scholarly journals FRI0172 THE INFLUENCE OF CALCINEURIN INHIBITORS ON DEVELOPMENT OF CANCER IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS: A RETROSPECTIVE OBSERVATIONAL STUDY IN THE LUNA REGISTRY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 670.1-670
Author(s):  
K. Ichinose ◽  
T. Igawa ◽  
M. Okamoto ◽  
A. Takatani ◽  
N. Yajima ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Multivariate Analysis ◽  
Lupus Erythematosus ◽  
Cervical Dysplasia ◽  
Calcineurin Inhibitors ◽  
Smoking History ◽  
Systemic Lupus ◽  
Treatment History ◽  
Steroid Dose ◽  
Risk Of Cancer

Background:It has been reported that the incidence of cancer in patients with systemic lupus erythematosus (SLE) is higher than that in healthy individuals, but the findings are inconsistent1. In the transplantation field, a few studies indicated an association between the use of immunosuppressants and an increased risk of cancer2. Calcineurin inhibitors (CNIs), which include cyclosporine and tacrolimus, have been used for >30 years to treat renal and extrarenal manifestations of SLE, but the effects of exposure to CNIs among SLE patients have not been established.Objectives:We investigated the incidence of various cancers (including cervical dysplasia) among SLE patients registered in the LUpus registry of NAtionwide institution (LUNA). We also investigate whether the registrants’ exposure to CNIs increased the risk of cancer.Methods:We calculated the standardized incidence ratio (SIR) of cancer among SLE patients based on the age-standardized incidence rate of cancer reported by Japan’s Ministry of Health, Labour and Welfare. A multivariate analysis of the risk of cancer was performed using the covariates of age, smoking history, CNI treatment history, maximum steroid dose in the past, and Systemic Lupus International Collaboration Clinics/American College of Rheumatology Damage Index [SDI]) value (excluding the occurrence of cancer) at the time of the patient’s registration.Results:We studied 714 patients (663 females; 88.9%).The median age at registry was 44 [interquartile range (IQR): 35–56] years. The median past max. steroid dose was 40 mg/day (IQR: 30–60 mg/day), and the SDI at registration was 1 (IQR 0–2). Smoking history was present in 248 patients (34.9%), and 53 patients (7.4%) experienced cancer complications. Gynecologic malignancies accounted for 71% of all cancers, including 12 cervical dysplasia cases. The standardized incidence rate of cancer in these SLE patients was 1.46 (95%CI: 1.07–1.85, p<0.01). The multivariate analysis showed that a CNI treatment history was not a risk factor for the development of cancer (OR 1.76, 95%CI: 0.63–4.88, p=0.30). After the covariance was adjusted for the propensity score, the risk of cancer in the CNIs group was not increased compared to the non-CNIs group (adjusted OR 2.46, 95%CI: 0.68–8.91, p=0.20).Conclusion:The incidence of cancer in SLE was higher in the LUNA cohort than in the general population. Our results suggest that CNI treatment for individuals with SLE is not a risk factor for the development of cancer.References:[1]Ladouceur A. et.al, Expert Rev Clin Immunol. 2018 Oct;14(10):793-802.[2]Gutierrez-Dalmau A. et.al, Drugs 2007;67(8):1167-98.Disclosure of Interests:None declared

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

The Early Protective Effect of Hydroxychloroquine on the Risk of Cumulative Damage in Patients with Systemic Lupus Erythematosus

2013 ◽  
Vol 40 (6) ◽  
pp. 831-841 ◽  
Author(s):  
Pooneh S. Akhavan ◽  
Jiandong Su ◽  
Wendy Lou ◽  
Dafna D. Gladman ◽  
Murray B. Urowitz ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Conditional Logistic Regression ◽  
Univariate Analysis ◽  
Damage Index ◽  
Immunosuppressive Drugs ◽  
Clinical Effects ◽  
Systemic Lupus ◽  
Steroid Dose ◽  
Damage Accrual

Objective.To assess whether hydroxychloroquine (HCQ) prevents early damage in patients with systemic lupus erythematosus (SLE).Methods.We updated an existing systematic review of literature on clinical effects of HCQ in patients with SLE. We conducted a nested case-control study embedded in an inception cohort of patients with SLE. Systemic Lupus International Collaborating Clinics Damage Index (SDI) at 3 years was considered as our primary outcome. Patients with SDI > 0 at 3 years were considered cases and patients with SDI = 0 were controls. Cases and controls were first compared by univariate analysis. Then conditional logistic regression models adjusting for potential confounders were done to study the effect of HCQ on damage accrual.Results.Included in the analysis were 481 patients who had 3 or more years of followup. Out of this cohort, we could match 151 cases with 151 controls. Univariate analysis identified age, the use of any immunosuppressive drugs, HCQ, and cumulative dose of steroids as significant covariates associated with damage accrual. In multivariate analysis, the use of HCQ remained significantly associated with less damage (OR 0.34, 95% CI 0.132–0.867), while age (OR 1.05, 95% CI 1.027–1.078) and a variable combining SLE activity and steroid dose (OR 1.73, 95% CI 1.306–2.295) were associated with damage at 3 years.Conclusion.We demonstrated that HCQ use was associated with less damage at 3 years after diagnosis of SLE when attention was given and adjustment done for disease activity and steroid dose, duration of disease, and calendar year of diagnosis.

scholarly journals Predisposition to Cervical Atypia in Systemic Lupus Erythematosus: A Clinical and Cytopathological Study

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Hend Hilal Al-Sherbeni ◽  
Ahmed Mohamed Fahmy ◽  
Nadine Sherif
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Complex Disease ◽  
Female Genital Tract ◽  
Cervical Dysplasia ◽  
Control Group ◽  
Pap Smears ◽  
Systemic Lupus ◽  
Noninvasive Technique ◽  
Rheumatological Disease

Introduction. Systemic lupus erythematosus (SLE) is a complex disease with variable presentations, course, and prognosis. The female genital tract may be a potential target organ in SLE since cervical inflammation may be associated with disease activity. An increase in cervical dysplasia, a precursor of cervical cancer, has been reported in females with SLE.Aim of the Work. This work aimed to study the prevalence of abnormal cervicovaginal smears in patients with systemic lupus erythematosus (SLE) and to correlate abnormal smear findings with exposure to infection with human papilloma virus (HPV) in SLE patients.Patients and Methods. Thirty-two patients with SLE, fulfilling the 1997 revised criteria for the classification of SLE, were included in this study. They were subjected to full history taking, clinical examination, laboratory investigations, and cervicovaginal smearing. Twenty healthy subjects not known to suffer from any rheumatological disease were used as controls, and they were subjected to cervicovaginal smearing.Results. Four out of 32 SLE patients showed abnormal Pap smears (12.5%) compared to none showing any cervical changes in the control group (0%). Among these 4 patients, 3 were having ASCU and one was having LSIL (HPV).Conclusion. Cervicovaginal smearing is an easy, economic, safe, repeatable, and noninvasive technique for screening and early detection of cervical neoplastic lesions in SLE.

High incidence of steroid complications related to cumulative steroid dose in systemic lupus erythematosus patients over the age of 50

1997 ◽  
Vol 6 (3) ◽  
pp. 141-147
Author(s):  
Domingo gnHernández ◽  
Aurelio Rodríguez ◽  
Margarita Rufino ◽  
Victor Lorenzo ◽  
Eduardo de Bonis ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Steroid Dose ◽  
High Incidence

scholarly journals Case Report: Hodgkin Lymphoma and Refractory Systemic Lupus Erythematosus Unveil Activated Phosphoinositide 3-Kinase-δ Syndrome 2 in an Adult Patient

2021 ◽  
Vol 9 ◽  
Author(s):  
Francesca Conti ◽  
Arianna Catelli ◽  
Cristina Cifaldi ◽  
Lucia Leonardi ◽  
Rita Mulè ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Immune Dysregulation ◽  
Iga Deficiency ◽  
Splice Acceptor Site ◽  
Acceptor Site ◽  
Systemic Lupus ◽  
Increased Risk ◽  
Phosphoinositide 3 Kinase ◽  
Risk Of Cancer

Introduction: Activated phosphoinositide 3-kinase-δ syndrome 2 (APDS2) is a rare primary immune regulatory disorder caused by heterozygous gain of function mutation in the PIK3R1 gene encoding PI3Kδ regulatory p85α subunit and resulting in PI3Kδ hyperactivation. Clinical features range from recurrent infections to manifestations of immune dysregulation like autoimmunity, inflammation, systemic lymphoproliferation, and increased risk of cancer. We describe a new dominant PIK3R1 mutation causing APDS2 presenting with lymphoma and systemic refractory autoimmunity.Case Presentation: A 30-year-old woman was referred to the Immunology Unit of our hospital for uncontrolled systemic lupus erythematosus, including chilblains lesions, systemic lymphoproliferation and IgA deficiency. At 19 years of age, she was diagnosed with Hodgkin's lymphoma. Subsequently, she presented systemic lupus erythematosus onset, with episodes of severe exacerbation, including autoimmune hemolytic anemia and pleuro-pericarditis. Initial clinical response to conventional treatments was reported. Immunological investigations performed during our first observation showed severe lymphopenia, IgA deficiency, elevated IgM with reduced IgG2 levels, and low vaccination antibody titers. Quantitative real-time polymerase chain reaction (PCR) assay for Cytomegalovirus and Epstein-Barr virus showed low viral loads for both viruses in serum. An increase of serum inflammatory markers highlighted persistent systemic hyperinflammation. The next-generation sequencing (NGS)-based gene panel tests for primary immunodeficiency showed a heterozygous A&gt;G substitution in the splice acceptor site at c.1300-2 position of PIK3R1, leading to exon-skipping.Conclusion: This case emphasizes the importance of suspecting primary immune regulatory disorders in young adults, predominantly showing a severe, aggressive, and refractory to treatment immune dysregulation phenotype, even in the absence of major infectious diseases at the onset. Different treatments can be promptly started, and a delayed diagnosis can highly impact the outcome. Targeted therapy against PI3Kδ pathway defect effectively improves drug-resistant autoimmunity, lymphoproliferation, and risk of progression to malignancy; eligible patients could benefit from its use even as a bridge therapy to transplantation, currently the only definitive curative treatment. Therefore, identifying genetic mutation and prompt targeted treatment are essential to control disease manifestations, prevent long-term sequelae, and enable curative HSCT in APDS2 patients.

Systemic lupus erythematosus–associated diffuse alveolar hemorrhage: a single-center, matched case–control study in China

Lupus ◽  
2020 ◽  
Vol 29 (7) ◽  
pp. 795-803 ◽  
Author(s):  
Yiduo Sun ◽  
Cong Zhou ◽  
Jiuliang Zhao ◽  
Qian Wang ◽  
Dong Xu ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Multivariate Analysis ◽  
Prognostic Factors ◽  
Lupus Erythematosus ◽  
Case Control ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Single Center ◽  
Systemic Lupus

Background This study described clinical characteristics and outcome in systemic lupus erythematosus (SLE) patients with diffuse alveolar hemorrhage (DAH), and investigated risk factors and prognostic factors for DAH. Methods We conducted a retrospective nested case–control analysis in a single-center cohort. We enrolled 94 SLE patients with DAH. For each case of DAH, two age-, sex-, and SLE courses–matched controls were randomly selected from our cohort. All patients were enrolled between 2004 and 2019 and were followed until death, end of registration with the physician’s practice, or end of January 2019. We estimated the risk factors for DAH and prognostic factors for mortality using multivariate analysis. Results We included 4744 patients diagnosed with SLE, with 94 cases of DAH, for an incidence rate of 2.0%. DAH may occur in any stage of SLE but mostly in the early phase of disease course. Lupus nephritis (LN) was the most common concomitant involvement at DAH diagnosis. By multivariate analysis, LN, anti-SSA positivity, thrombocytopenia and elevated C-reactive protein (CRP) were significantly associated with DAH in SLE patients. All-cause mortality was increased in SLE with DAH compared with SLE without DAH (adjusted hazard ratio 6.0, 95% confidence interval 2.8–13.0, p < 0.0001). Intravenous cyclophosphamide (CTX) showed an increased tendency for better survival in DAH after adjusting for Systemic Lupus Erythematosus Disease Activity Index 2000, acute kidney injury and mechanical ventilation. Conclusions LN, anti-SSA positivity, thrombocytopenia and elevated CRP were independent risk factors of DAH in lupus patients. Due to a high early death rate of DAH and little long-term damage, DAH patients may benefit from early diagnosis and intensive treatment, and CTX-based therapy can be a preferential choice.

scholarly journals The role of clinically significant antiphospholipid antibodies in systemic lupus erythematosus

Reumatismo ◽  
2016 ◽  
Vol 68 (3) ◽  
pp. 137 ◽  
Author(s):  
M. Taraborelli ◽  
M.G. Lazzaroni ◽  
N. Martinazzi ◽  
M. Fredi ◽  
I. Cavazzana ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Multivariate Analysis ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Valvular Disease ◽  
Systemic Lupus ◽  
Increased Risk ◽  
Clinically Significant ◽  
Cutaneous Lupus

The objective is to investigate the role of clinically significant antiphospholipid antibodies (aPL) in a cohort of systemic lupus erythematosus (SLE) patients. All SLE patients followed for at least 5 years and with available aPL profile at the beginning of the follow-up in our center were studied. Clinically significant aPL were defined as: positive lupus anticoagulant test, anti-cardiolipin and/or anti- β2Glycoprotein I IgG/IgM &gt;99<sup>th</sup> percentile on two or more occasions at least 12 weeks apart. Patients with and without clinically significant aPL were compared by univariate (Chi square or Fisher’s exact test for categorical variables and Student’s <em>t</em> or Mann-Whitney test for continuous variables) and multivariate analysis (logistic regression analysis). P values &lt;0.05 were considered significant. Among 317 SLE patients studied, 117 (37%) had a clinically significant aPL profile at baseline. Such patients showed at univariate analysis an increased prevalence of deep venous thrombosis, pulmonary embolism, cardiac valvular disease, cognitive dysfunction and antiphospholipid syndrome (APS), but a reduced prevalence of acute cutaneous lupus and anti-extractable nuclear antigens (ENA) when compared with patients without clinically significant aPL. Multivariate analysis confirmed the association between clinically significant aPL and reduced risk of acute cutaneous lupus [p=0.003, odds ratio (OR) 0.43] and ENA positivity (p&lt;0.001, OR 0.37), with increased risk of cardiac valvular disease (p=0.024, OR 3.1) and APS (p&lt;0.0001, OR 51.12). Triple positivity was the most frequent profile and was significantly associated to APS (p&lt;0.0001, OR 28.43). Our study showed that one third of SLE patients had clinically significant aPL, and that this is associated with an increased risk, especially for triple positive, of APS, and to a different clinical and serological pattern of disease even in the absence of APS.

The association between systemic lupus erythematosus and bipolar disorder – a big data analysis

2017 ◽  
Vol 43 ◽  
pp. 116-119 ◽  
Author(s):  
S. Tiosano ◽  
Z. Nir ◽  
O. Gendelman ◽  
D. Comaneshter ◽  
H. Amital ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Bipolar Disorder ◽  
Multivariate Analysis ◽  
Big Data ◽  
Data Analysis ◽  
Lupus Erythematosus ◽  
Univariate Analysis ◽  
Big Data Analysis ◽  
Medical Database ◽  
Systemic Lupus

AbstractBackground:Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease that has a wide variety of physical manifestations, including neuropsychiatric features. Bipolar disorder (BD) is a chronic, episodic illness, that may present as depression or as mania. The objective of this study was to investigate the association between SLE and BD using big data analysis methods.Methods:Patients with SLE were compared with age- and sex-matched controls regarding the prevalence of BD in a cross-sectional study. Chi-square and t-tests were used for univariate analysis and a logistic regression model was used for multivariate analysis, adjusting for confounders. The study was performed utilizing the chronic disease registry of Clalit Health Services medical database.Results:The study included 5018 SLE patients and 25,090 matched controls. BD was found in a higher prevalence among SLE patients compared to controls (0.62% vs. 0.26%, respectively, P < 0.001). BD patients had a greater prevalence of smokers compared to non-BD patients (62.5% vs 23.5%, respectively, P < 0.001). In a multivariate analysis, smoking and SLE were both found to be significantly associated with BD.Conclusions:SLE was found to be independently associated with BD. These findings may imply that an autoimmune process affecting the central nervous system among SLE patients facilitates the expression of concomitant BD.

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