scholarly journals POS0780 SKIN LESIONS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND ANTIPHOSPHOLIPID SYNDROME

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 643.2-644
Author(s):  
A. Shumilova ◽  
F. Cheldieva ◽  
T. Reshetnyak ◽  
A. Lila

Background:In more than 40% of cases, systemic lupus erythematosus (SLE) is associated with the presence of highly positive antiphospholipid antibodies, with 50-70% of patients developing antiphospholipid syndrome (APS) over the next 10 years of the disease. Both diseases have similar and different clinical manifestations of skin lesions. The variety of skin lesions in SLE and APS requires a differential diagnosis and can make it difficult to diagnose a systemic autoimmune disease in a timely manner.Objectives:To study the frequency of skin manifestations in SLE and APS, depending on the positivity of aPL.Methods:The study included 116 patients with SLE (104 women and 12 men), mean age 37.9±12.9, disease duration 8.5 [1.15-13.0]; 40 patients with APS (33 women and 7 men), mean age 36.2±9.39. All patients were evaluated for skin lesions, and patients with APS were determined by IgG/IgM-aCL and IgG/IgM-aß2HP1 by enzyme immunoassay (ELISA), 19 of them were determined by IgA-aCL, IgA-aß2HP1 and IgG-aß2HP1-D1 chemiluminescence analysis (CMA).Results:Acute skin lesions in past history were noted in 58 (50%) patients, chronic lesions -I n 21 (18.1%) patients; photosensivity and alopecia were indicated in 46 (39.6%) patients, mucosal lesions were noted in 36 (31%) of 116 patients, which corresponds to the literature data on the frequency of skin lesions and its appendages in SLE. At the time of inclusion in the study, skin lesions were detected in 20 patients. Score according to the CLASI index in patients with skin lesions: activity index=1.55 [0-22]; damage index=1.81 [0-36].Skin lesions are the second most common signs of SLE onset (debut in 26 (22.4%) patients), second only to arthritis (38 (32.5%) patients), while the detection of immunological disorders (highly positive ab to dsDNA) was observed in 7 patients (6%) with reliable APS and probable SLE, who may not have had time to develop a clinic for reliable SLE.Livedo, as one of the most frequent skin manifestations of APS, was detected in 60 patients and was significantly associated with IgM-aCL and IgM-aß2HP1 positivity (p<0.0001). Significantly, positivity for IgG-aCL, IgG-aß2HP1 and IgG-aß2HP1-D1 (p=0.0001) and IgA-aCL (p=0.008) was associated with the development of comminuted hemorrhages, which occurred in 7 patients with APS and was associated with positivity of IgG-aCL, IgG-aß2HP1, IgG-aß2HP1-D1 (p=0.0001) and IgA-aCL (p=0.008).The development of ulcerative-necrotic vasculitis with deep skin necrosis was observed in 3 patients, 2 of them were highly positive for IgG-aCL, IgG-aß2HP1, IgG-aß2HP1-D1. Melanoma was detected in the past history in 2 patients with highly positive for IgG-aCL, which is not a manifestation of the underlying disease, but confirms an increased risk of malignancy with aPL-positivity.Conclusion:More than half of the patients had acute skin lesions, and about a quarter of the cases had skin lesions at the onset of the disease. The presence of comminuted hemorrhages was associated with positivity of IgG-aCL, IgG-aß2HP1, IgG-aß2HP1-D1) and IgA-aCL. Assessment of skin activity and damage (in particular, according to the CLASI index) is necessary for a comprehensive analysis of the dynamics of the disease and the response to therapy. The detection of aPL is necessary not only for the purpose of predicting thrombotic catastrophes, but also for the development of skin manifestations of APS.Disclosure of Interests:None declared

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 605.2-606
Author(s):  
F. Cheldieva ◽  
T. Reshetnyak ◽  
M. Cherkasova ◽  
N. Seredavkina ◽  
A. Lila

Background:The study of antiphospholipid antibodies (aPL), not included in the Sydney diagnostic criteria, in antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) is poorly understood.Objectives:To determine the frequency of detection of IgA-aCL and IgA-aβ2GP1 and IgG antibodies to β2GP1 domain 1 (IgG-aβ2GP1-D1) in patients with APS with and without SLE.Methods:ELISA and chemiluminescence assays (CMA) were used to test 63 sera of patients: 22 (35%) with primary APS (pAPS) and 41 (65%) patients with APS and with SLE (secondary APS (sAPS)), with mean age 38,0 [33,0 – 43,0] years and disease duration 4,0 [0,1 – 9,9]. Both methods were used to test of IgG/IgM-aCL and IgG/IgM-aβ2GP1. CMA was used for research IgG/IgM/IgA-aCL, IgG/IgM/IgA-aβ2GPI and IgG-aβ2GP1-D1. Of them 49 (78%) (18 – with pAPS; 31 – with sAPS) displayed major thrombotic events and 18 of 22 pregnant women had pregnancy morbidity in past history. Lupus anticoagulant (LA) positivity was in 9 out of 12 patients who had it determined. LA was not investigated due to anticoagulant therapy in the remaining 52 patients.Results:IgG/IgM-aCL and IgG/IgM-aß2GP1 were recorded in 44/18 and 50/17 patients by ELISA and in 55/19 and 59/16 by CMA, respectively.IgA-aCL positivity was found in 35 (56%) of 63 patients. Thirty IgA-positive patients were positive for IgG-aCL by ELISA: 22 – IgG-aCL – highly positive, 6 – medium positive and 2 – low positive patients. IgM-aCL by ELISA was detected in 13 (37%) of 35 IgA-aCL positive patients: 11 – highly positive, 1 – medium positive and 1 – low positive. IgA-aCL was combined with IgG-aCL in 34 patients and with IgM-aCL in 16 patients in the CMA. IgG-aß2GP1 in ELISA was detected in 32 patients with IgA-aCL (24 –highly positive, 5 – medium positive and 3 – low positive) and in 34 – in CMA. IgM-aß2GP1 was combined with IgA-aß2GP1 with the same frequency in both methods (in 13 patients).IgA-aß2GP1 was detected in 30 (48%) of 63 patients. They were combined with both IgG-aCL and IgG-aß2GP1 in all cases in both methods. IgM-aCL and IgM-aß2GP1 were detected in 14 and 11 of 30 patients with IgA-aß2GP1, respectively. The combination of IgA-aß2GP1 with IgG-aCL by ELISA was in 27 (in most cases highly positive – 20) and with IgM-aCL – in 10 (highly positive - 8). IgG-aß2GP1 was detected in 28 patients with IgA-aß2GP1 (high positive – 21) and in 11 patients with IgM-aß2GP1 (high positive –7).IgG-aß2GP1-D1 was revealed in 48 (76%) patients. It was combined with IgG-aCL – in 38, with IgM-aCL – in 15 patients by the ELISA. The combination of IgG-aß2GP1-D1 by CMA was as follows: with IgG-aCL – in 46, with IgM-aCL – in 17, and with IgA-aCL – in 33 patients. In most cases, IgG-aß2gp1-D1 was combined with highly positive aCL levels. IgG-aß2GP1-D1 positivity was associated with IgG-aß2GP1 positivity in 42 – by ELISA and 47 – by CMA, IgМ-aβ2GP1 – in 13 and 14 patients by ELISA and CMA, respectively, and IgA-aß2GP1 – in 29. Isolated IgG-aß2GP1-D1 positivity was not observed.Conclusion:The frequency of IgA-aCL detection was 56% (35 patients out of 63), IgA-aβ2GP1 – 48% (30 patients out of 63), IgG-aβ2GP1-D1 – 76% (48 patients out of 63). There was not isolated positivity of this “extra” criterial antibodies. The presence of IgA-aCL, IgA-aβ2GP1, IgG-aβ2GP1-D1 was associated with highly positivity of IgG/IgM-aCL and IgG/IgM- aβ2GP1.Disclosure of Interests:None declared


2011 ◽  
Vol 38 (9) ◽  
pp. 1914-1919 ◽  
Author(s):  
LI-HSIN LIN ◽  
PIN LING ◽  
MING-FEI LIU

Objective.Type I interferons (IFN), especially IFN-α, have been proposed to underlie the pathogenesis of systemic lupus erythematosus (SLE). Members of the IFN regulatory factor (IRF) family, which regulate IFN expression, have been implicated as risk factors for SLE. Our aims were to investigate the expression of IRF7 and its correlation with disease activity and to explore the association in Taiwanese patients between 2 genetic single-nucleotide polymorphisms (SNP) of IRF7 and SLE.Methods.IRF7 messenger RNA (mRNA) levels were measured in peripheral blood mononuclear cells by real-time reverse transcription polymerase chain reaction in 51 adult patients with SLE and 65 age-matched and sex-matched controls. Their serum IFN-α levels were determined by ELISA and the clinical manifestations were recorded at the same time. Two IRF7 SNP, rs1061501 and rs1061502, were examined by genotyping across 92 patients with SLE and 92 age and sex-matched healthy control subjects.Results.Compared with controls, the expression of IRF7 mRNA was significantly increased in patients with SLE and was positively correlated with both the serum level of IFN-α and lupus disease activity. The distribution of SNP rs1061501 by genotype (CC, CT, and TT) and by allele (C, T) was significantly different between the SLE and the control group (p = 0.028 for genotype and p = 0.009 for allele). There were no significant differences for SNP rs1061502.Conclusion.The results suggest that dysregulation of IRF7 might mediate an excessive production of IFN-α, which then exerts a crucial effect on the pathogenesis of human SLE. The IRF7 SNP rs1061501 TT genotype and T allele are enriched in Taiwanese patients with SLE and thus would seem to be associated with an increased risk of developing SLE.


2009 ◽  
Vol 36 (1) ◽  
pp. 68-75 ◽  
Author(s):  
JUANITA ROMERO-DÍAZ ◽  
ICELLINI GARCÍA-SOSA ◽  
JORGE SÁNCHEZ-GUERRERO

ObjectiveTo determine the risk of thrombosis in patients with systemic lupus erythematosus (SLE) and other autoimmune diseases of recent onset.MethodsA retrospective cohort of 482 patients, mean age 28.3 years, with SLE or other autoimmune diseases was analyzed. Followup started at diagnosis or first appointment within 12 months since diagnosis until the development of thrombosis, end of study, loss to followup, or death. Thromboses were diagnosed upon clinical manifestations and confirmed by appropriate studies. Clinical variables were retrieved from the medical records, and SLE activity was assessed from the medical notes at onset of thrombosis, or at a dummy date for thrombosis, using the SLE Disease Activity Index-2K.ResultsDuring 2936 patient-years of followup, thromboses occurred in 49 patients (20.3%) with SLE and 6 patients (2.5%) with other autoimmune diseases. The incidence rate of thrombosis was 36.3 and 3.8 per 1000 patient-years in SLE and in other autoimmune diseases, respectively; relative risk 9.6 (95% CI 4.1–27.4, p < 0.0001). Throughout the disease course, the risk of thrombosis remained high in the SLE group, while in patients with other autoimmune diseases this risk was lower. The incidence of venous and arterial thrombosis was similar among SLE patients and patients with other autoimmune diseases. SLE and venous insufficiency were associated with thromboses in the total study population, and with venous insufficiency, vasculitis, and disease activity in the SLE group.ConclusionPatients with autoimmune diseases, particularly SLE, are at an increased risk of thrombosis. In patients with SLE, the risk remains elevated throughout the course of the disease.


2002 ◽  
Vol 55 (3-4) ◽  
pp. 89-96 ◽  
Author(s):  
Gorana Mitic

The aim of the study was the assessment of the prevalence of antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE). 72 patients with SLE had been investigated, 66 females and six males, aged 17 to 70 years, average 37,03. The presence of APA was determined using both ELISA assay for antiphospholipid antibodies ASSERACHROM APA by Diagnostica Stago and clotting tests for lupus anticoagulant: activated partial thromboplastin time (aPTT), tissue thromboplastin inhibition test (TTI) and dilute Russell viper venom time (dRVVT). Antiphospholipid antibodies have been found in 24 patients (33.44%), 10 of them were. with positive lupus anticoagulant tests, 6 of them were with positive ELISA test, while 8 of them had positive coagulation and immunological tests. Clinical manifestations that could be related to antiphospholipid syndrome were present in 22 patients (30.5%). The most common were thrombotic complications in 16 patients (22.25), recurrent spontaneous abortions in 7 patients (9.7%) and thrombocytopenia in 1 patient (1.39%). Presence of antiphospholipid syndrome was determined in 15 patients (20.83%). We can conclude that there is a significant correlation between presence of antiphospholipid antibodies and both thrombotic events and recurrent spontaneous abortions in SLE patients. Occurrence of thrombotic complications is in direct correlation with the level of antiphospholipid antibodies.


2021 ◽  
Vol 8 ◽  
Author(s):  
Yi Liu ◽  
Xuelei Liang ◽  
Haixuan Wu ◽  
Fenglin Zhuo

Introduction: Lichen planus/lupus erythematosus overlap syndrome is rarely seen in the clinic and has the characteristic clinical manifestations, histopathology, and immunopathology of lichen planus (LP) and lupus erythematosus (LE). This is the first reported case of bullous lichen planus (BLP)/systemic lupus erythematosus (SLE) overlap syndrome with hair loss as the first symptom.Case Presentation: A 48-year-old female presented with alopecia for half a year, and skin lesions accompanied by itching on her face, trunk, and limbs for 3 months. She had a history suggestive of photosensitivity. Laboratory tests and histopathology were performed for diagnosis. Histopathologic features of the upper arm and back of the hand were consistent with BLP, whereas the scalp lesion indicated LE. Laboratory examination indicated positive for antinuclear antibody (ANA) (1:160), leukopenia, increased urinary protein, decreased C3/C4, and normal BP180. The patient was given glucocorticoid combined with acitretin and immunosuppressive therapy after a definite diagnosis of BLP/SLE overlap syndrome. The lesions of the patient disappeared and some hair had regrown during the two years of follow-up.Conclusion: This is the first reported case of BLP/SLE overlap syndrome which responded well to glucocorticoids, retinoids, and immunosuppressive drugs. Multiple biopsies from characteristic lesions will guide doctors to avoid misdiagnoses and delayed treatment.


Kardiologiia ◽  
2019 ◽  
Vol 59 (12) ◽  
pp. 92-96
Author(s):  
N. A. Kosheleva ◽  
N. M. Nikitina ◽  
E. U. Andreeva

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology characterized by a wide range of clinical manifestations with damage to various organs and systems of the body. There are bad prognostic factors for SLE: damage to the heart, kidney, central nervous system, the development of hematological crises and secondary antiphospholipid syndrome. A number of authors consider systemic lupus erythematosus a “new” risk factor for atherosclerosis. The overall risk of myocardial infarction (MI) in patients with SLE is 10 times higher than in the general population. The article presents clinical case report of the development of myocardial infarction in a woman with SLE, receiving therapy for secondary antiphospholipid syndrome.


2019 ◽  
Vol 22 (1) ◽  
Author(s):  
Katarzyna Odzimkowska-Łata ◽  
Aleksandra Rybkowska ◽  
Edyta Olesińska

Juvenile systemic lupus erythematosus is an autoimmune, chronic, multisystemic inflammatory disease with an unknown etiology. The average age of juvenile systemic lupus erythematosus (jSLE) diagnosis is 11-14 years old, with a significant female predominance. Clinical manifestations of JSLE are extremely variable, from a relatively mild disease characterised by facial rash, joint pains, fever, fatigue, weight loss, alopecia and arthralgias to a severe life threatening illness.These and other symptoms of diffuse generalized inflammation including lymphadenopathy and hepatosplenomegaly occur both at onset and during disease flares.The authors present the case of a girl hospitalized in the Rheumatology Clinic due to fevers, skin lesions and haematological disorders. The diagnostic difficulty may have been the fact that the symptoms appear also in the course of many pediatric diseases. Finally, based on an in-depth medical history, physical examination and the results of immunological tests, they were recognized at the outset. Juvenile systemic lupus erythematosus is a disease with a diverse clinical picture, therefore, early diagnosis is not easy, but it is necessary to implement effective treatment. Knowledge and experience of the spectrum of paediatric and adolescent disease is important as well as recognition of when features merit further investigation.


Lupus ◽  
2018 ◽  
Vol 27 (10) ◽  
pp. 1644-1651 ◽  
Author(s):  
H H Kwon ◽  
S Y Bang ◽  
S Won ◽  
Y Park ◽  
J H Yi ◽  
...  

Objectives Avascular necrosis (AVN) is one of the most common causes of organ damage in patients with systemic lupus erythematosus (SLE) and often causes serious physical disability. The aims of this study were to investigate clinical risk factors associated with symptomatic AVN and to analyze their synergistic effects in a large SLE cohort in Korea. Methods Patients with SLE were enrolled and followed from 1998 to 2014 in the Hanyang BAE Lupus cohort, and damage was measured annually according to the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). AVN was confirmed by imaging study if patients had symptoms. To determine risk factors for AVN, clinical, laboratory and therapeutic variables were analyzed by logistic regression. Relative excess risk due to interaction (RERI), attributable proportion (AP), and synergy index (S) were calculated to measure interactions between significant variables. Results Among 1219 SLE patients, symptomatic AVN was the most common type of musculoskeletal damage (10.8%, n = 132). SLE patients with AVN showed an earlier onset age, demonstrated AVN more commonly in conjunction with certain other clinical manifestations such as renal and neuropsychiatric disorders, and received significantly higher total cumulative corticosteroid dose and immunosuppressive agents than did patients without AVN. However, in multivariable analysis, only two variables including use of a cumulative corticosteroid dose greater than 20 g (odds ratio (OR) 3.62, p = 0.015) and use of immunosuppressants including cyclophosphamide or mycophenolate mofetil (OR 4.51, p < 0.001) remained as significant risk factors for AVN. Patients with cumulative corticosteroid dose > 20 g and immunosuppressant use had a 15.44-fold increased risk for AVN, compared with patients without these risk factors ( p < 0.001). RERI, AP and S, which define the strength of interactions between two risk factors, were 9.01 (95% confidence interval (CI) 1.30–16.73), 0.58 (95% CI 0.36–0.81) and 2.66 (95% CI 1.42–4.99), respectively, supporting the presence of synergistic interactions in the development of symptomatic AVN in our Korean lupus cohort. Conclusions An individual risk assessment for AVN development should be made prior to and during treatment for SLE, especially in patients with high-dose corticosteroid and immunosuppressant use regardless of clinical manifestations and disease activity.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4685-4685
Author(s):  
Yang He ◽  
Weiwen Yang ◽  
Changgeng Ruan

Abstract Abstract 4685 Background: The occurrence of thrombotic thrombocytopenic purpura (TTP) in patients with systemic lupus erythematosus (SLE) is rare TTP frequence. In pregnancy women is increased. TTP with simultaneous SLE, and antiphospholipid syndrome (APS) occurring in the same patient is extremely rare. Case report: We described a 41-year-old Chinese female with TTP in her third trimester (33+4 gestational weeks) of pregnancy who suffered from SLE. and APS at the same time. The clinical features and positive laboratory abnormalities included fever, repeated abortion in first trimester, palpitation, dyspnea, orthopnea, hypertension (180/113mmHg), proteinuria, renal dysfunction, thrombocytopenia (platelets count 12×109/L), hemolytic anemia (Hb 67g/L), positive Coombs test, positive lupus test, positive antinuclear antibody, anti-Sm antibody, anti-insulin antibody, anti-ENA antibody and anti-RNP antibody. The level of plasma ADAMTS13 activity was below 5%, and ADAMTS13 inhibitor was present in the patient's plasma. This patient was treated with a combination of corticosteroids and plasma infusion and plasmapheresis. At 33+5 weeks of gestation she delivered a healthy infant via cesarean section. The patient improved quickly with these therapies. Conclusion: This is a rare TTP case with simultaneous SLE, APS and preeclampsia. Her overlaping clinical manifestations and laboratory abnormalities made the diagnosis very difficult. These data indicate that plasma therapy and corticosteroids can improve the prognosis of TTP associated with SLE and APS. In addition, the termination of pregnancy may be also helpful for controlling the development of the disease. Disclosures: No relevant conflicts of interest to declare.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1180.1-1180
Author(s):  
A. Borisova ◽  
T. Lisitsyna ◽  
D. Veltishchev ◽  
T. Reshetnyak ◽  
O. Seravina ◽  
...  

Background:Cognitive impairment (CI) in systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) patients have been poorly described and recognized.Objectives:to describe the rates and spectrum of CI in primary (PAPS) and secondary to systemic lupus erythematosus (SLE) (SAPS) APS patientsMethods:113 patients (70 with APS (37 – PAPS, 33 – SAPS) and 43 - SLE without APS), 89 (78,8%) – women, were consecutively enrolled in the study. The mean (M±SD) age was 37,9±11,9 years. SLE activity was measured by SLEDAI scale. Mental disorders (MD) were diagnosed by psychiatrist in accordance with ICI-10 in semi-structured interview. CI were diagnosed with psychology and neuropsychology methodsResults:CI of varying severity were found in 105 (92,9%) patients: 62,9% - mild, 23,8% - moderate and 13,3% - severe. Severe and moderate CI were more associated with APS (48,6% in PAPS and 39,5% in SAPS vs 18,6% in SLE, p=0,004 and p=0,04, accordingly). CI were predominantly organic origin in all patients, but vascular dementia was detected only among patients with APS (10,8% of PAPS and 3,03% of SAPS patients). There was no association of CI with clinical manifestations and SLE activity. In patients with PAPS CI was associated with stroke, livedo reticularis and lupus anticoagulant positivity. In 84 patients (74,3%) CI were also specifically bounded to MD. Current MD were detected in 100 (88,5%) patients: schizotypal disorder was found in 10 (8,85%) patients and was associated with PAPS (13,5% vs 9,09% in SAPS and 4,65% in SLE); anxiety-depressive spectrum disorders (ADDs) - in 95 (84,1%) (chronic and recurrent depression prevailed 37 (32,7%) and 42 (37,2%) resp.); the structure of MDs in accordance with ICI-10 differed slightly between groups, but no statistically significant differences were obtained.Conclusion:сognitive impairment, mainly of an organic type, are characteristic of most patients with SLE and APS. The significant associations of сognitive impairment with clinical manifestations and activity of SLE were not identified, but patients with сognitive impairments were more likely to have anxiety and depressive disorders, strokes, livedo reticularis and lupus anticoagulant positivityDisclosure of Interests:None declared


Sign in / Sign up

Export Citation Format

Share Document