Prevalence of beta 2-glycoprotein I antibody in systemic lupus erythematosus patients with beta 2-glycoprotein I dependent antiphospholipid antibodies.

OBJECTIVE--To investigate whether beta 2-glycoprotein I antibody (aGPI) exists in sera that are positive for antiphospholipid antibodies (aPL). METHODS--Thirty six patients with systemic lupus erythematosus (SLE) and 20 healthy volunteers were recruited. GPI independent and dependent aPL (anticardiolipin (aCL), antiphosphatidylserine, antiphosphatidylinositol, antiphosphatidic acid) were measured using a conventional enzyme linked immunosorbent assay (ELISA). aGPI was measured by an ELISA using a gamma irradiated plate coated with purified GPI. RESULTS--Twenty of 36 SLE patients were positive for at least one of the GPI-dependent aPL. Five of these 20 were also positive for aGPI. Another five of the 20 showed low GPI dependency aCL antibodies; however, three of these patients required GPI for binding to phosphatidylserine, phosphatidylinositol, or both. CONCLUSION--We confirmed the existence of aGPI in patients with true GPI dependent aPL. From these results, we recommend the use of a gamma irradiated plate with GPI as antigen for the detection of true GPI dependent aPL or aGPI.     

Anti-beta2-glycoprotein I antibodies in pediatric systemic lupus erythematosus and antiphospholipid syndrome

OBJECTIVE: To determine whether serum beta2-glycoprotein I antibody (anti-beta2GPI) detection improves identification of pediatric subjects at risk for antiphospholipid syndrome (APS). METHODS: Serum antiphospholipid antibodies (aPL) were identified by anticardiolipin enzyme-linked immunosorbent assay (ELISA), lupus anticoagulant assays, and syphilis screening in children with primary APS, systemic lupus erythematosus (SLE), or SLE plus APS. Anti-beta2GPI level and isotype were determined by beta2GPI ELISA and correlated with clinical manifestations and other aPL assays. RESULTS: One hundred-ten subjects under 22 years of age and of mixed ethnicity were evaluated. Fifty-seven had SLE (including 14 with APS), 25 had primary APS, 16 had SLE-like APS, 6 were healthy children with aPL detected incidentally, 4 had other rheumatic diseases and 2 had other conditions. Anti-beta2GPI were detected in 48% of SLE subjects and did not improve aPL detection over standard tests. Anti-beta2GPI were associated with stroke (P = 0.014), but not with other APS manifestations, and were rarely detected in primary APS. Among subjects with APS manifesting as chronic thrombocytopenia, anti-beta(2)GPI distinguished subjects with SLE from those with primary APS. CONCLUSIONS: With the exception of stroke, anti-beta2GPI detection does not improve identification of pediatric APS over that of traditional aPL assays. Anti-beta2GPI are rare in pediatric primary APS, but may predict evolution of chronic thrombocytopenia to SLE.     

Immune responses to native beta(2)-glycoprotein I in patients with systemic lupus erythematosus and the antiphospholipid syndrome

OBJECTIVE: To identify HLA class II associations with anti beta(2)-glycoprotein I (beta2GPI) antibodies in a cohort of Caucasian patients with systemic lupus erythematosus (SLE) and to determine whether these HLA genotypes act as restriction elements for lymphocyte proliferation to native human beta2GPI in vitro. METHODS: Anti-beta2GPI antibodies were detected in patient sera using enzyme-linked immunosorbent assays (ELISAs). HLA class II alleles (DRB1, DQB1) were determined by polymerase chain reaction-based DNA genotyping. In vitro peripheral blood mononuclear cell (PBMC) responses to native human beta2GPI were measured in a 7-day proliferation assay. RESULTS: We identified three groups of Caucasian SLE patients using these ELISAs. In group 1, 16 out of 18 SLE patients (89%) with anti-beta2GPI antibodies were positive for HLA-DRB1*0401/4/8, DR11 or DRB1*1302 (P=0.001 vs controls) compared with 23 out of 53 patients (43%) in group 2 with anti-cardiolipin antibodies only, 57 out of 151 patients (38%) in group 3 (SLE patients without anticardiolipin antibodies) and 109 out of 225 controls (48%). Fourteen patients with anti-beta2GPI antibodies had greater median stimulation indices to beta2GPI in vitro compared with the 15 controls studied (P=0.04). CONCLUSION: The HLA class II and PBMC proliferation data suggest that beta2GPI may be both a T- and B-cell autoantigen in SLE.     

Aortoarteritis with systemic lupus erythematosus and secondary antiphospholipid antibody syndrome: a rare association

A 41-year-old woman diagnosed with aortoarteritis since 1988 was admitted with unstable angina. She also had anemia, thrombocytopenia, aortic regurgitation and pulmonary artery hypertension. She gave a history of recurrent fetal loss and myocardial infarction, following which angioplasty to the left anterior descending artery had been done. After investigation, a diagnosis of aortoarteritis with systemic lupus erythematosus and associated antiphospholipid antibody syndrome was made. Aortoarteritis may coexist with systemic lupus erythematosus and associated antiphospholipid antibody syndrome.     

Isotypes of anti-beta2-glycoprotein I antibodies: association with thrombosis in patients with systemic lupus erythematosus

OBJECTIVE: To evaluate the association between isotypes of anti-beta2-glycoprotein I antibodies (anti-beta2-GPI) and thrombosis and to identify antiphospholipid antibodies (aPL) that are most associated with thrombosis in patients with systemic lupus erythematosus (SLE). METHODS: IgG anticardiolipin antibody (aCL) and isotypes of anti-beta2-GPI were measured by ELISA, and clinical evidence of thrombosis was analyzed in 270 patients with SLE. RESULTS: IgG, IgM, and IgA anti-beta2-GPI were positive in 38.1, 13.7, and 34.8% of patients, respectively. Patients with a history of thrombosis were significantly more likely to have lupus anticoagulant (LAC), IgG aCL, and the 3 anti-beta2-GPI isotypes. Arterial thrombosis was associated with the presence of IgG aCL and the 3 anti-beta2-GPI isotypes, whereas venous thrombosis was associated with LAC, IgG aCL, and IgA anti-beta2-GPI. In stepwise multivariate logistic regression analysis, the variable that was associated with thrombosis was IgA anti-beta2-GPI. The occurrence of arterial thrombosis was associated with IgG aCL and that of venous thrombosis was related to IgA anti-beta2-GPI in stepwise multivariate analysis. The IgG, IgM, and IgA anti-beta2-GPI titers were closely correlated with IgG aCL titers. The IgA anti-beta2-GPI titers were also significantly correlated with those of IgG and IgM anti-beta2-GPI. CONCLUSION: The results suggest that anti-beta2-GPI isotypes are related to the occurrence of thrombosis, and measurements of IgA anti-beta2-GPI may be useful for predicting thrombotic episodes in patients with SLE.     

Heterogeneity of antibodies to beta2-glycoprotein 1 from patients with systemic lupus erythematosus

We studied antibodies to beta2-glycoprotein 1 (anti-beta2GP1) from 72 patients with systemic lupus erythematosus (SLE) with or without antiphospholipid syndrome (APS) or with or without anticardiolipin antibodies (aCL). Fifteen patients had APS and positive antiphospholipid antibodies [clinical APS(+)/aPL(+)], 12 patients had APS, negative serum IgG and IgM aCL, antiphosphatidylethanolamine, anti-phosphatidylserine and no lupus anticoagulant [clinical APS(+)/ aPL(-)]. A third group included 16 patients without APS but high aCL levels [clinical APS(-)/ aPL(+)]. In a fourth group we studied 29 patients without clinical manifestations of APS or aCL [clinical APS(-)/aPL(-)]. One hundred anticardiolipin and VDRL-negative normal sera were studied as controls. IgG antibodies to cardiolipin proper in a bovine beta2GP-free system, to human beta2GP1 immobilized on cardiolipin or to human beta2GP1 alone were detected in all sera by ELISA using irradiated and nonirradiated plates from two manufacturers. Sera from APS(+)/aPL(+) patients showed IgG binding to CL, CL + beta2GP1 and beta2GP1 in irradiated and nonirradiated plates. APS(+)/ aPL(-) sera had more significant IgG binding to beta2GP1 than normal controls when studied in both irradiated or nonirradiated plates (P = 0.001). This binding was inhibited by solid-phase cardiolipin in a dose-dependent manner. Sera from the APS(-)/aPL(+) subgroup had comparable IgG activity in both the CL and CL + beta2GP1 assays, while no anti-beta2GP1 activity was detected in these sera. Sera from the clinical APS(-)/aPL(-) patients were negative in the three ELISA systems. Antibodies to human beta2GP1 from SLE patients recognize various epitopes. Those from APS(+)/ aPL(+) patients appear to react with an epitope boosted by cardiolipin in addition to another one present in the native protein. In contrast, anti-beta2GP1 from patients with APS(+)/aPL(-) are blocked by cardiolipin, suggesting that their epitope is the phospholipid-binding site.     

IgG anti-beta2-glycoprotein I antibodies in adult patients with systemic lupus erythematosus: prevalence and diagnostic value for the antiphospholipid syndrome

OBJECTIVE: To investigate the prevalence of serum anti-beta2-glycoprotein I (anti-beta2-GPI) antibodies and other antiphospholipid antibodies (aPL) in patients with systemic lupus erythematosus (SLE). To study their diagnostic value for the antiphospholipid syndrome (APS). METHODS: Anti-beta2-GPI and IgG anticardiolipin (aCL) were determined in sera from 102 consecutive patients with SLE using ELISA. Serum and plasma tests were also done for lupus anticoagulant (LAC), syphilis, and antibodies to dsDNA. Clinical and laboratory features of APS were observed. RESULTS: Prevalences were 23.5% for aCL and 18.6% for anti-beta2-GPI. Correlations between the presence of aCL and anti-beta2-GPI and between their titers were statistically significant (p<0.0001). No associations were found between anti-beta2-GPI and disease activity criteria (SLEDAI, ECLAM, dsDNA). Anti-beta2-GPI were significantly associated with LAC (p = 0.005), APS (p = 0.005), and a high aCL titer (aCL > 5 SD; p< or =0.001). LAC was the best diagnostic criterion for APS. CONCLUSION: These data suggest that determination of anti-beta2-GPI in addition to aCL and LAC is unlikely to improve the diagnosis of APS in patients with SLE.     

Antibodies to high-density lipoprotein and beta2-glycoprotein I are inversely correlated with paraoxonase activity in systemic lupus erythematosus and primary antiphospholipid syndrome

OBJECTIVE: To determine the prevalence of anti-high-density lipoprotein (anti-HDL) antibodies and to establish a possible relationship between anti-HDL, anticardiolipin antibodies (aCL), anti-beta(2)-glycoprotein I (anti-beta(2)GPI), and paraoxonase (PON) activity in patients with systemic lupus erythematosus (SLE) and primary antiphospholipid syndrome (APS). METHODS: Thirty-two patients with SLE and 36 with primary APS were enrolled in a cross-sectional study. Twenty age- and sex-matched healthy subjects were used as controls. Serum levels of IgG and IgM aCL, anti-beta(2)GPI, and antiprothrombin antibodies and IgG anti-HDL were measured by enzyme-linked immunosorbent assay. Total cholesterol, HDL cholesterol, HDL(2), and HDL(3) were determined by standard enzymatic techniques. PON activity was assessed by quantification of nitrophenol formation, and total antioxidant capacity (TAC) by chemiluminescence. RESULTS: Levels of total HDL, HDL(2), and HDL(3) were reduced in patients with SLE compared with controls (mean +/- SD 0.51 +/- 0.3, 0.37 +/- 0.3, and 0.14 +/- 0.1 mmoles/liter, respectively, versus 1.42 +/- 0.9, 1.01 +/- 0.7, and 0.40 +/- 0.2). Patients with SLE and primary APS had higher titers of anti-HDL antibodies and lower PON activity than controls. In the SLE population, PON activity was inversely correlated with IgG anti-HDL titers (r = -0.48, P = 0.005) whereas in the primary APS population, IgG anti-beta(2)GPI was the only independent predictor of PON activity (r = -0.483, P = 0.003). In the SLE group, anti-HDL was inversely correlated with TAC (r = -0.40, P < 0.02), and PON activity was positively correlated with TAC (r = 0.43, P < 0.02). CONCLUSION: IgG anti-HDL and IgG anti-beta(2)GPI antibodies are associated with reduced PON activity in patients with SLE and primary APS. Since the physiologic role of PON is to prevent low-density lipoprotein oxidation with its attendant atherogenic effects, the reported interactions may be relevant to the development of atherosclerosis in SLE and primary APS.     

抗β2-糖蛋白Ⅰ抗体在系统性红斑狼疮中的临床意义

目的了解抗!2-糖蛋白Ⅰ抗体(抗!2-GPⅠ抗体)与系统性红斑狼疮(SLE)临床特点的关系及在其诊断中的价值。方法采用酶联免疫吸附试验(ELISA)检测112例SLE患者、40例类风湿关节炎(RA)患者、30例干燥综合征(SS)患者和40名正常人血清中的抗!2-GPⅠ抗体水平。同时,测定患者血清中的抗心磷脂抗体(ACL)等指标,并分析其与患者的临床特点(如:血栓、流产)的关系及其临床意义。统计学分析采用t检验、!2检验和Spearman检验。结果抗!2-GPⅠ抗体在SLE中敏感性为21.4%(24/112),特异性为88.6%,在RA和SS敏感性分别为15.0%(6/40)和6.7%(2/30),40名正常对照均为阴性。抗!2-GPⅠ抗体与血栓形成密切相关(P<0.01),与ACL-IgG和IgM型水平呈正相关(r=0.479,P=-0.032;r=0.400,P=0.045)。抗!2-GPⅠ抗体与其他临床及实验室指标无明显相关性。结论抗!2-GPⅠ抗体在SLE中具有一定的敏感性,且可能在SLE的血栓形成中发挥作用,联合检测ACL与抗!2-GPⅠ抗体能够辅助诊断SLE伴血栓形成。     

Chorea in systemic lupus erythematosus: association with antiphospholipid antibodies.

Chorea is a rare manifestation of systemic lupus erythematosus (SLE). In this report the clinical features of two cases of chorea associated with SLE are presented. Of special interest were the raised titres of antiphospholipid antibodies in both cases. The possible pathogenic role of these antibodies is briefly discussed.     

Atherogenic oxidized low-density lipoprotein/beta2-glycoprotein I (oxLDL/beta2GPI) complexes in patients with systemic lupus erythematosus and antiphospholipid syndrome

Oxidized low-density lipoprotein (oxLDL) interacts in vitro with beta2-glycoprotein I (beta2GPI) via LDL-derived specific ligands forming oxLDL/beta2GPI complexes. Circulating oxLDL/beta2GPI complexes have been demonstrated in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Autoimmune vascular inflammation and oxidative stress contribute to oxLDL/beta2GPI complex formation. Immunohistochemical staining of atherosclerotic lesions suggest that these complexes are formed in the arterial wall and released into circulation. The demonstration of antibodies to oxLDL/beta2GPI complexes indicates that these complexes are immunogenic, and the coexistence of complexes and antibodies suggest an active pro-thrombotic/pro-atherogenic role in the development of autoimmune vascular complications. Circulating oxLDL/beta2GPI complexes can be measured by ELISA using a monoclonal antibody specific to complexed human beta2GPI to capture beta2GPI bound to oxLDL. An enzyme-conjugated monoclonal antibody to human Apo B 100 allows the specific detection of oxLDL/beta2GPI complexes. OxLDL/beta2GPI complexes were common in SLE and APS patients suggesting an underlying process of inflammation and oxidation. Using oxLDL/beta2GPI complexes as capture antigen, antibodies to oxLDL/beta2GPI can be measured by ELISA. Serum levels of IgG anti-oxLDL/beta2GPI antibodies were significantly higher in SLE patients with APS compared to SLE controls without APS. Further, high titers of these IgG antibodies were observed in APS patients with a history of arterial thrombosis. The presence of circulating oxLDL/beta2GPI complexes and IgG antibodies to these complexes indicates significant vascular injury and oxidative stress as well as an active role in autoimmune-mediated atherothrombosis.     

Valine/Leucine247 polymorphism of beta2-glycoprotein I in patients with antiphospholipid syndrome: lack of association with anti-beta2-glycoprotein I antibodies

In antiphospholipid syndrome (APS) the presence of anti-beta2-glycoprotein I (beta2GPI) antibodies is strongly associated with thromboembolic complications. It has been suggested that the common beta2GPI Valine/Leucine247 (Val/Leu247) polymorphism could be found more commonly in APS and might influence the generation of anti-beta2GPI antibodies. Therefore we studied beta2GPI Val/Leu247 single-nucleotide polymorphism (SNP) by PCR in 338 patients with various autoimmune diseases (46 with secondary and 84 with primary APS) and 147 sex and age-matched healthy controls. In all patients lupus anticoagulant, anticardiolipin and anti-beta2GPI antibodies (both IgG and IgM) were also determined. All patients and controls were Caucasians. Frequencies of the SNP genotypes in patients did not depart from genetic equilibrum and did not differ from those found in controls. There was also no association between the presence of beta2GPI Val/Leu247genotypes and the presence or absence of lupus anticoagulant, anticardiolipin antibodies, anti-beta2GPI antibodies or clinical APS symptoms in all patients studied. In conclusion, among the exclusively Caucasian, Polish population of autoimmune patients beta2GPI Val/Leu247SNP has the same distribution as in healthy subjects and does not influence the production of anti-beta2GPI antibodies.     

Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies

OBJECTIVE: To apply the new American College of Rheumatology nomenclature for neuropsychiatric systemic lupus erythematosus (NPSLE), determine the prevalence of the different neuropsychiatric (NP) syndromes, and evaluate which of these manifestations correlates with the presence of antiphospholipid antibodies (aPL). Methods. Clinical, serological, and imaging data of 323 consecutive patients with SLE were retrospectively reviewed. Neuropsychometric testing was applied by a neuropsychologist. Univariate and multivariate statistical analyses were applied to evaluate the association bewteen NP manifestations, magnetic resonance imaging (MRI) abnormalities, and aPL. RESULTS: In total, 185 patients (57.3%) had NP manifestations at any time during followup. Headache was the most frequent manifestation, present in 78 patients (24%). Cerebrovascular disease (CVD) was diagnosed in 47/323 patients (14.5%), with a total of 57 events. Mood disorders were found in 54 (16.7%), cognitive disorders in 35 (10.8%), and seizures in 27 patients (8.3%). Psychosis was diagnosed in 25 (7.7%), anxiety disorder in 24 (3.7%), and acute confusional state in 12 patients (3.7%). Less common manifestations were polyneuropathy, mononeuritis, myasthenia gravis, cranial neuropathy, myelopathy, chorea, demyelinating disease, and Guillain-Barré syndrome. The presence of aPL was associated with NP manifestations (p < 0.001). Multivariate analysis showed that aPL were independently associated with CVD (OR 6.17, 95% CI 2.94-12.9, p = 0.001), headache (OR 2.04, 95% CI 1.17-3.55, p = 0.01), and seizures (OR 2.89, 95% CI 1.18-7.10, p = 0.02). The presence of lupus anticoagulant (LAC) was independently associated with white matter hyperintensity lesions on MRI (OR 3.0, 95% CI 1.12-8.05, p = 0.027). CONCLUSION: The new ACR criteria for NPSLE are useful to define NP manifestations in SLE with accuracy. NP manifestations are significantly associated with aPL. CVD, headache, and seizures were independently associated with these antibodies.     

Dual antibody reactivity to beta2-glycoprotein I and protein S: increased association with thrombotic events in the antiphospholipid syndrome

The antiphospholipid syndrome (APS) is a thrombotic disorder leading to spontaneous abortions, venous thromboses, myocardial infarctions and strokes. Although the syndrome is associated with characteristic autoantibodies, these tests have poor predictive value for thrombosis. The aim of the study was to determine whether the combined presence of two types of antiphospholipid antibodies can be associated with a high-risk subset of thrombosis-prone patients. One hundred and thirty-four sera from a lupus clinic were tested for antibodies to beta2-glycoprotein I (beta2GPI), protein S and prothrombin. In a group of 29 patients for whom plasma was available, free (functional) protein S levels were also measured. Autoantibodies to beta2GPI and protein S are associated with each other. Dual reactivity to beta2GPI and protein S correlates with increased history of thrombotic events (69% of doubly reactive patients) when compared to either type of autoantibody alone (37% of patients with only anti-beta2GPI and 38% with only anti-protein S, P=0.04 and P=0.01, respectively) or neither reactivity (37%). Among 29 patients tested for free (functional, anticoagulant) protein S levels, the lowest levels were found in patients with antibodies to beta2GPI and/or protein S, and all four patients with a history of thrombosis had below-normal free protein S levels. These associations were not found with antiprothrombin antibodies. In conclusion dual autoantibodies to beta2GPI and protein S are associated with increased history of thrombosis in the antiphospholipid syndrome.     

Stroke in patients with systemic lupus erythematosus and antiphospholipid antibody syndrome

Opinion statement  Primary prevention of strokes in patients with antiphospholipid antibodies (APLs) with or without systemic lupus erythematosus (SLE) is not well known. The same applies to patients with SLE and valvular heart disease. The decision should be made on an individual basis until further studies become available. Special consideration for preventive antithrombotic treatment should be given to patients with persistent high titers of immunoglobulin G APLs, which require protein cofactor for detection, presence of lupus anticoagulant, or left-sided cardiac valve lesions. High-level oral anticoagulation with warfarin is still the preferred treatment for secondary prevention of strokes in patients with antiphospholipid antibody syndrome (APS) with or without SLE or with cardiac valvular lesions. Immunosuppression should only be used in patients with active SLE disease. There is no evidence so far to support its use in patients with primary APS. Advances in identifying unique APL features that are associated with increased risk for thrombosis will hopefully allow a more rational treatment for primary and secondary prevention of strokes in these patients in the near future.     

Stroke in patients with systemic lupus erythematosus and antiphospholipid antibody syndrome

Opinion statement Primary prevention of strokes in patients with antiphospholipid antibodies (APLs) with or without systemic lupus erythematosus (SLE) is not well known. The same applies to patients with SLE and valvular heart disease. The decision should be made on an individual basis until further studies become available. Special consideration for preventive antithrombotic treatment should be given to patients with persistent high titers of immunoglobulin G APLs, which require protein cofactor for detection, presence of lupus anticoagulant, or left-sided cardiac valve lesions. High-level oral anticoagulation with warfarin is still the preferred treatment for secondary prevention of strokes in patients with antiphospholipid antibody syndrome (APS) with or without SLE or with cardiac valvular lesions. Immunosuppression should only be used in patients with active SLE disease. There is no evidence so far to support its use in patients with primary APS. Advances in identifying unique APL features that are associated with increased risk for thrombosis will hopefully allow a more rational treatment for primary and secondary prevention of strokes in these patients in the near future.     

Neuropsychiatric manifestations in pediatric systemic lupus erythematosus and association with antiphospholipid antibodies

OBJECTIVE: To determine the prevalence of neuropsychiatric (NP) manifestations in children with systemic lupus erythematosus (SLE) using the 1999 American College of Rheumatology case definitions for NP syndromes in SLE, and their association with antiphospholipid antibodies (aPL). METHODS: We performed a retrospective cohort study of 106 pediatric and adolescent SLE patients at 2 academic medical centers. Clinical and laboratory data were obtained by medical record review. All aPL testing was performed in standard clinical laboratories. RESULTS: Twenty-five patients (23.6%) had NP manifestations, including seizures (9.4%), headaches (4.7%), mood disorders (4.7%), cognitive dysfunction (4.7%), cerebrovascular accident (CVA), psychosis and pseudotumor (2.8% each), aseptic meningitis (0.9%), acute confusional state (0.9%), anxiety (0.9%), and cranial neuropathy (0.9%). NP events were not necessarily accompanied by an SLE flare. aPL were positive in 70% of all SLE patients, including anticardiolipin antibodies (aCL) in 64%, aCL IgG in 56%, aCL IgM in 35%, rapid plasma reagin or Venereal Disease Research Laboratory test in 13%, and lupus anticoagulant (LAC) in 18%. The only significant association between NP manifestations and aPL was for CVA and IgM aCL (p=0.03). LAC was slightly more common among patients with NP events, and the finding of LAC on more than one occasion was significantly associated with developing a NP event (p = 0.01). CONCLUSION: NP manifestations occur in about one-fourth of children with SLE, are an early event in the course of the disease, and are not necessarily accompanied by an SLE flare. Seizures are the most frequent symptom. Although aPL are common, their association with NP events, unlike in adults, is weak, except for CVA, suggesting a different pathogenic mechanism for NP manifestations in pediatric SLE.     

Pancreatitis related to antiphospholipid antibody syndrome in a patient with systemic lupus erythematosus.

A 39-year-old woman was admitted with abdominal pain and dyspnea, and a diagnosis of systemic lupus erythematosus with renal involvement was established. Laboratory tests revealed highly elevated anticardiolipin antibody, thrombocytopenia and false positive VDRL. Generalized thrombus formation and Libman-Sacks endocarditis were found at postmortem examination. The pancreas showed chronic inflammation with thrombi in pancreatic arteries, but no vasculitic change was observed. Lowering of pancreatic blood flow because of arterial thrombi was a possible cause of pancreatitis in this patient. The spectrum of antiphospholipid antibody associated diseases may be extended to include pancreatitis as a thrombotic complication.