OBJECTIVE: To investigate the distribution and clinical significance of antiphospholipid antibody (aPL) in patients with Behcet disease (BD). METHODS: A total of 222 BD patients admitted to the Department of Rheumatology and Immunology in Peking University People' s Hospital from February 2008 to July 2024 were selected retrospectively. General data of the patients including age and gender were collec-ted. Clinical manifestations (including oral ulcers, genital ulcers, and thrombosis) and laboratory indexes (including aPL, human leukocyte antigen-B51, and anti-endothelial cell antibody) were collec-ted. The recurrence of thrombosis in the BD patients with thrombosis was followed up. Chi-square test was used to compare the clinical symptoms and laboratory indicators between aPL positive group and aPL negative group. Log-rank test was used to compare the recurrence rates of the aPL positive group and the aPL negative group, and P correction was performed by Two-stage method. Finally, Graphpad prism was used for plotting. RESULTS: The prevalence of single aPL, double aPL and triple aPL positivity in the BD patients were 22.1%, 0.5% and 1.4%, respectively. The positive rates of anti-cardiolipin antibody, anti-β2 glycoprotein Ⅰ antibody and lupus anticoagulant (LAC) were 10.4%, 1.8% and 13.1%, respectively. The incidence of thrombosis in the aPL positive group was significantly higher than that in the aPL negative group (44.9% vs. 16.9%, P < 0.001). The erythrocyte sedimentation rate [(20.78±4.91) mm/h vs. (15.85±4.29) mm/h, P=0.005], C-reactive protein [(12.97±5.17) mg/L vs. (7.49± 4.22) mg/L, P=0.010] and IgM [(1.55±0.95) g/L vs. (1.12±0.72) g/L, P < 0.001] in the aPL positive group were significantly higher than those in the aPL negative group. LAC positivity was an independent risk factor for thrombosis in the BD patients (OR=8.51, 95%CI: 2.71-26.72, P < 0.001). The recurrence rate of the aPL positive group was higher than that of the aPL negative group, but there was no statistical difference (69.23% vs. 52.17%, P=0.932). CONCLUSION Positive LAC and aneurysm are independent risk factors for thrombosis in BD patients. At the same time, positive antiphospholipid antibody can also significantly increase the risk of thrombosis in BD patients, which has important significance for guiding the treatment of BD.
Humans ; Behcet Syndrome/blood* ; Antibodies, Antiphospholipid/blood* ; Thrombosis/immunology* ; Male ; Female ; Retrospective Studies ; Adult ; Recurrence ; Antibodies, Anticardiolipin/blood* ; Clinical Relevance

OBJECTIVE: To investigate whether anti-phosphatidylserine/prothrombin antibodies and its IgG or IgM subtypes were correlated with unexplained recurrent miscarriages. METHODS: In our a single-center retrospective study, 283 patients with at least one unexplained miscarriage who visited the Third Hospital of Peking University between January 2021 and August 2023, aged between 18-40 years, and tested for anti-phosphatidylserine/prothrombin antibodies IgG or IgM subtypes, were included. The patients with either positive IgG or IgM anti-phosphatidylserine/prothrombin antibody were regarded as positive for anti-phosphatidylserine/prothrombin antibody. SPSS 26.0 software was used for statistical analysis. Chi-square test and Logistic regression analysis were used to study the correlation of anti-phosphatidylserine/prothrombin antibodies and its IgG or IgM subtypes with unexplained recurrent miscarriages. And the diagnostic sensitivity, specificity, the positive predictive value, the negative predictive value of anti-phosphatidylserine/prothrombin antibodies and its IgG or IgM subtypes in unexplained miscarriages was calculated with four-fold table. RESULTS: Chi-square analysis showed that anti-phosphatidylserine/prothrombin antibodies and its IgM subtypes were correlated with recurrent miscarriages (both P < 0.05), while the IgG subtype was not correlated with recurrent miscarriages (P>0.05). After adjusting with anticardiolipin antibodies, anti-β₂ glycoprotein antibodies, lupus anticoagulants, antinuclear antibodies, and age by Logistic regression analysis, anti-phosphatidylserine/prothrombin antibodies were correlated with unexplained recurrent miscarriages (OR=2.084, 95%CI 1.045-4.155, P < 0.05), and anti-phosphatidylserine/prothrombin antibody IgM subtypes were correlated with unexplained recurrent miscarriages (OR=2.368, 95%CI 1.187-4.722, P < 0.05).The sensitivity of anti-phosphatidylserine/prothrombin antibody in recurrent miscarriage was 65.43%, the specificity was 48.51%, the positive predictive value was 33.76%, and the negative predictive value was 77.78%. In the patients with recurrent miscarriages with negative classical antiphospholipid antibodies, the sensitivity of anti-phosphatidylserine/prothrombin antibody was 59.09%, the specificity was 63.23%, the positive predictive value was 40.63%, and the negative predictive value was 78.40%. The sensitivity of the anti-phosphatidylserine/prothrombin antibody IgM subtype for the diagnosis of recurrent miscarriage was 65.43%, the specificity was 50.99%, the positive predictive value was 34.87%, and the negative predictive value was 78.63%. CONCLUSION Anti-phosphatidylserine/prothrombin antibody and IgM subtype antibody are correlated with unexplained recurrent miscarriages in patients with at least one unexplained miscarriage. Whether positive anti-phosphatidylserine/prothrombin antibody or IgM subtype could predict future unexplained recurrent miscarriages warrants a prospective study.
Pregnancy ; Female ; Humans ; Adolescent ; Young Adult ; Adult ; Prothrombin ; Retrospective Studies ; Phosphatidylserines ; Prospective Studies ; beta 2-Glycoprotein I ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome/diagnosis* ; Antibodies, Anticardiolipin ; Abortion, Habitual ; Immunoglobulin G ; Immunoglobulin M

To evaluate the data obtained from the external quality assurance program initiated by Chinese Rheumatism Data Center (CRDC-QAP) for autoantibodies detection in 2021, so as to assess the consensus and differences in cross-laboratory testing to autoantibodies in China. This is a retrospective study. After collecting data from the first half year (from May 15th to July 10th) and the second half year (from August 15th to November 19th) of CRDC-QAP program for autoantibody detection in 2021, it firstly analyzed the qualitative consensus of the cross-laboratory results. Secondly, it compared the positivity grade of numeric results according to the Sample to cut-off ratio (S/CO ratio) calculation. Finally, the mean and coefficient variation (CV) of numeric results from three major manufacturers were calculated. A total of 303 and 332 clinical labs voluntarily participated in the first half year and the second half year of CRDC-QAP program for autoantibody detection in 2021, respectively. Except for anti-β2 glycoprotein type I (aβ2-GPI) IgM, the cross-laboratory consensus of qualitative results for the other autoantibodies is greater than 96%. As for anti-cyclic citrullinated peptide antibody (anti-CCP) and anti mitochondrial antibody-M2 (AMA-M2), the numeric results from more than 90% laboratories showed the same positivity grade. More than 50% of laboratories used chemiluminescence immunoassay (CLIA) for quantitative evaluation of autoantibody. The CV of numeric results from different manufacturers showed certain differences(P<0.01) with the range from 0 to 238%. Although high consensus can be observed in term of qualitative result for autoantibody detection in cross-laboratory, there are still certain differences in numeric results in term of positivity grade and manufacturer-based CV.
Humans ; Autoantibodies ; Antibodies, Anticardiolipin/analysis* ; Retrospective Studies ; East Asian People ; beta 2-Glycoprotein I ; Rheumatic Diseases

OBJECTIVE: To investigate the clinical and immunological characteristics of systemic lupus erythematosus (SLE) with retinopathy. METHODS: Fifty SLE patients with retinopathy without hypertension and diabetes (retinopathy group) who were hospitalized in the Peking University People's Hospital from January 2009 to July 2022 were screened. Fifty SLE patients without blurred vision during the course of the SLE and without retinopathy in the fundus examinations (non-retinopathy group) matched for sex and age were selected. Their clinical manifestations, laboratory tests and lymphocyte subsets were statistically analyzed. RESULTS: The most common fundus ocular change of the SLE patients with retinopathy was cotton-wool spots (33/50, 66.0%), followed by intraretinal hemorrhage (31/50, 62.0%). Retinopathy could occur at any stage of SLE duration, with a median of 1 year (20 days to 30 years). The proportion of lupus nephritis (72.0% vs. 46.0%, P=0.008) and serositis (58.0% vs. 28.0%, P=0.002) in the retinopathy group were significantly higher than those in the non-retinopathy group. The proportion of neuropsychiatric systemic lupus erythematosus (NPSLE) in the retinopathy group was higher, but there was no significant difference between the two groups. Compared with the non-retinopathy group, the proportion of positive anti-cardiolipin antibody (30.0% vs. 12.0%, P=0.027), the SLEDAI score (median 22.0 vs. 10.5, P < 0.001), erythrocyte sedimentation rate (P < 0.001), C-reactive protein (P=0.019) and twenty-four hours urine total protein level (P=0.026) in the retinopathy group were significantly higher, and the hemoglobin level was significantly lower [(91.64±25.18) g/L vs. (113.96±18.57) g/L, P < 0.001]. The proportion of CD19⁺ B cells in peripheral blood of the patients with SLE retinopathy was significantly increased (P=0.010), the proportion of CD4⁺ T cells was significantly decreased (P=0.025) and the proportion of natural killer (NK) cells was lower (P=0.051) when compared with the non-retinopathy group. CONCLUSION Retinopathy in SLE suggests a higher activity of SLE disease with more frequent hematologic and retinal involvement. It is recommended to perform fundus examination as soon as a patient is diagnosed with SLE. SLE patients with retinopathy may have stronger abnormal proliferation of B cells, and aggressive treatment should be applied to prevent other important organs involvement.
Humans ; Lupus Erythematosus, Systemic ; Lupus Vasculitis, Central Nervous System ; Lupus Nephritis ; Antibodies, Anticardiolipin ; Serositis

OBJECTIVE: To investigate the significance and distribution of oxidized low-density lipoprotein antibodies (ox-LDL-Ab) in patients with antiphospholipid syndrome (APS). METHODS: In this study, 334 patients who were hospitalized in the Department of Rheumatology and Immunology, Peking University People's Hospital were included. There were 162 APS patients, 122 patients with other autoimmune diseases without thrombosis or obstetric disease as disease control and 50 healthy controls. The clinical data and laboratory indicators were retrospectively collected. The ox-LDL-Ab, anticardiolipin (aCL) IgG/IgA/IgM, and anti-β2-glycoprotein Ⅰ (aβ2GPI) IgG/IgA/IgM were detected by enzyme-linked immunosorbent assay (ELISA). The relationship between ox-LDL-Ab and clinical and laboratory parameters were analyzed by SPSS 27.0. RESULTS: In APS group, 60.5% of patients had thrombosis, 48.1% had pregnancy morbidity, 34.0% had thrombocytopenia. The positive rates of aCL, aβ2GPI and lupus anticoagulant (LAC) were 17.9%, 34.6%, and 46.9%, respectively. The ox-LDL-Ab titers and positive rate in APS group were higher than that in healthy controls [titers: 40.8 (25.4-66.0) U/mL vs. 24.1 (12.3-36.5) U/mL, P=0.001; positive rate: 67.3% vs. 36.0%, P=0.001]. The diffe-rences in titers and positive rate of ox-LDL-Ab between APS patients and disease controls were not statistically significant [titers: 40.8 (25.4-66.0) U/mL vs. 35.9 (24.2-53.1) U/mL, P=0.118; positive rate: 67.3% vs. 61.5%, P=0.318]. The area under curve (AUC) for aβ2GPI, aCL, and ox-LDL-Ab were 0.745 (95%CI: 0.692-0.797), 0.666 (95%CI: 0.608-0.724), 0.609 (95%CI: 0.549-0.669), respectively. The Youden's index was 0.388, 0.269, and 0.132, respectively. The AUC for ox-LDL-Ab in seronegative APS patients was 0.562 (95%CI: 0.480-0.645). The sensitivity and specificity of ox-LDL-Ab in seronegative APS patients were 63.9% and 47.0%, respectively, and the Youden's index was 0.109. The ox-LDL-Ab positive group had higher positive rate of aβ2GPI (42.2% vs. 18.9%, P=0.003) and aCL (22.9% vs. 7.5%, P=0.017) than the ox-LDL-Ab negative group. There was no correlation between ox-LDL-Ab and thrombosis, coronary artery disease, pregnancy morbidity, hyperlipidemia, hypocomplementemia, and LAC positivity. CONCLUSION Ox-LDL-Ab was correlated with aCL and aβ2GPI, and no association were observed between ox-LDL-Ab and thrombosis, coronary artery disease, and pregnancy morbidity.
Pregnancy ; Female ; Humans ; Antiphospholipid Syndrome ; Antibodies, Anticardiolipin ; Retrospective Studies ; Coronary Artery Disease ; Clinical Relevance ; beta 2-Glycoprotein I ; Lupus Coagulation Inhibitor ; Lipoproteins, LDL ; Autoantibodies ; Immunoglobulin G ; Immunoglobulin A ; Thrombosis ; Immunoglobulin M

A 58-year-old female was referred to our department with intermittent suffocation for 1.5 years, aggravated for a month. 1.5 years before she developed oral ulcer, raynaud phenomenon, proteinuria, bilateral pleural effusion, ANA and anti-dsDNA positive. This patient was diagnosed with systemic lupus erythematosus (SLE). After given hormones, hydroxychloroquine sulfate (HCQ), her symptom relieved soon. The patient stopped her pills 1 year ago. One month ago, she had chest tightness, increased urine foam, and suffered from oliguria. Her admission medical examination: blood pressure (BP) 130/80 mmHg, conjunctiva pale, and lower lung breath sounds reduced. There was no tenderness, rebound pain and abdominal muscle tension in the abdomen. Liver and spleen rib inferior, mobile dullness negative, and lower extremity edema. Blood routine tests were performed with hemoglobin (HGB) 57 g/L. Urine routine: BLD (3+). 24-hour urinary protein 3.2 g. serum albumin 20.5 g/L, C-reactive protein (CRP) 12.85 mg/L, erythrocyte sedimentation rate (ESR) 140 mm/h. Antinuclear antibody (ANA) (H)1:10 000, anti-dsDNA antibody 1:3 200; anti-Smith antibody, anti-U1-snRNP/Sm antibody were positive, blood complement 3(C3) 0.43 g/L, complement 4(C4) 0.07 g /L. Anticardiolipin antibody (ACL), anti-β2-GP1, lupus anticoagulant (LA) were negative; HRCT suggested bilateral medial pleural cavity product liquid. Admission diagnosis: SLE lupus nephritis, anemia, pleural effusion, and hypoproteinemia. We treated her with methylprednisolone 1 000 mg×3 d, late to 48 mg/d and cyclophosphamide 1.0 g, HCQ 0.2 g bid, gamma globulin 10 g×5 d. Day 2 of treatment, this patient developed acute right upper quadrant pain, not accompanied by nausea, vomiting, blood stool and diarrhea. Antipyretic antispasmodic treatment was invalid, after the morning to ease their own abdominal pain. Day 4 of treatment, daytime blood HGB 77 g/L. Bilateral renal vascular ultrasound: bilateral renal artery blood flow velocity was reduced. The abdominal pain of the above symptoms recurred at night, BP was 120/80 mmHg, and no positive signs were found on abdominal examination. No abnormality was found in the vertical abdominal plain film. Blood routine examination: HGB 53 g/L, Plasma D dimer 2 515 μg/L, amylase in hematuria was normal, the stool occult blood was negative. Abdominal computed tomography (CT): normal structure of right adrenal gland disappeared, irregular mass shadow could be seen in adrenal region, CT value was about 50 HU. Morphological density of left adrenal gland was not abnormal. The retroperitoneum descended along the inferior vena cava to the right iliac blood vessel and showed a bolus shadow. The density of some segments increased. The lesion involved the right renal periphery and reached the left side of abdominal aorta. Most lesions surrounded the inferior vena cava, the right renal vein and part of the small intestine. The boundary between the upper lesion and the vena cava was unclear. Iodinecontaining contrast agent was taken orally. No sign of contrast agent overflowing was found in the abdominal cavity. Hematoma and exudative changes were considered in retroperitoneum. CONCLUSION of contrast-enhanced ultrasound of blood vessels: The retroperitoneal inferior vena cava (volume 3.5 cm×3.5 cm×1.5 cm) was hypoechoic and had no blood flow lesion. The adrenal gland had a high possibility of origin. Left renal vein thrombosis extended to inferior vena cava. According to the above data, it was analyzed that the cause of retroperitoneal hematoma of the patient was left adrenal vein thrombosis caused by hypercoagulable state, which led to vascular rupture and hemorrhage caused by increased vascular pressure in adrenal gland. Therefore, on the basis of continuing to actively treat the primary disease, and on the basis of dynamic observation of no active hemorrhage for 3 days, the anticoagulant therapy was continued with 10 mg/d of apixaban. Clinical symptoms were gradually eased, HGB did not decrease. Two weeks later, the ultrasonic examination showed that the irregular cluster hypoechoic range behind the inferior vena cava was significantly smaller than that before (1.8 cm×1.2 cm×0.7 cm). Abdominal CT examination after 1 month showed that there was no abnormal morphological density of bilateral adrenal glands and basic absorption of retroperitoneal exudation. Adrenal hemorrhage is uncommon. SLE with adrenal hemorrhage is rarer. In SLE patients, especially those complicated with APS, if abdominal pain accompanied by HGB decrease occurs, except after gastrointestinal hemorrhage, the possibility of adrenal hemorrhage should be warned.
Antibodies, Anticardiolipin ; Antiphospholipid Syndrome ; Aorta, Abdominal ; Female ; Hemorrhage ; Humans ; Lupus Erythematosus, Systemic ; Middle Aged

OBJECTIVE: To investigate prevalence of antiphospholipid antibody (APA) in Korean infertile women undergoing the first in vitro fertilization (IVF) treatment and to evaluate the influence of APA on the subsequent IVF outcomes. METHOD: Two hundred nineteen infertile women who destined the first IVF were prospectively enrolled in 2 infertility centers. Male factor or uterine factor infertility and women with past or current endocrine or immunologic disorders were completely excluded. Plasma concentration of lupus anticoagulant was measured by clot-based method, and anticardiolipin antibody (IgG/IgM), and anti-β2-glycoprotein 1 antibody (IgG/IgM) was measured by enzyme-linked immunosorbent assay method before starting ovarian stimulation for IVF. RESULTS: APA was positive in 13 women (5.9%). Lupus anticoagulant was positive in 2 women (0.9%), anticardiolipin antibody was positive in 7 women (3.2%), and anti-β2-glycoprotein 1 antibody was positive in 4 women (1.8%). In 193 women entering embryo transfer, clinical characteristics and stimulation outcomes were comparable between APA-positive (n=12) and APA-negative group (n=181). The clinical pregnancy rate (66.7% vs. 45.9%), ongoing pregnancy rate (58.3% vs. 37.0%), and miscarriage rate (12.5% vs. 19.3%) were all similar between APA-positive and APA-negative group. CONCLUSION: The prevalence of APA is low in Korean infertile women undergoing the first IVF cycle, and the presence of APA appears to neither decrease their first IVF success nor increase abortion rate.
Abortion, Induced ; Abortion, Spontaneous ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid* ; Embryo Transfer ; Enzyme-Linked Immunosorbent Assay ; Female ; Fertilization in Vitro* ; Humans ; In Vitro Techniques* ; Infertility ; Lupus Coagulation Inhibitor ; Male ; Methods ; Ovulation Induction ; Plasma ; Pregnancy ; Pregnancy Rate ; Prevalence ; Prospective Studies

OBJECTIVE: To detect the serum levels of soluble endothelial glycoprotein endoglin (s-Eng) in patients with antiphospholipid syndrome (APS) and to evaluate the correlation between s-Eng levels and clinical features and laboratory parameters. METHODS: The levels of serum s-Eng were measured by enzyme linked immunosorbent assay (ELISA) in 139 patients with APS, 44 patients with SLE but no APS, 37 patients with primary Sjögren's syndrome (pSS), 23 patients with Bechet's disease (BD), 22 patients with systemic sclerosis (SSc) and 22 persistent anticardiolipin antibody (aCL) positive individuals without SLE or APS (simply aCL positive group) and 87 health controls (HC) without any auto-immune diseases. These APS patients included 64 primary APS patients and 75 APS patients secondary to SLE.The correlation between the clinical data, laboratory parameters, and serum s-Eng levels were analyzed.Independent samples t test, paired t test, Chi-square Test, Mann-Whitney U test, Pearson's χ² test were used for statistical analyses. RESULTS: (1) The serum levels of s-Eng were significantly higher in the patients with APS whether primary or secondary to SLE than in the health controls and simply aCL positive group and the patients with other autoimmune diseases, including SLE, pSS, BD and SSc (P<0.001). There was no significant difference in the serum s-Eng levels between simply aCL positive group and health controls [(5.17±2.00) mg/L vs. (5.04±1.11) mg/L, P>0.05]. (2) The best cut-off value for the diagnosis of APS was no less than 8.37 mg/L as mean ± 3SD value, with the sensitivity at 0.772 and the specificity at 0.928. The Youden index was 0.700. These results indicated good validity of s-Eng as a diagnostic marker for APS. (3) The proportions of artery thrombosis and pathological pregnancy were higher in the group of s-Eng-positive APS patients than that in s-Eng-negative group (46/81 vs. 19/58, 29/65 vs. 10/44, respectively, all P<0.05). The levels of PLT were lower in the group of s-Eng-positive APS patients (72.00×10⁹/L vs. 119.00×10⁹/L, P<0.001). (4) The proportions of the presence (93.83% vs. 37.93%, P<0.001) and titer (61.70 U/mL vs. 15.45 U/mL, P<0.001) of aCL were both higher in the group of s-Eng-positive APS patients than in s-Eng-negative group. The proportions of the presence (61.73% vs. 43.10%, P<0.05) and titer (33.48 U/mL vs.17.40 U/mL, P<0.05) of anti-β2-glycoprotein I antibody were both higher in the group of s-Eng-positive APS patients than in s-Eng-negative group too. CONCLUSION s-Eng serum levels were significantly increased in the patients with APS, and it may play a role as acomplementary serological marker for the diagnosis and risk prediction of APS.
Antibodies, Anticardiolipin ; Antiphospholipid Syndrome/diagnosis* ; Autoantibodies ; Endoglin/blood* ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Pregnancy

A 73-year-old female with diabetes admitted for treatment of an intertrochanter fracture of the femur and a urinary tract infection (UTI) with Escherichia coli developed thrombosis in her right azygos vein, which was thought to be associated with antiphospholipid and immunoglobulin M anticardiolipin antibodies. After antibiotic therapy, antiphospholipid antibody was undetectable, and a repeat chest computed tomography showed complete resolution of the azygos vein thrombosis. A wide variety of infections can be associated with thrombotic events in patients with transient antiphospholipid syndrome (APS), and this case serves as a reminder that the possibility of transient APS should be considered in patients with venous thrombosis in the setting of a UTI.
Aged ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Azygos Vein* ; Escherichia coli* ; Escherichia* ; Female ; Femur ; Humans ; Immunoglobulin M ; Thorax ; Thrombosis ; Urinary Tract Infections* ; Urinary Tract* ; Venous Thrombosis

The incidence of intracerebral hemorrhage in those aged 45–84 years is 0.3-0.5%. In people over 80 years of age, this incidence increases 25-fold compared with that of the total population. The most common causes of spontaneous intracerebral hemorrhage in the younger population are vascular malformation, aneurysm, and overuse of drugs. In contrast, common causes in the elderly include hypertension, tumors, and coagulation disorders. Here, we present a case involving a 72-year-old male patient who, without any of these predisposing conditions, was admitted to the hospital with spontaneous intracerebral hemorrhage and showed signs of multifocal intracerebral hemorrhage during his stay. We conclude that spontaneous intracerebral hemorrhage can occur without any predisposing factors, and can lead to a patient's death. Therefore, the possibility of recurrent spontaneous intracerebral hemorrhage must be considered in patients with primary spontaneous intracerebral hemorrhage.
Aged ; Aneurysm ; Antibodies, Anticardiolipin ; Autoimmune Diseases ; Causality ; Cerebral Hemorrhage ; Hematoma* ; Humans ; Hypertension ; Incidence ; Male ; Risk Factors* ; Vascular Malformations