome. Following the literature review and collecting experiences from both international professionals as well as patients, the International Task Force on Nomenclature of Sjögren disease published the "2023 International Rome Consensus for the Nomenclature of Sjögren disease" in 2025. The consensus issued five recommendations, officially recommended the use of "Sjögren disease" as nomenclature for this disease, and the acronym "SjD" be used for its abbreviation. It also recommended the descriptor "associated" should be used in lieu of "secondary" for Sjögren disease occurring in association with a second systemic autoimmune disease. The change from "Sjögren syndrome" to "Sjögren disease" improved clarity in both clinical practice and research, highlighted the distinct pathogenesis of this disorder.

Objective: To systematically compare serum metabolome differences between patients with thrombocytopenia in primary Sjögren syndrome (pSS) and those with normal platelet count using non- targeted metabolomics technology, so as to identify differential metabolites, analyze the relationship between the relative quantification of these metabolites and platelet counts, and screen metabolic pathways associated with platelet counts in pSS patients with thrombocytopenia.

Methods: The patients with pSS were selected and grouped according to the presence or absence of thrombocytopenia. Serum samples were collected from the study subjects and analyzed by liquid chromatography-mass spectrometry (LC-MS). The samples were analysed by human metabolome database (HMDB), lipid metabolites and pathways strategy (LIPID MAPS) and other databases for classification and annotation. The samples were analyzed by principal component analysis (PCA) and partial least squares discriminant analysis (PLS-DA) for multi-variate statistical analysis to screen the differential metabolites between the groups, and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analysis was made to study the functions and metabolic pathways of the metabolites. Correlation analysis was performed between the abundance of serum differential metabolites and platelet counts of pSS patients with thrombocytopenia.

Results: This study included 62 patients with pSS, of whom 32 had thrombocytopenia and 30 had normal platelet counts. A total of 137 differentially expressed metabolites, enriched in 54 metabolic pathways, were found in the serum of patients with thrombocytopenia compared with those without thrombocytopenia. Among them, the expression of desoxycorticosterone, hydrocortisone, and taurine was positively correlated with platelet count, and the expression of neopterin was negatively correlated with platelet count. Enrichment analysis showed that desoxycorticosterone and hydrocortisone were enriched in the steroid hormone biosynthesis pathway, taurine was enriched in the metabolic pathway of taurine and taurine, and neopterin was enriched in the folate metabolic pathway.

Conclusion: Thrombocytopenia in pSS patients may be related to the reduced activity of steroid hormone biosynthesis pathway and the metabolic pathway of taurine and taurine, and the increased activity of the pathway of folate metabolism.

Objective: To assess the associations between brachial-ankle pulse wave velocity (baPWV), ankle brachial index (ABI) and all-cause and cardiovascular mortality in a rural population in north China.

Methods: The current study utilized the baseline data of Beijing Fangshan family cohort study and the data of the death surveillance system of the Beijing Fangshan District Center for Disease Prevention and Control. The main outcomes were all-cause mortality and cardiovascular mortality. Cardiovascular deaths which included deaths from coronary heart disease (CHD), stroke, heart failure, sudden cardiac death and arrhythmia, were coded according to the International Classification of Diseases, Ninth Revision (ICD-9) and Tenth Revision (ICD-10). The R4.2.2 software was used for statistical analysis, and the adjusted hazard ratios (HR) for all-cause and CVD mortality associated with baPWV and ABI were calculated using Cox proportional hazards regressions with shared frailty models.

Results: A total of 7 686 participants were followed up for a median of 6.35 years in Fangshan District, Beijing, China. Totally 576 deaths were identified, with a mortality density of 11.88/1 000 person-years, of which 335 deaths were from cardiovascular diseases. We found that baPWV (HR=1.40, 95%CI: 1.02-1.92) and ABI (HR=3.32, 95%CI: 2.57-4.28) were associated with all-cause mortality after adjusting for confounding factors. ABI was more strongly associated with cardiovascular mortality than baPWV. There was no significant difference in the risk of all-cause mortality among different subgroups. The risk of cardiovascular mortality was significantly increased in the participants with hypertension (HR=1.72, 95%CI: 1.30-2.27).

Conclusion: baPWV and ABI were associated with all-cause and cardiovascular mortality in a rural population of north China. The association of ABI and cardiovascular mortality was more significant than that of baPWV. And abnormal baPWV or ABI was associated with cardiovascular mortality, especially in people with hypertension.

Objective: To investigate the correlation between superb microvascular imaging (SMI) blood flow grading, ultrasound semi-quantitative scores, and clinical symptom severity in patients with primary knee osteoarthritis (KOA).

Methods: A total of 94 knees from 47 patients with primary KOA were evaluated. Ultrasound semi-quantitative scoring and synovial SMI grading were performed for each knee joint. Clinical assessments included the Western Ontario and McMaster Universities osteoarthritis index (WOMAC), visual analog scale (VAS) for pain, and serum biomarkers, such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Correlations between clinical scores and ultrasound parameters were analyzed.

Results: SMI demonstrated a significantly higher synovial blood flow detection rate than power Doppler (PD) (14.9% vs. 9.6%, Z=-2.531, P=0.011). WOMAC stiffness scores showed positive correlations with synovitis, articular cartilage damage, and elevated SMI scores (all P < 0.05). WOMAC function scores were positively correlated with osteophyte severity, synovial thickening, and elevated SMI scores (all P < 0.05). The total WOMAC scores were positively associated with synovitis, synovial thickening, articular cartilage damage, and osteophyte severity (all P < 0.05). However, non-parametric Bootstrap analysis (B=2 000 replicates) revealed no independent associations between SMI blood flow grading, age, or body mass index (BMI) and WOMAC pain, stiffness, function, or total scores (all P>0.05, 95%CI contained zero).

Conclusion: The SMI technique demonstrates significantly higher sensitivity than PD in detecting intensity grade of synovial microvascular flow. While SMI compensates for the limitations of PD in identifying low-grade inflammation, its high sensitivity to low- velocity blood flow did not correlate with symptom severity or WOMAC pain scores. These findings suggest that SMI serves as a valuable tool for visualizing microvascular activity in subclinical synovitis, but its role as a direct indicator of clinical symptom severity in KOA remains limited. Further studies with larger sample sizes are warranted to validate its clinical utility.