scholarly journals FRI0224 IDENTIFICATION OF RISK AND PROGNOSTIC FACTORS FOR POLYARTERITIS NODOSA PATIENTS WITH DIGITAL GANGRENE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 695.2-695
Author(s):  
D. Xu ◽  
X. Tian ◽  
X. Zeng ◽  
F. Zhang ◽  
L. Zhao ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Prognostic Factors ◽  
Retrospective Study ◽  
Polyarteritis Nodosa ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Response To Therapy ◽  
Smoking History ◽  
Systemic Lupus ◽  
Poor Outcomes

Background:Polyarteritis nodosa (PAN) is a segmental, necrotizing vascular disease that primarily impacts medium-sized muscle arteries. The estimated annual incidence of PAN is still lacking in China. Digital gangrene is an ischemic manifestation of the limb. However, the causes and the treatment methods vary from case to case, and the outcome is unpredictable. These features emphasize the need to identify measurable variables that accelerate digital gangrene development in PAN patients. However, little effort has been made to identify the clinical and laboratory factors that affect PAN patients with digital gangrene to anticipate their natural history and response to therapy.Objectives:Many patients with polyarteritis nodosa (PAN) complicated with digital gangrene have poor outcomes and related research information is limited. This study was carried out to identify the associated risk and prognostic factors.Methods:We conducted a retrospective study of 148 PAN patients admitted to Peking Union Medical College Hospital (PUMCH) from September 1986 to December 2018. The characteristics, therapeutic regimens, and outcome data for patients with and without gangrene were compared. The Kaplan–Meier method and Cox hazard regression model were used to evaluate the prognostic factors.Results:Forty-seven (31.8%) PAN patients had digital gangrene complications. The average age was 40.4±17.9 years and the average disease duration was 11 (4-27) months. The presence of digital gangrene was correlated with smoking history [odds ratio (OR), 4.27; 95% confidence interval (95% CI), 1.56-11.66] and eosinophil elevation (28.12; 10.30-76.8). Thirty-two (68.1%) gangrene patients received methylprednisolone pulse therapy and all of these patients were treated with cyclophosphamide. Nine patients suffered irreversible organ injury and two died. Disease duration ≥ 24 months and elevated serum C-reactive protein (CRP) were identified as hazardous factors for poor prognosis in patients with gangrene (P=0.003, HR=8.668, 95% CI 2.11, 35.55 andP=0.042, HR=27.062, 95% CI 1.13, 648.57, respectively).Conclusion:Smoking history and eosinophil elevation in PAN patients were more prone to digital gangrene and high serum CRP level predicted poor outcomes. PAN patients with smoking history and elevated eosinophils need to be seriously evaluated by clinicians. Furthermore, the CRP level should be efficiently controlled for good prognosis.References:[1]De Virgilio A, Greco A, Magliulo G, Gallo A, Ruoppolo G, Conte M, et al. Polyarteritis nodosa: A contemporary overview. Autoimmun Rev. 2016;15:564-70.[2]Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62:616-26.[3]Xu D, You X, Wang Z, Zeng Q, Xu J, Jiang L, et al. Chinese Systemic Lupus Erythematosus Treatment and Research Group Registry VI: Effect of Cigarette Smoking on the Clinical Phenotype of Chinese Patients with Systemic Lupus Erythematosus. PLoS One. 2015;10:e0134451.Acknowledgments:NoDisclosure of Interests:Dong Xu: None declared, Xinping Tian: None declared, Xiaofeng Zeng Consultant of: MSD Pharmaceuticals, Fengchun Zhang: None declared, Lin Zhao: None declared, Shangzhu Zhang: None declared, Jiaxin Zhou: None declared, Jiu-liang Zhao: None declared, Xiaodan Kong: None declared

scholarly journals Pattern and prevalence of neuropsychiatric lupus: a retrospective study from a tertiary level hospital in Bangladesh

2021 ◽  
Vol 57 (1) ◽  
Author(s):  
Fahima Hossain ◽  
Mohammad Delwer Hossain Hawlader ◽  
Dipak Kumar Mitra ◽  
Mohammad Hayatun Nabi ◽  
Md. Mujibur Rahman
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Retrospective Study ◽  
Cognitive Dysfunction ◽  
Lupus Erythematosus ◽  
Neuropsychiatric Symptoms ◽  
Tertiary Care ◽  
Care Hospital ◽  
Systemic Lupus ◽  
Case Definitions ◽  
American College Of Rheumatology

Abstract Background Neuropsychiatric systemic lupus erythematosus (NPSLE) is well known for its varying presentations and poor outcomes, but little is evident about its distribution and characteristics among the Bangladeshi population. This study aimed to assess the pattern and prevalence of neuropsychiatric symptoms in female systemic lupus erythematosus (SLE) patients of Bangladesh. A retrospective study was conducted at a tertiary care hospital in Dhaka, Bangladesh, between January and December 2018. One hundred female SLE patients were included in the study purposively. Data were collected on sociodemographic and clinical characteristics of diagnosed SLE cases visiting the SLE clinic and indoor medicine department. Neuropsychiatric (NP) syndromes were defined according to the widely accepted American College of Rheumatology (ACR) nomenclature and case definitions. Results A total of 244 NP events were identified in fifty-five patients. Headache was the most frequent symptom (55%), followed by cognitive dysfunction (50%), anxiety (49%), psychosis (43%), seizure (23%), depression (17%), and cerebrovascular disease (ischemic type, 7%). The NP manifestations were more prevalent among urban residents (58.2%), younger patients (41.8%), and patients with graduate-level education (34.5%). Besides, young age at diagnosis (p = 0.038), Raynaud’s phenomenon (p = 0.015), other organ involvement (p < 0.001), and time of NPSLE development (p < 0.001) were found to be significantly associated with the development of these manifestations. Conclusion NP damage is prevalent among Bangladeshi female SLE patients (55%) with headache and cognitive dysfunction being the most common symptoms. Routine screening for neuropsychiatric symptoms among suspected SLE cases and further evaluation with a larger population are warranted.

scholarly journals POS0712 “EXTRA”- CRITERIA ANTIPHOSPHOLIPID ANTIBODIES IN PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME AND SYSTEMIC LUPUS ERYTHEMATOSUS (PRELIMINARY DATA)

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 605.2-606
Author(s):  
F. Cheldieva ◽  
T. Reshetnyak ◽  
M. Cherkasova ◽  
N. Seredavkina ◽  
A. Lila
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Preliminary Data ◽  
Antiphospholipid Antibodies ◽  
Disease Duration ◽  
Past History ◽  
Igg Antibodies ◽  
Systemic Lupus ◽  
Pregnancy Morbidity

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

scholarly journals AB0421 EFFECT OF BODY WEIGHT ON COMPLEMENT LEVELS IN SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1510.3-1510
Author(s):  
L. Kondrateva ◽  
T. Popkova ◽  
E. Nasonov ◽  
A. Lila
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Normal Weight ◽  
World Health ◽  
Systemic Lupus ◽  
Obesity Status ◽  
Health Organization ◽  
The Complement System

Background:The complement system is a recognized biomarker for diagnosis or monitoring of disease activity in systemic lupus erythematosus (SLE) patients (pts). But on the other hand, it has been linked to insulin resistance and obesity in general population.Objectives:To find out whether overweight/obesity can modify C3 or C4 levels in SLE pts.Methods:A total of 92 SLE pts (83 women, 9 men, 39 [34;47] years old) were enrolled in the study. Median disease duration was 6[2;14] years, and SLE activity using SLEDAI-2K was 4[2;8]. SLE pts were treated with glucocorticoids (89%), hydroxychloroquine (78%), immunosuppressants (28%), biologics (10%). The overweight/obesity status was determined by World Health Organization criteria in patients with body mass index (BMI) ≥25kg/m2.Results:Overweight/obesity were established in 46% SLE pts. Overweight/obese SLE pts were older than pts with normal BMI (40[36;48] vs 37[31;44] years, р=0,02), and had lower SLEDAI-2K (3[2;6] vs 6[4;8], p<0,01). Lower C3 concentrations were found in 36% overweight/obese pts vs 68% pts with normal weight (р<0,01), decreased C4 levels - in 19% vs 30% pts (p=0,33), median C3 concentrations were 0,98[0,81;1,14] g/l vs 0,84[0,69;0;96] g/l (р<0,01), and C4 levels were 0,15[0,10;0,19] g/l vs 0,12[0,09;0,16] g/l, respectively (p=0,03). C3 and C4 levels negatively correlated with SLEDAI-2K (r=-0,5, p<0,01 for both), the effect was more strongly pronounced in patients with BMI≥25kg/m2 (r=-0,6, p<0,01 for both) than in those with normal weight (r=-0,2, p=0,09 for C3, r=-0,3, p=0,04 for C4).Conclusion:Overweight/obesity status in SLE pts was associated with increased levels of complement proteins, therefore decreased C3 or C4 levels in patients with BMI≥25kg/m2 are more likely related to disease activity and, can potentially induce SLE flares.Disclosure of Interests: :None declared

scholarly journals SAT0232 PERCEPTION OF THE DISEASE IN PATIENTS WITH EARLY SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1059.3-1059
Author(s):  
M. Garabajiu ◽  
L. Mazur-Nicorici ◽  
T. Rotaru ◽  
V. Salaru ◽  
S. B. Victoria ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Activity Index ◽  
Damage Index ◽  
Organ Damage ◽  
Systemic Lupus

Background:Systemic lupus erythematosus is an autoimmune disease with a major impact on patient’s quality of life.Objectives:To evaluate patient’s attitude toward early disease and factors that influence it.Methods:Performed case-control study included SLE patients that fulfilled SLICC, 2012 classification criteria. The research included two groups of patients: early SLE – 1stgroup (disease duration ≤24 months) and non-early SLE – 2ndgroup control (disease duration >24 months). The pattern of the disease activity was assessed by patient global assessment (PGA), Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and Systemic Lupus Activity Measure (SLAM), for SLE activity, SLICC/ACR Damage Index (DI) for disease irreversible changes and SF-8 for the Quality of Life (QoL).Results:A total of 101 SLE patients with 34 in the 1stgroup (early SLE) and 67 in the 2ndgroup (non-early SLE) was analyzed. The disease activity showed high disease activity in both groups by SLEDAI (7,02±4,16 and 6,26±4,43 points, p>0,05) and SLAM (7,47±4,40 and 7,31±4,10 points, p>0,05) such as (46,97±19,39 vs 47,98±22,41 points). The QoL was appreciated as low, by both components (mental and physical), in groups. The damage index was higher in the 2nd group (0,23±0,43 and 1,07±1,29, p<0,001), which can be explained by the development of irreversible changes with the increase of disease duration.The PGA in early SLE was influenced by subjective symptoms contained in SLAM index (r=0,48, p<0,05), such as fatigue and depression, and the level of the quality of life (r=0,65, p<0,001). Meantime, PGA in patients with longer disease duration (>2 years), was influenced by the presence of organ damage by SLICC/ACR DI (0,23, p<0,05) and objective findings of the disease activity contained in SLEDAI (r=0,33, p<0,005) and SLAM (0,44, p<0,001).Conclusion:The disease recognition in patients with early SLE was determined by subjective and psycho-emotional signs, while in patients with longer disease duration it was influenced by organ damage and complications.References:no referencesDisclosure of Interests:None declared

scholarly journals Mitochondrial DNA damage is associated with damage accrual and disease duration in patients with Systemic Lupus Erythematosus

Lupus ◽  
2014 ◽  
Vol 23 (11) ◽  
pp. 1133-1141 ◽  
Author(s):  
L López-López ◽  
M Nieves-Plaza ◽  
M del R Castro ◽  
YM Font ◽  
CA Torres-Ramos ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Dna Damage ◽  
Mitochondrial Dna ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Systemic Lupus ◽  
Mitochondrial Dna Damage ◽  
Damage Accrual

Increased Serum Soluble OX40 in Patients with Systemic Sclerosis

2008 ◽  
Vol 35 (12) ◽  
pp. 2359-2362 ◽  
Author(s):  
KAZUHIRO KOMURA ◽  
AYUMI YOSHIZAKI ◽  
MASANARI KODERA ◽  
YOHEI IWATA ◽  
FUMIHIDE OGAWA ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Systemic Sclerosis ◽  
Lupus Erythematosus ◽  
Tumor Necrosis ◽  
Disease Duration ◽  
Tumor Necrosis Factor Receptor ◽  
Healthy Individuals ◽  
Systemic Lupus ◽  
Clinical Associations ◽  
Necrosis Factor

ObjectiveTo determine levels of serum soluble OX40 (also termed CD134, a member of the tumor necrosis factor receptor superfamily) and their clinical associations in patients with systemic sclerosis (SSc).MethodsSerum soluble OX40 levels were examined by ELISA in 53 patients with SSc, 15 patients with systemic lupus erythematosus (SLE), and 32 healthy individuals.ResultsOX40 levels were significantly elevated in SSc patients (125.7 ± 5.7 pg/ml) compared to patients with SLE (80.7 ± 1.7 pg/ml; p < 0.005) and controls (88.2 ± 3.0 pg/ml; p < 0.0001). Elevated OX40 levels were found to be associated with disease duration of less than 2 years (p < 0.05).ConclusionOur results suggest that serum soluble OX40 levels correlate with the early-onset of SSc disease.

scholarly journals An Early Unexpected Immune Thrombotic Thrombocytopenic Purpura Relapse Associated with SARS-CoV-2 Infection: A Case Report and Literature Review

2021 ◽  
pp. 1-5
Author(s):  
Maya Kornowski Cohen ◽  
Liron Sheena ◽  
Yair Shafir ◽  
Vered Yahalom ◽  
Anat Gafter-Gvili ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Response To Therapy ◽  
Intravascular Hemolysis ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura ◽  
History Of ◽  
Triggering Factor

SARS-CoV-2 has been reported as a possible triggering factor for the development of several autoimmune diseases and inflammatory dysregulation. Here, we present a case report of a woman with a history of systemic lupus erythematosus and antiphospholipid syndrome, presenting with concurrent COVID-19 infection and immune thrombotic thrombocytopenic purpura (TTP). The patient was treated with plasma exchange, steroids, and caplacizumab with initial good response to therapy. The course of both TTP and COVID-19 disease was mild. However, after ADAMTS-13 activity was normalized, the patient experienced an early unexpected TTP relapse manifested by intravascular hemolysis with stable platelet counts requiring further treatment. Only 3 cases of COVID-19 associated TTP were reported in the literature thus far. We summarize the literature and suggest that COVID-19 could act as a trigger for TTP, with good outcomes if recognized and treated early.

Sign in / Sign up

Export Citation Format

Share Document