This article reports the diagnosis and treatment course of a patient with granulomatosis with polyangiitis (GPA) complicated with Kennedy disease (KD). The patient presented with headache, purulent and bloody rhinorrhea, ocular pain, decreased hearing and vision, multiple nodular erythema with ulcers on both lower extremities, and lower limb weakness. Chest imaging revealed a nodule with cavity formation in the right lung, and cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) and proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) were positive. The patient was initially diagnosed with granulomatosis with polyangiitis. After treatment with glucocorticoids and cyclophosphamide, the vasculitis-related symptoms were relieved while lower limb weakness got worse. Genetic testing identified 50 CAG repeats in exon 1 of the androgen receptor (AR) gene, and the final diagnosis was GPA complicated with KD. After rehabilitation therapy, the patient′s lower limb weakness improved. Methylprednisolone combined with leflunomide was administered for maintenance treatment, and the condition remained stable during long-term follow-up.

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: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.

The 21-year-old male patient was admitted to the Department of Rheumatology and Immunology at Peking Union Medical College Hospital with chief complaints of "skin rash for 1 year and edema for 2 months". He was diagnosed with systemic lupus erythematosus (SLE) with renal, cardiac and hematological involvement. Remission was not achieved after glucocorticoid pulse treatment. The patient experienced oliguria, malignant hypertension, accompanied by thrombocytopenia and low serum complements, and elevated lactate dehydrogenase and serum creatinine. Schistocytes were seen in the peripheral blood smear. Thrombotic microangiopathy (TMA) secondary to SLE was diagnosed. Though plasma exchange was partially effective, TMA could not be controlled yet. The activity of serum von Willebrand factor -cleaving protease (ADAMTS 13) was 100%, and ADAMTS 13 inhibitor was negative. Finally, remission of the disease was achieved after second glucocorticoid pulse therapy and rituximab treatment. At the 3-month follow-up, the patient′s condition was stable with mild anemia and normal platelet count. Patients with TMA secondary to SLE are heterogenous, while normal ADAMT 13 activity indicates poor prognosis. Early and aggressive treatment is important for disease control, and plasma exchange is helpful as a supportive care.

A 31-year-old woman was admitted to Peking Union Medical College Hospital presented with intermittent vomiting and abdominal pain for 2 years, and recurrence with paroxysmal dizziness for 1 month. This patient was diagnosed with systemic lupus erythematosus (SLE) 2 years ago with involvement of gastrointestinal and urinary tracts. One month ago, repeated vomiting and nausea recurred. No laboratory and imaging abnormalities were found in central nervous system and gastrointestinal evaluation. Orthostatic hypotension and fluctuation of blood pressure were recorded during hospitalization. Combined with sexual dysfunction, left adie pupil, anhidrosis and abnormal sympathetic skin response, autonomic nerve dysfunction related to SLE was diagnosed. After treated with pulse glucocorticoids and intravenous immunoglobulin, the patient′s symptoms improved remarkably. Orthostatic hypotension in SLE patients may link to autonomic nerve dysfunction.

Objective: To describe the status of non-steroidal anti-inflammatory drugs (NSAIDs) use in areas with a high incidence of upper gastrointestinal cancer in China. Methods: This study was based on the National Key Research and Development Program of "National Precision Medicine Cohort of Esophageal Cancer" and "Study on Identification and Prevention of High-risk Populations of Gastrointestinal Malignancies (Esophageal cancer, Gastric cancer and Colorectal cancer)" . From January 2017 to August 2018, 212 villages or communities with a high incidence of esophageal cancer or gastric cancer were selected from 12 regions in 6 provinces. A total of 35 910 residents aged between 40 and 69 years old who met the inclusion criteria and signed the informed consent were investigated and enrolled in this study. The use of NSAIDs, demographic characteristics, health-related habits, height, weight, and blood pressure were collected by the questionnaire and physical examination. The status of main NSAIDs (aspirin, acetaminophen and ibuprofen) use with the difference varying in genders, age groups and regions were analyzed by using χ² test and Cochran-Armitage trend analysis method. Results: Of 35 910 subjects, the mean age was (54.6±7.1) years old and males accounted for 43.42% (15 591). The overall prevalence of NSAIDs intake was 4.56% (1 638), but it significantly varied in different provinces (P<0.001). The overall prevalence of NSAIDs intake was 4.87% (1 750) in females, which was significantly higher than that in males 4.24% (1 524) (P<0.001). The prevalence of NSAIDs intake increased with age (P for trend <0.001). As the frequency of NSAIDs intake increased, the incidence of gastrointestinal symptoms, gastrointestinal ulcers and black stools increased (P for trend <0.05 for all). Conclusion The use of NSAIDs is prevalent in some areas with a high incidence of upper gastrointestinal cancer in China. The increased use of NSAIDs may lead to more adverse effects related to the gastrointestinal tract.