Objective:To improve clinical understanding of secondary antiphospholipid antibody (aPLs) related multiple organ damage in systemic lupus erythematosus (SLE).Methods:A woman of reproductive age, admitted to Peking Union Medical College Hospital in June 2023, presented with recurrent disease manifestation, this case of SLE with secondary APS with multiple systems involvement such as the hematological system, heart valves, and nervous system was reported. The diagnosis and treatment process were discussed and analyzed.Results:A woman of reproductive age presented with recurrent symptoms. Initially, she was clinically diagnosed with idiopathic thrombocytopenic purpura. Over the course of the disease, she subsequently developed chronic renal insufficiency, mitral valve vegetations, and recurrent episodes of acute stroke. Laboratory investigations revealed positivity for antinuclear antibodies and aPLs. Her condition was generally improved after standardized anticoagulation therapy combined with intensive immunosuppressive treatment.Conclusion:In the context of SLE, elucidating whether the mechanisms underlying organ involvement are associated with aPLs is of significant importance for understanding the pathogenesis of the disease and guiding therapeutic strategies.

Objective:To improve clinical understanding of diffuse alveolar hemorrhage (DAH) secondary to antiphospholipid syndrome (APS).Methods:A patient with DAH as the initial symptom and positive for antiphospholipid antibodies (aPLs) was reported, and their clinical characteristics and diagnosis and treatment process were analyzed.Results:A 43 year old male patient presented with "cough and hemoptysis for more than 3 months", and the imaging and bronchoalveolar lavage fluid confirmed DAH. During the course of the disease, there was a decrease in platelets and an increase in inflammatory markers, as well as high titers of various antiphospholipid antibodies. After excluding other potential causes, the final diagnosis was APS secondary DAH. After receiving high-dose glucocorticoid and immunosuppressant, the patient′s condition was relieved, pulmonary lesions were absorbed, platelets recovered, and aPLs titer decreased.Conclusion:For unexplained DAH, it is necessary to further exclude infection and other factors before screening autoimmune diseases. The patient was diagnosed as APS-DAH after actively screening the cause, and the lesion was absorbed after hormone and cyclophosphamide treatment.Early identification of microvascular events is crucial for improving patient prognosis.

Systemic lupus erythematosus (SLE) is a highly heterogeneous systemic autoimmune disease characterized by multi-organ involvement, recurrent flares, and chronic progression. With advances in diagnostics and therapeutics, SLE management is shifting from disease control toward long-term remission and organ protection. Incorporating recent global evidence and characteristics of the Chinese population, the National Clinical Research Center for Dermatologic and Immunologic Diseases and the Chinese SLE Treatment and Research Group (CSTAR) have developed the Chinese guidelines for the diagnosis and treatment of systemic lupus erythematosus (2025 edition). Centered on a treat-to-target strategy, the guidelines prioritize clinical remission and low disease activity as primary goals, optimizing the use of glucocorticoids, immunosuppressants, biologics, and novel therapies. Key updates include individualized management recommendations for lupus nephritis, multi-organ involvement, and antiphospholipid syndrome, alongside first-ever evidence-based recommendations on vaccination in SLE patients. By integrating large-scale domestic cohort studies and real-world data, this edition provides more precise and actionable guidance, offering significant value for standardizing and improving SLE diagnosis, treatment, and long-term management in China.

Objective:To improve clinical understanding of secondary antiphospholipid antibody (aPLs) related multiple organ damage in systemic lupus erythematosus (SLE).Methods:A woman of reproductive age, admitted to Peking Union Medical College Hospital in June 2023, presented with recurrent disease manifestation, this case of SLE with secondary APS with multiple systems involvement such as the hematological system, heart valves, and nervous system was reported. The diagnosis and treatment process were discussed and analyzed.Results:A woman of reproductive age presented with recurrent symptoms. Initially, she was clinically diagnosed with idiopathic thrombocytopenic purpura. Over the course of the disease, she subsequently developed chronic renal insufficiency, mitral valve vegetations, and recurrent episodes of acute stroke. Laboratory investigations revealed positivity for antinuclear antibodies and aPLs. Her condition was generally improved after standardized anticoagulation therapy combined with intensive immunosuppressive treatment.Conclusion:In the context of SLE, elucidating whether the mechanisms underlying organ involvement are associated with aPLs is of significant importance for understanding the pathogenesis of the disease and guiding therapeutic strategies.

Objective:To improve clinical understanding of diffuse alveolar hemorrhage (DAH) secondary to antiphospholipid syndrome (APS).Methods:A patient with DAH as the initial symptom and positive for antiphospholipid antibodies (aPLs) was reported, and their clinical characteristics and diagnosis and treatment process were analyzed.Results:A 43 year old male patient presented with "cough and hemoptysis for more than 3 months", and the imaging and bronchoalveolar lavage fluid confirmed DAH. During the course of the disease, there was a decrease in platelets and an increase in inflammatory markers, as well as high titers of various antiphospholipid antibodies. After excluding other potential causes, the final diagnosis was APS secondary DAH. After receiving high-dose glucocorticoid and immunosuppressant, the patient′s condition was relieved, pulmonary lesions were absorbed, platelets recovered, and aPLs titer decreased.Conclusion:For unexplained DAH, it is necessary to further exclude infection and other factors before screening autoimmune diseases. The patient was diagnosed as APS-DAH after actively screening the cause, and the lesion was absorbed after hormone and cyclophosphamide treatment.Early identification of microvascular events is crucial for improving patient prognosis.

Objective:To analyze the relationship between the level of erythrocyte sedimentation rate (ESR) and clinical manifestations, and to discuss the clinical significance of ESR in patients with SSc.Methods:Patients with SSc registered in Peking Union Medical College Hospital from January 2009 and May 2022 in the database of National Rheumatism Data Center (CRDC) were included. All patients fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SSc. The clinical features and laboratory tests were analyzed. T test was used for analyzing the mea-surement data with normal distribution, and the results were expressed as mean±SD deviation. Wilcoxon signed-rank test or Wilcoxon rank sum test were used to analyze the measurement data that did not conform to normal distribution. The results were expressed as M ( Q1, Q3). The count data were compared with Chisquare test or Fisher's exact test. Binary logistic regression analysiswas used to analyze independent variables. P val-ue<0.05 was considered to be statistically significant. Results:A total of 308 SSc patients were enrolled in the single center of Peking Union Medical College Hospital, including 280 females (90.9%), with the average age of (47 ±12) years old. SSc patients with elevated ESR combined woth anemia were more common. Compared with normal ESR group, elevated ESR group had higher incidence of pulmonary interstitial disease (80.8% vs. 67.6%, χ2=5.89, P=0.015), pulmonary hypertension (31.7% vs. 21.1%, χ2=4.20, P=0.040) and myositis(26.9% vs. 15.7%, χ2=5.54, P=0.019). In laboratory tests, anemia is highly frequent in SSc patients with increased ESR. The increase in CK, hs-CRP, IgA and IgG were more common, and the val-ues of IgA and IgG were sta-tistically higher. In antibody tests, anti-dsDNA antibody, anti-RNP antibody, anti-SSA and anti-SSB antibodies positivity were higher, and anti-Scl70 antibody positivity was less common ( P<0.05). Multivariate regression analysis indicated elevated IgG [ B=1.733, OR(95% CI)=5.657(2.839, 11.272), P<0.001], anemia [ B=1.083, OR(95% CI)=2.952(1.376, 6.333), P=0.005)], positive anti-SSA antibody [ B=1.665, OR(95% CI)=5.287 (2.367, 11.809), P<0.001] were independent factors for SSc patients with elevated ESR are more common. IgG and positive anti-SSA antibody were strong risk factor for increased ESR. Conclusion:SSc patients with elevated ESR are more commonl with anemia, elevated IgG and positive anti-SSA anti-body, which may be related to disease activity. Long-term follow-up for these patients is helpful to guide clini-cal doctors′ treatment choices.

Objective:We sought to investigate the clinical characteristics and risk factors of antiphospholipid syndrome (APS) complicated by autoimmune hemolytic anemia (AIHA).Methods:Retrospective anaysis.Three hundred fifteen consecutive patients with APS were enrolled at the Department of Rheumatology of Peking Union Medical College Hospital between May 2017 to May 2021, and their clinical manifestations[including initial symptoms, time interval between APS onset and diagnosis, systemic lupus erythematosus(SLE), thrombotic events, obstetric morbidity, and extra-criteria manifestations] and laboratory test results[including blood routine, antiphospholipid antibodies(aPLs), blood lipid profile, homocysteine, anti-nuclear antibody profile, immunoglobulin levels, and complement levels] were collected. Then, univariate and multivariate logistic regression analyses were performed. Clinical features and risk factors were analyzed using univariable and multivariable logistic regression analysis.Results:Among 315 APS patients, 37 cases (11.7%) were complicated by AIHA, and AIHA was the first manifestation or co-occurrence. The median time interval between APS onset and diagnosis was 12 months. The proportion of SLE in APS patients combined with AIHA was higher than that in APS patients without AIHA[62.2%(23/37) vs. 19.4%(54/278), P<0.001]. There was no significant difference in the proportions of thrombosis and pregnancy morbidity between the two groups. In terms of extra-criteria manifestations, APS patients with AIHA had a significantly ( P<0.05) greater risk of thrombocytopenia ( OR=6.19, 95% CI 2.81-13.65) and higher proportions of hypocomplementemia, a positive lupus anticoagulant (LA) result, double aPLs positivity[i.e., any two of the following antibodies were positive: LA, anticardilolipin antibody(aCL), and anti-β2 glycoprotein Ⅰ(β2GPⅠ)], and triple aPLs positivity (i.e., LA, aCL, and anti-β2GPⅠ antibodies were all positive). Multivariate logistic regression analysis showed that SLE ( OR=3.46,95% CI 1.60-7.48), thrombocytopenia ( OR=2.56,95% CI 1.15-5.67), and hypocomplementemia ( OR=4.29,95% CI 2.03-9.04) were independent risk factors for the complication of APS. In the primary APS subgroup, multivariate logistic regression analysis showed that livedo reticularis ( OR=10.51,95%CI 1.06-103.78), thrombocytopenia ( OR=3.77, 95% CI 1.23-11.57), and hypocomplementemia ( OR=5.92,95% CI 1.95-17.95) were independent risk factors for the complication of APS. Conclusions:AIHA is not rare in APS patients; moreover, it occurs more frequently in APS secondary to SLE and is more likely to present with a variety of extra-criteria manifestations. Patients with AIHA should be promptly tested for antiphospholipid antibody profiles and alerted to the possibility of thrombotic events.

Sjögren's syndrome （SS）， a disorder of immune system， is one of the dominant diseases treated by traditional Chinese medicine （TCM）. China Association of Chinese Medicine organized experts in the field of TCM and western medicine rheumatology and pharmacology to discuss the advantages and optimal regimens of TCM for the treatment of SS. The experts generally agreed on the low early diagnosis rate of SS and the lack of targeted therapeutic drugs. In addition， autoimmune abnormality is the key factor in the occurrence of SS and deficiency of both Qi and Yin is the core pathogenesis. SS has unique tongue manifestations， which is expected to allow for the early diagnosis and treatment with integrated traditional Chinese and western medicine. TCM has advantages in treating SS in terms of alleviating clinical symptoms and systemic involvement， individualized treatment， relieving sleep and mood disorders， preventing the occurrence in the early stage， and enhancing the effectiveness and reducing toxicity in the treatment by integrated TCM and western medicine. In general， TCM has advantages in different stages of SS. Internal and external use of TCM， acupuncture， and acupotome are all available options. The optimal regimens should be determined on the basis of pattern identification， stage of disease， and the advantages of TCM. Clinical characteristics and biomarkers of SS should be studied to classify patients， so as to design precision evidence-based TCM regimens for SS. On the basis of unique tongue manifestations of SS， models for early diagnosis and poor prognosis identification of SS should also be established to achieve early prevention and treatment and to improve the prognosis. In the future， we should vigorously carry out high-quality evidence-based medical research on the treatment of SS by TCM and integrated traditional Chinese and western medicine and develop relevant guidelines to optimize and standardize current diagnosis and treatment， thereby laying a basis for clarifying and explaining the advantages of TCM in treating SS.

Humans ; Tacrolimus/therapeutic use* ; Lupus Erythematosus, Systemic/drug therapy* ; Immunosuppressive Agents/therapeutic use* ; Treatment Outcome

Interstitial lung disease (ILD) is a frequent complication of patients with connective tissue disease (CTD) and significantly affects morbidity and mortality. Disease course may vary from stable or mildly progressive to more severe, with rapid loss of lung function. At present, there are great challenges and poor prognosis in the diagnosis and treatment of CTD-ILD. Based on the evidence and guidelines from China and other countries, experts from the Chinese Rheumatology Association developed standardization of diagnosis and treatment of CTD-ILD. The aim is to strengthen the early identification of, standardize the diagnosis and treatment of CTD-ILD, and delay the progress of the disease.