Systemic autoimmune diseases represent a group of disorders characterized by loss of immune tolerance to self-antigens, leading to abnormal immune responses and subsequent tissue damage. Typical examples include systemic lupus erythematosus and systemic sclerosis. These conditions are marked by multi-system involvement, chronic progression, and recurrent flares. The pancreas, as a vital digestive and endocrine organ rich in glandular tissue and vascular supply, can also be affected by autoimmune processes. Pancreatic injury often indicates active and difficult-to-control disease, posing a serious threat to patient survival. Due to its relative rarity, diverse underlying mechanisms across different autoimmune diseases, and frequently nonspecific clinical presentations, pancreatic involvement is easily overlooked, resulting in delayed diagnosis and treatment.This article focuses on the clinical features and potential pathophysiological mechanisms of pancreatic injury associated with autoimmune diseases, such as systemic lupus erythematosus, systemic sclerosis, Sjögren's syndrome, and rheumatoid arthritis, aiming to enhance clinical awareness and facilitate early recognition and diagnosis of this condition.

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 the clinical diagnostic ability of antiphospholipid syndrome (APS) with onset of autoimmune hemolytic anemia (AIHA).Methods:The diagnosis and treatment of one APS patient with AIHA as the initial manifestation were reported and discussed.Results:A young female patient admitted to Peking Union Medical College Hospital on October 15, 2022 suffered from AIHA and persistent lupus anticoagulant (LA) positivity. After being treated with high-dose glucocorticoid, both symptoms and indicators were improved. However, relapses occurred when the glucocorticoid was tapered. Subsequent attempts to combine multiple immunosuppressants and biologics for treatment was ineffective. During the course of the disease, the patient had experienced intermittent intracranial hypertension which was revealed as cerebral venous sinus thrombosis(CVST) by MRV. Laboratory test revealed that antiphospholipid antibodies and antiphospholipid serine/prothrombin antibodies (aPS/PT) were all positive. She was finally diagnosed with APS. After being treated with high-dose glucocorticoids and immunosuppressants, combined with warfarin and aspirin, the patient′s clinical symptoms were significantly improved.Conclusion:AIHA is one of the extra-criteria manifestations of APS. Patients with AIHA and persistent antiphospholipid antibody profiles should be alerted to the possibility of thrombotic events. It is difficult to control APS-CVST-AIHA patients′disease development and recurrence. Early and adequate antithrombotic therapy is essential for improvement of prognosis. Furthermore, some drugs may lead to false positive in LA testing, making aPS/PT a viable alternative method for assessing LA.

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 improve the clinical diagnostic ability of antiphospholipid syndrome (APS) with onset of autoimmune hemolytic anemia (AIHA).Methods:The diagnosis and treatment of one APS patient with AIHA as the initial manifestation were reported and discussed.Results:A young female patient admitted to Peking Union Medical College Hospital on October 15, 2022 suffered from AIHA and persistent lupus anticoagulant (LA) positivity. After being treated with high-dose glucocorticoid, both symptoms and indicators were improved. However, relapses occurred when the glucocorticoid was tapered. Subsequent attempts to combine multiple immunosuppressants and biologics for treatment was ineffective. During the course of the disease, the patient had experienced intermittent intracranial hypertension which was revealed as cerebral venous sinus thrombosis(CVST) by MRV. Laboratory test revealed that antiphospholipid antibodies and antiphospholipid serine/prothrombin antibodies (aPS/PT) were all positive. She was finally diagnosed with APS. After being treated with high-dose glucocorticoids and immunosuppressants, combined with warfarin and aspirin, the patient′s clinical symptoms were significantly improved.Conclusion:AIHA is one of the extra-criteria manifestations of APS. Patients with AIHA and persistent antiphospholipid antibody profiles should be alerted to the possibility of thrombotic events. It is difficult to control APS-CVST-AIHA patients′disease development and recurrence. Early and adequate antithrombotic therapy is essential for improvement of prognosis. Furthermore, some drugs may lead to false positive in LA testing, making aPS/PT a viable alternative method for assessing LA.

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.

Objectives:Analyze the clinical characteristics of patients with primary antiphospholipid syndrome (PAPS) progressing to systemic lupus erythematosus (SLE).Explore the risk factors for the progression from PAPS to SLE.Methods:The clinical data of 262 patients with PAPS enrolled in Peking Union Medical College Hospital from February 2005 to September 2021 were evaluated. Assessments included demographic data, clinical manifestations, laboratory tests (serum levels of complement, anti-nuclear antibodies, anti-double-stranded DNA antibodies), treatment, and outcomes. Kaplan-Meier analysis was used to calculate the prevalence of SLE in patients with PAPS. Univariate Cox regression analysis was employed to identify the risk factors for PAPS progressing to SLE.Results:Among 262 patients with PAPS, 249 had PAPS (PAPS group) and 13 progressed to SLE (5.0%) (PAPS-SLE group). Univariate Cox regression analysis indicated that cardiac valve disease ( HR=6.360), positive anti-double-stranded DNA antibodies ( HR=7.203), low level of complement C3 ( HR=25.715), and low level of complement C4 ( HR=10.466) were risk factors for the progression of PAPS to SLE, whereas arterial thrombotic events ( HR=0.109) were protective factors ( P<0.05 for all). Kaplan-Meier analysis showed that the prevalence of SLE in patients suffering from PAPS with a disease course>10 years was 9%-15%. Hydroxychloroquine treatment had no effect on the occurrence of SLE in patients with PAPS ( HR=0.753, 95% CI 0.231-2.450, P=0.638). Patients with≥2 risk factors had a significantly higher prevalence of SLE compared with those with no or one risk factor (13-year cumulative prevalence of SLE 48.7% vs. 0 vs. 6.2%, P<0.001 for both). Conclusions:PAPS may progress to SLE in some patients. Early onset, cardiac-valve disease, positive anti-dsDNA antibody, and low levels of complement are risk factors for the progression of PAPS to SLE (especially in patients with≥2 risk factors). Whether application of hydroxychloroquine can delay this transition has yet to be demonstrated.