scholarly journals Evolving phenotype of systemic lupus erythematosus in Caucasians: low incidence of lupus nephritis, high burden of neuropsychiatric disease and increased rates of late-onset lupus in the ‘Attikon’ cohort

Lupus ◽  
2020 ◽  
Vol 29 (5) ◽  
pp. 514-522 ◽  
Author(s):  
D Nikolopoulos ◽  
M Kostopoulou ◽  
A Pieta ◽  
T Karageorgas ◽  
D Tseronis ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Tertiary Care ◽  
Disease Diagnosis ◽  
Systemic Lupus ◽  
Neuropsychiatric Disease ◽  
Damage Accrual ◽  
Caucasian Patients

Objective This study aimed to analyse the phenotype of systemic lupus erythematosus (SLE) at first presentation and during follow-up in a newly established SLE cohort based at ‘Attikon’ University Hospital. The hospital combines primary, secondary and tertiary care for the region of Western Attica, Greece. Methods This study comprised a mixed prevalent and incident cohort of 555 Caucasian patients diagnosed with SLE according to American College of Rheumatology 1997 criteria and/or the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) 2012 criteria. Demographic and clinical characteristics, patterns of severity, treatments and SLICC damage index were recorded for each patient at the time of diagnosis and at last evaluation. Results The mean age at lupus diagnosis was 38.3 years (standard deviation = 15.6 years), with a median disease duration at last follow-up of two years (interquartile range 1-11). At initial presentation, the most common ‘classification’ manifestations were arthritis (73.3%), acute cutaneous lupus (65%) and unexplained fever (25%), while among symptoms not included in any criteria set, Raynaud’s phenomenon (33%) was the most common. Kidney and neuropsychiatric involvement as presenting manifestations were present in 10.3% and 11.5% cases, respectively. Irreversible damage accrual was present in 17.8% within six months of disease diagnosis, attributed mainly to thrombotic and neuropsychiatric disease. At last evaluation, 202 (36.4%) patients had developed severe disease, of whom more than half were treated with pulse cyclophosphamide. Conclusion In this cohort of Caucasian patients, lupus nephritis is not as common as in older cohorts, while neuropsychiatric disease is emerging as a major frontier in lupus prevention and care. These data may help to document changes in the natural history and treatment of SLE over time and may have implications for its early recognition and management.

Characteristics of lupus and lupus nephritis at a tertiary care center in Lebanon

Lupus ◽  
2019 ◽  
Vol 28 (13) ◽  
pp. 1598-1603 ◽  
Author(s):  
S H Koubar ◽  
J Kort ◽  
S Kawtharani ◽  
M Chaaya ◽  
M Makki ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Kidney Disease ◽  
Complete Remission ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Partial Remission ◽  
Care Center ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Systemic Lupus

Introduction Systemic lupus erythematosus affects 4.8–78.5 people per 100,000 worldwide, 90% of whom are females. Geography and ethnicity have been shown to significantly affect the prevalence and natural history of the disease. Lupus nephritis affects around half of patients with systemic lupus erythematosus. Data about systemic lupus erythematosus and lupus nephritis in the Middle East are still scarce. In this study, we aimed to describe the characteristics of systemic lupus erythematosus and lupus nephritis at a tertiary care center in Lebanon. Methods This is a retrospective chart review of all biopsy-proven lupus nephritis patients admitted to the American University of Beirut medical center between January 2000 and December 2018. Patients above 12 years of age who had any International Society of Nephrology/Renal Pathology Society (ISN/RPS) class of lupus nephritis on their renal biopsy were included in the study. Results The study included 55 patients with lupus nephritis. Upon presentation of systemic lupus erythematosus, the most common clinical feature was arthritis, seen in 83% of patients, followed by anemia (82%) and malar rash (48%). In total 93% had positive ANA, 89% had positive anti-dsDNA and 98% of patients had proteinuria. The most common ISN/RPS class of lupus nephritis in our series was IV (49%). At the time of the biopsy 15% of patients underwent dialysis. At 6 months, 11/27 had complete remission, 6/27 had partial remission and 10/27 had no remission. At 1 year, 8/23 had complete remission, 4/23 had partial remission and 11/23 had no remission. During the study period, 15 out of 35 patients available for analysis had chronic kidney disease (CKD) and six out of 34 patients developed end-stage kidney disease requiring renal replacement therapy. In comparison to other series in the region, our series had more males affected, higher creatinine at the time of biopsy and greater degree of proteinuria. Conclusion Our study provided insight on the demographics, characteristics, and outcomes of lupus nephritis in Lebanon. Interestingly, male gender was present in a quarter of patients. This warrants further investigation and confirmation. We are hoping to expand this experience into a national prospective registry to further characterize this entity in our region.

Idiopathic membranous nephropathy preceding membranous lupus nephritis: a case report

Lupus ◽  
2020 ◽  
Vol 29 (3) ◽  
pp. 340-343
Author(s):  
C Gökalp ◽  
G Aygun ◽  
A F Dogan ◽  
U Usta ◽  
I Kurultak ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Membranous Nephropathy ◽  
Adult Population ◽  
Idiopathic Membranous Nephropathy ◽  
Systemic Lupus ◽  
Common Causes ◽  
Membranous Lupus Nephritis

Membranous nephropathy is one of the most common causes of nephrotic syndrome in the adult population. According to the underlying etiology, membranous nephropathy is classified as either primary or secondary. Systemic lupus erythematosus is an autoimmune disease that can affect the kidneys in 50% of patients in the course of the disease. Renal disease may be the first manifestation of systemic lupus erythematosus and the development of systemic findings may be delayed for about 1–5 years following the diagnosis of lupus nephritis. We present a 59-year-old male patient who had a diagnosis of idiopathic membranous nephropathy since 2007 and developed membranous lupus nephritis during the 12-year follow-up without any extrarenal systemic lupus erythematosus findings.

scholarly journals Clinical And Morphological Improvement Of Lupus Nephritis Treated With Rituximab

Folia Medica ◽  
2014 ◽  
Vol 56 (4) ◽  
pp. 245-252 ◽  
Author(s):  
Maria E. Tsanyan ◽  
Sergey K. Soloviev ◽  
Stefka G. Radenska-Lopovok ◽  
Anna V. Torgashina ◽  
Ekaterina V. Nikolaeva ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Cell Therapy ◽  
Disease Activity ◽  
B Cell ◽  
Renal Biopsy ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Renal Biopsies

Abstract Aim: TO assess the effects of rituximab (RTM) therapy on clinical and morphologic activity of lupus nephritis (LN). Material and methods: The study included 45 patients with confirmed diagnosis of systemic lupus erythematosus (SLE), unaffected by previously received standard therapy with glucocorticoids (GCs) and cytostatics. The disease activity was assessed using Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2K); to assess the LN activity we used the SLICC RA/RE index. Forty-five patients with LN were given puncture renal biopsy prior to prescribing RTM; 16 patients had repeated renal biopsy 1 year and more after beginning the anti-B-cell therapy. LN was graded histologically in accordance with the WHO classification (2003) with indices of activity (AI) and chronicity (CI). Results: The predominant number of patients had class III - IV of LN. The repeated renal biopsies demonstrated that LN had undergone a transition into a more favourable morphologic class, which was associated, in most of these cases, with a positive therapeutic effect. The follow-up dynamics showed a statistically significant reduction of AI (p=0.006), and no statistically significant changes in the CI (p = 0.14). Conclusion: The long-term follow-up in the study has showed that repeated courses of anti-B-cell therapy with RTM have a positive effect both on SLE activity and generally on the renal process. The reduction of the morphologic class of LN as assessed in the repeated renal biopsies is a convincing proof for this. Eleven out of 16 patients experienced transition of the morphologic class into a more favourable type, which in most cases was combined with lower AI (p = 0.006). We found no evidence of increase in the CI (p = 0.14).

scholarly journals Childhood Systemic Lupus Erythematosus: A Tertiary Care Centre Experience

2016 ◽  
Vol 35 (2) ◽  
pp. 111-116
Author(s):  
Luna Bajracharya ◽  
Surya Bahadur Thapa
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Tertiary Care ◽  
Disease Onset ◽  
University Teaching ◽  
Systemic Lupus ◽  
Tertiary Care Centre ◽  
Number Of Patients ◽  
Active Lupus

Introduction: Systemic lupus erythematosus (SLE) is a chronic immunologic disorder with multisystem manifestations. Even more awareness is required to diagnose the disease at younger age. Objective of this study was to explore clinico-laboratory manifestations and management of SLE in children at Tribhuvan University Teaching Hospital (TUTH).Materials and Methods: The study was retrospective hospital based study conducted from 15th July, 2008 to 14th July, 2014. Medical charts of all children and adolescent (6- 16years of age) with SLE admitted at TUTH were reviewed for analysis of data.Results: The total number of patients was 33, with 28(84.8%) girls and 5 (15.2%) boys. The mean age of diagnosis was 12.12 (SD 1.89). Facial puffiness (27.3%) and arthralgia (24.2%) were the commonest presentations at disease onset. The most frequent clinical features during the entire course of illness were edema (78.9%), anemia (69.7%) and fever (66.7%). Twenty three (69.6%) patients underwent renal biopsy in which class IV was the commonest lupus nephritis. The commonly used drugs after prednisolone were intravenous cyclophosphamide, intravenouse methylprednisolone and mycophenolate mofetil. Total 17 (51.5%) patients went into remission. Two patients died due to active lupus and four due to sepsis.Conclusion: Lupus nephritis was the commonest feature at disease onset, at the time of diagnosis and throughout the disease course among Nepalese children with SLE. The most frequently used medications were prednisolone and iv cyclophosphamide. Infection and active lupus were the leading causes of complications and death.J Nepal Paediatr Soc 2015;35(2):111-116

scholarly journals AB0376 DETERMINANTS AND PROTECTIVE EFFECTS OF A LOW DISEASE ACTIVITY STATE IN SYSTEMIC LUPUS ERYTHEMATOSUS: RESULTS FROM A PROSPECTIVE CHINESE COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1488-1489
Author(s):  
Y. Hao ◽  
L. Ji ◽  
D. Gao ◽  
Y. Fan ◽  
E. F. Morand ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Asia Pacific ◽  
Systemic Lupus ◽  
Low Disease Activity ◽  
Disease Flare ◽  
Damage Accrual ◽  
Activity State

Background:The concept of treat to target in systemic lupus erythematosus has moved forward in recent years. The Lupus low disease activity state (LLDAS) defined by the Asia-Pacific Lupus Collaboration (APLC) in 2016 has been validated prospectively in the APLC cohort itself and retrospectively in multiple other cohorts.Objectives:The concept of treat to target in systemic lupus erythematosus has moved forward in recent years. The Lupus low disease activity state (LLDAS) defined by the Asia-Pacific Lupus Collaboration (APLC) in 2016 has been validated prospectively in the APLC cohort itself and retrospectively in multiple other cohorts. The aim of this study was to investigate the frequency and determinants of achieving LLDAS, and the influence of LLDAS on short term outcomes including disease flare and damage accrual in Chinese lupus patients.Methods:The baseline and follow-up data of all consecutive patients in a longitudinal lupus cohort from January 2017 to December 2018 were collected prospectively. SLEDAI-2K, PGA and disease flare were assessed at each follow-up visit, and further compared to the previous routine clinical visits. Irreversible disease damage was captured using the SLICC damage index and the short form (36) health survey for health-related quality of life was completed annually.Results:One hundred and forty-nine patients were enrolled, with the median disease duration at recruitment of 2.4 (0.9–8.2) years, and median follow-up of 15.4 (10.1-18.2) months. By the end of the study, 104 (69.8%) patients achieved LLDAS at least once; 59 patients achieved LLDAS for≥50% of observations. Multivariate logistic regression analysis showed that age at disease onset< 30 years (OR=0.05, 95%CI [0.01-0.59], p=0.017), 24-hour urine total protein (UTP) level at recruitment (OR=0.9992, 95%CI [0.9987-0.9998], p=0.007), and C3 level (OR=1.004, 95%CI [1.001-1.008], p=0.024) had independent associations with achieving LLDAS for≥50% of all observations (Table 1). During follow-up, 56 (37.6%) patients experienced disease flare including 14 (9.4%) patients with severe flare. Kaplan-Meier analyses showed significant differences in flare rates according to whether LLDAS was achieved and the percentage follow-up time in LLDAS (Figure 1). Multivariate cox analysis revealed that the percentage time of time in LLDAS was an independent negative determinant of disease flare (HR=0.18, 95% CI [0.07-0.48], p=0.001) (Table 2). There were 16 (15.0%)/107 patients who had damage accrual after one year of follow-up. Multivariate logistic analysis showed a tendency for achieving LLDAS during follow-up being protective for damage accrual (OR=0.27, 95%CI [0.07-1.00], p=0.050).Conclusion:In this Chinese early disease cohort, LLDAS was an attainable goal in clinical practice. Age at onset, UTP and C3 level at recruitment influenced achievement of LLDAS. LLDAS was negatively associated with disease flare and damage accrual; this needs to be confirmed by future longer follow-up.Acknowledgments:The data in this cohort was collected and recorded using the framework of the lupus low disease activity status (LLDAS) study from the Asia-Pacific Lupus Collaboration (APLC).Disclosure of Interests:Yanjie Hao: None declared, Lanlan Ji: None declared, Dai Gao: None declared, Yong Fan: None declared, Eric F. Morand Grant/research support from: AstraZeneca, Consultant of: AstraZeneca, Speakers bureau: AstraZeneca, Mandana Nikpour: None declared, Zhuoli Zhang: None declared

Longitudinal associations of active renal disease with irreversible organ damage accrual in systemic lupus erythematosus

Lupus ◽  
2019 ◽  
Vol 28 (14) ◽  
pp. 1669-1677 ◽  
Author(s):  
R Kandane-Rathnayake ◽  
J R Kent ◽  
W Louthrenoo ◽  
S -F Luo ◽  
Y -JJ Wu ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Renal Damage ◽  
Organ Damage ◽  
Systemic Lupus ◽  
Damage Accrual ◽  
Active Lupus Nephritis ◽  
Longitudinal Associations ◽  
Active Lupus

Objective To examine longitudinal associations of active lupus nephritis with organ damage accrual in patients with systemic lupus erythematosus (SLE). Methods This study was performed using data from a large multinational prospective cohort. Active lupus nephritis at any visit was defined by the presence of urinary casts, proteinuria, haematuria or pyuria, as indicated by the cut-offs in the SLE Disease Activity Index (SLEDAI)-2K, collected at each visit. Organ damage accrual was defined as a change of SLICC-ACR Damage Index (SDI) score >0 units between baseline and final annual visits. Renal damage accrual was defined if there was new damage recorded in renal SDI domains (estimated glomerular filtration rate <50%/proteinuria >3.5 g per 24 h/end-stage kidney disease). Time-dependent hazard regression analyses were used to examine the associations between active lupus nephritis and damage accrual. Results Patients ( N = 1735) were studied during 12,717 visits for a median (inter-quartile range) follow-up period of 795 (532, 1087) days. Forty per cent of patients had evidence of active lupus nephritis at least once during the study period, and active lupus nephritis was observed in 3030 (24%) visits. Forty-eight per cent of patients had organ damage at baseline and 14% accrued organ damage. Patients with active lupus nephritis were 52% more likely to accrue any organ damage compared with those without active lupus nephritis (adjusted hazard ratio = 1.52 (95% confidence interval (CI): 1.16, 1.97), p < 0.02). Active lupus nephritis was strongly associated with damage accrual in renal but not in non-renal organ domains (hazard ratios = 13.0 (95% CI: 6.58, 25.5) p < 0.001 and 0.96 (95% CI: 0.69, 1.32) p = 0.8, respectively). There was no effect of ethnicity on renal damage accrual, but Asian ethnicity was significantly associated with reduced non-renal damage accrual. Conclusion Active lupus nephritis measured using the SLEDAI-2K domain cut-offs is associated with renal, but not non-renal, damage accrual in SLE.

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