P0418LUPUS NEPHRITIS: A MONOCENTRIC STUDY

Abstract Background and Aims Systemic lupus erythematosus is a multi-visceral autoimmune disease. Renal involvement is one of the most common and serious manifestations of this disease. The histological lesions are highly polymorphic and the renal biopsy remains crucial for the therapeutic management of lupus nephritis (LN). The aim of our investigation was to study the epidemiological, clinical, biological and histological characteristics, outcomes and to evaluate the therapeutic protocols used for lupus nephritis’ treatment and to identify predictive factors of renal prognosis in patients with lupus nephritis. Method It was a retrospective study including patients over 16 years old with lupus nephritis proved by kidney biopsy and followed up over a period of 17 years in our department. Results We collected 155 women and 19 men with a sex ratio F / H of 8.2. The mean age at the time of the discovery of LN was 32.6 years with a maximum between 15 years and 45 years. The most frequent extra-renal manifestations were articular and dermatological manifestations (79%). Renal symptomatology was dominated by proteinuria noted in all patients, associated to a nephrotic syndrome in 68% of patients. At the time of diagnosis of LN, hematuria was present in 69% of patients and renal failure was present in half of cases. Immunologically, antinuclear antibody were positive in 89.1% of cases, anti DNA positive in 73.4% of cases, anti Sm positive in 79.8% of cases and Antiphospholipids were positive in 50% of cases, associated with an antiphospholipid syndrome in 14.9% of cases. We performed 243 renal biopsies with 174 initial and 69 iterative biopsies. The histological lesions were polymorphic dominated by LN class IV (36.6%) isolated or associated with LN class V (17.7%). All patients received a corticosteroid for induction or maintenance treatment. It was associated with immunosuppressive treatment according to different treatment regimens. The median duration of follow-up was 81.2 months. Renal outcome was marked by complete and sustained remission in 36.7% of cases, incomplete remission with chronic kidney disease in 34.5% of cases, chronic renal failure in 28.7% of cases. At univariate analysis, we identified the young age below 35 years at the time of the discovery of LN, the male sex, increased serum creatinine at the time of biopsy, proliferative forms, the presence of histological signs of chronicity and lesions of thrombotic microangiopathy as predictive factors of poor renal outcomes. Conclusion Lupus nephritis is one of the most common and serious manifestations of Systemic lupus erythematosus. The generalization of renal biopsy, the use of early codified therapeutic protocols and regular monitoring and evaluation of disease activity according to the appropriate scores can improve management and survival of patients with renal impairment.

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SAT0211 RENAL INJURY IN SYSTEMIC LUPUS ERYTHEMATOSUS CHARACTERIZED BY THROMBOTIC MICROANGIOPATHY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.1198 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1048.1-1048

Author(s):

W. Hu

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Renal Biopsy ◽

Lupus Erythematosus ◽

Thrombotic Microangiopathy ◽

Renal Injury ◽

Renal Outcome ◽

Pathological Examination ◽

Systemic Lupus ◽

Tubulointerstitial Lesions

Background:Classical lupus nephritis (LN) is characterized by glomerular immune complex(IC) deposition with glomerular proliferation, basement membrane destruction and cell infiltration. Non-IC mediated renal injury with thrombotic microangiopathy (TMA) was also reported in patients with systemic lupus erythematosus (SLE-renal TMA), but most studies were reported in patients with both LN and renal TMA.Objectives:In this study, clinical features and outcomes of SLE-renal TMA in absence of obvious IC in SLE patients were analyzed.Methods:Patients with glomerular TMA and/or vascular TMA in the absence of obvious subendothelial or epithelial immune deposits were screened out from 2332 biopsied in SLE patients who underwent first renal biopsy from January 2005 to August 2016. Their clinical, histological features and outcomes were retrospectively analyzed.Results:In 2332 renal biopsies obtained from SLE patients, 257 (11.0%) showed renal TMA, of which 237 showed both renal TMA and LN, and 20 biopsies had only renal TMA (SLE-renal TMA). There were 2 males and 18 females with an average age of (25 ± 10) years. The median course of SLE and LN were 3.0(1.0, 6.0) and 0.8(0.5, 1.9) months. All 20 patients deserved acute kidney injury, of which 11 (55%) needed renal replacement therapy (RRT) and 12 (60%) were nephrotic syndrome. Blood system involvement was found in all cases, including 13 cases (65.0%) with TMA triad (microvascular hemolytic anemia, thrombocytopenia and elevated lactate dehydrogenase).Pathological examination showed that 17 cases (85.0%) had both glomerular TMA and vascular TMA. Immunofluorescence and electron microscopy showed that 8 cases (40%) had no IC deposition in glomerulus and 12 cases (60%) had only IC deposition in mesangium. Acute tubulointerstitial lesions in patients requiring RRT were more serious than those no needing for RRT((43.6±24.9) %vs(21.7±20.1) %,P=0.047). The fusion range of foot process was positively correlated with proteinuria (r2= 0.347,P=0.006).All patients received high-dose methylprednisolone pulse therapy. Four patients received plasma exchange and three patients received gamma globulin, respectively. Eleven patients requiring RRT all stop RRT in a median time of 16.0 (9.0, 30.0) days. During a median follow-up of 58.0 (36.0, 92.3) months, complete remission (CR) was obtained in 15 cases, partial remission in 4 cases and no remission in 1 case. Six cases (30%) relapsed. No case died or progressed to end stage renal disease.Conclusion:Renal injury characterized by TMA is not uncommon in SLE renal biopsy cases. The clinical manifestation is special and the renal injury is serious. The renal outcome is good by intensive immunosuppressive therapy. It should be considered as a unique type of renal injury in SLE.References:[1]Moake JL. Thrombotic microangiopathies. N Engl J Med. 2002. 347(8): 589-600.[2]Anders HJ, Weening JJ. Kidney disease in lupus is not always ‘lupus nephritis’. Arthritis Res Ther. 2013. 15(2): 108.[3]Song D, Wu LH, Wang FM, et al. The spectrum of renal thrombotic microangiopathy in lupus nephritis. Arthritis Res Ther. 2013. 15(1): R12.[4]Hu WX, Liu ZZ, Chen HP, Zhang HT, Li LS, Liu ZH. Clinical characteristics and prognosis of diffuse proliferative lupus nephritis with thrombotic microangiopathy. Lupus. 2010. 19(14): 1591-8.[5]Tomov S, Lazarchick J, Self SE, Bruner ET, Budisavljevic MN. Kidney-limited thrombotic microangiopathy in patients with SLE treated with romiplostim. Lupus. 2013. 22(5): 504-9.[6]Li C, Yap D, Chan G, et al. Clinical Outcomes and Clinico-pathological Correlations in Lupus Nephritis with Kidney Biopsy Showing Thrombotic Microangiopathy. J Rheumatol. 2019 .[7]Chen MH, Chen MH, Chen WS, et al. Thrombotic microangiopathy in systemic lupus erythematosus: a cohort study in North Taiwan. Rheumatology (Oxford). 2011. 50(4): 768-75.[8]Park MH, AUID- Oho, Caselman N, Ulmer S, Weitz IC, AUID- Oho. Complement-mediated thrombotic microangiopathy associated with lupus nephritis. Blood Adv. 2018. 2(16): 2090-2094.Disclosure of Interests:None declared

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Clinical and immunological characteristics of 150 systemic lupus erythematosus patients in Jamaica: a comparative analysis

Lupus ◽

10.1177/0961203317707828 ◽

2017 ◽

Vol 26 (13) ◽

pp. 1448-1456 ◽

Cited By ~ 4

Author(s):

K C Maloney ◽

T S Ferguson ◽

H D Stewart ◽

A A Myers ◽

K De Ceulaer

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Disease Activity ◽

Renal Biopsy ◽

Diagnostic Criteria ◽

Lupus Erythematosus ◽

Antinuclear Antibodies ◽

Damage Index ◽

Systemic Lupus ◽

Dsdna Antibodies

Background Epidemiological studies in systemic lupus erythematosus have been reported in the literature in many countries and ethnic groups. Although systemic lupus erythematosus in Jamaica has been described in the past, there has not been a detailed evaluation of systemic lupus erythematosus patients in urban Jamaica, a largely Afro-Caribbean population. The goal of this study was to describe the clinical features, particularly disease activity, damage index and immunological features, of 150 systemic lupus erythematosus subjects. Methods 150 adult patients (≥18 years) followed in rheumatology clinic at a tertiary rheumatology hospital centre (one of two of the major public referral centres in Jamaica) and the private rheumatology offices in urban Jamaica who fulfilled Systemic Lupus International Collaborating Clinics (SLICC) criteria were included. Data were collected by detailed clinical interview and examination and laboratory investigations. Hence demographics, SLICC criteria, immunological profile, systemic lupus erythematosus disease activity index 2000 (SLEDAI-2K) and SLICC/American College of Rheumatology (ACR) damage index (SDI) were documented. Results Of the 150 patients, 145 (96.7%) were female and five (3.3%) were male. The mean age at systemic lupus erythematosus onset was 33.2 ± 10.9. Mean disease duration was 11.3 ± 8.6 years. The most prevalent clinical SLICC criteria were musculoskeletal, with 141 (94%) of subjects experiencing arthralgia/arthritis, followed by mucocutaneous manifestations of alopecia 103 (68.7%) and malar rash 46 (30.7%), discoid rash 45 (30%) and photosensitivity 40 (26.7%). Lupus nephritis (biopsy proven) occurred in 42 (28%) subjects and 25 (16.7%) met SLICC diagnostic criteria with only positive antinuclear antibodies/dsDNA antibodies and lupus nephritis on renal biopsy. The most common laboratory SLICC criteria were positive antinuclear antibodies 136 (90.7%) followed by anti-dsDNA antibodies 95 (63.3%) and low complement (C3) levels 38 (25.3%). Twenty-seven (18%) met SLICC diagnostic criteria with only positive antinuclear antibodies/anti-dsDNA antibodies and lupus nephritis on renal biopsy. Mean SLEDAI score was 6.9 ± 5.1 with a range of 0–32. Organ damage occurred in 129 (86%) patients; mean SDI was 2.4 ± 1.8, with a range of 0–9. Conclusion These results are similar to the clinical manifestations reported in other Afro-Caribbean populations; however, distinct differences exist with respect to organ involvement and damage, particularly with respect to renal involvement, which appears to be reduced in our participants.

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Do Asian patients have worse lupus?

Lupus ◽

10.1177/0961203310375832 ◽

2010 ◽

Vol 19 (12) ◽

pp. 1384-1390 ◽

Cited By ~ 37

Author(s):

MY Mok ◽

WL Li

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Renal Outcome ◽

Receptor Subtypes ◽

Systemic Lupus ◽

Asian Populations ◽

Asian Patients ◽

Geographical Regions ◽

White Patients

The predisposition to and clinical phenotype of systemic lupus erythematosus, an autoimmune disease that is associated with significant morbidity and mortality, are affected by genetic and environmental factors. This article aims to examine whether Asians have worse lupus by reviewing the literature on genetic predisposition and clinical outcomes, including major organ involvement, damage score and mortality in Asian populations compared with other ethnicities. A number of lupus nephritis susceptibility genes have been identified in Asians and White patients, with further variations among different Asian populations. Meta-analysis studies on various Fcγ receptor subtypes revealed that FcγRIIIA-F158 allele, which is associated with low binding affinity to IgG1 and IgG3, predisposed to lupus nephritis in Asian patients. Asian patients were reported to have higher rates of lupus nephritis-associated autoantibodies, lupus nephritis and more active glomerulonephritis compared with White patients. Renal outcome and the level of immunosuppressant use in Asians were comparable to Afro-American Blacks in some studies. Asians were also found to have higher overall damage scores compared with Whites. The difference in mortality between Asian patients and other ethnicities in different geographical regions was found to vary depending on socioeconomic factors such as access to health care. Poverty, education level, cultural and behavioural factors are confounders to ethnicity in determining clinical outcome of systemic lupus erythematosus.

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Clinical And Morphological Improvement Of Lupus Nephritis Treated With Rituximab

Folia Medica ◽

10.1515/folmed-2015-0003 ◽

2014 ◽

Vol 56 (4) ◽

pp. 245-252 ◽

Cited By ~ 2

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).

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POS0764 EULAR RECOMMENDATION-BASED QUALITY INDICATORS (QIS) FOR SYSTEMIC LUPUS ERYTHEMATOSUS (SLE): ELABORATION, FINAL SET, PERFORMANCE AND INITIAL VALIDATION

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3181 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 635.2-636

Author(s):

K. Chavatza ◽

M. Kostopoulou ◽

D. Nikolopoulos ◽

O. Gioti ◽

K. Togia ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Adverse Outcomes ◽

P Value ◽

Sustained Remission ◽

Systemic Lupus ◽

Eular Recommendations ◽

Work Up

Background:Targets of therapy and quality of care are receiving increased attention in systemic lupus erythematosus (SLE).Objectives:To develop Quality Indicators (QIs) for the care of SLE patients based on the EULAR recommendations, and assess their performance.Methods:Using the published EULAR recommendations for SLE, we developed 44 candidate QIs. These were independently rated for validity and feasibility by 12 experts, analysed by a modified RAND/UCLA model and further scrutinized based on the scorings and expert opinion. (Fig.1) Adherence to the final set of QIs was tested in a cohort of 220 SLE patients combined with an assessment on its impact on disease outcomes such as flares, hospitalizations and organ damage.Results:The panel rated 18 QIs as valid and feasible. These involve diagnosis; disease and damage assessment; monitoring for lupus nephritis and drug toxicity; therapy and targets of therapy; fertility and pregnancy; and adjunct therapy (preventive measures for osteoporosis, vaccination, cardiovascular disease). On average, SLE patients received 54% (95%CI 52–56%) of the indicated care with adherence ranging from 41% for QIs related to monitoring to 88% for treatment-related QIs. Regarding targets of therapy, sustained remission or low disease activity were achieved in 27%, while 94% of patients received low-dose glucocorticoids, and 92% the recommended hydroxychloroquine dose. Dependent upon individual QI tested, adherence for lupus nephritis-related QIs was 88% for receiving appropriate adjunct therapy (ACE inhibitors) to 100% for being treated with the indicated immunosuppressive treatment. In contrast, adherence to QIs related to preventive measures and other adjunct therapies was moderate to low. Notably, patients who were eligible for cardiovascular risk modification, vaccination, and osteoporosis management received lower quality of care (40.5%, 47.7% and 45.5% respectively) while 91.4% had sunscreen protection. In reference to laboratory work-up and monitoring, complete laboratory work-up at diagnosis was performed in 48%, while disease activity and damage, were fully assessed only in 14.1% (in three consecutive visits) and 28.6% (annually) respectively, Similarly, reproductive health and pregnancy counselling adherence rates were modest estimated at 50% and 62% respectively. Higher adherence to the indicated care during follow-up (monitoring QIs) was associated with reduced risk for adverse outcomes during the last year of observation (OR 0.97, 95%CI 0.96-0.99). Patients who achieved sustained remission or LLDAS, exhibited fewer flares (OR=0.15, p-value<0.001) and damage accrual (OR=0.35, p-value<0.001). Of interest, patients who received low-dose of GCs or were appropriately vaccinated, had a lower risk of experiencing a flare (OR=0.23 and 0.46 respectively).Conclusion:A set of 18 QIs based on the EULAR recommendations for SLE was developed to be used towards improving care in SLE. Initial real-life data suggest variable degree of adherence with higher adherence resulting in reduced adverse outcomes.References:[1]Fanouriakis, et al., 2019 Update of the EULAR recommendations for the management of systemic lupus erythematosus. In Annals of the Rheumatic Diseases (Vol. 78, Issue 6, pp. 736–745). BMJ Publishing Group. https://doi.org/10.1136/annrheumdis-2019-215089.[2]Nikolopoulos, D., et al., 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, 29(5), 514–522. https://doi.org/10.1177/0961203320908932.Acknowledgements:This project has received funding from the European Research Council (ERC) under the European Union’s Horizon 2020 research and innovation programme (grant agreement No 742390)Disclosure of Interests:None declared

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Renal outcome after kidney-transplantation in Korean patients with lupus nephritis

Lupus ◽

10.1177/0961203317725591 ◽

2017 ◽

Vol 27 (3) ◽

pp. 461-467 ◽

Cited By ~ 6

Author(s):

E S Park ◽

S S Ahn ◽

S M Jung ◽

J J Song ◽

Y-B Park ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Kidney Transplantation ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Graft Failure ◽

Renal Outcome ◽

Class Iii ◽

Systemic Lupus ◽

Renal Flare ◽

Class Iv

We investigated renal outcome of kidney-transplantation in 19 Korean recipients with biopsy-proven lupus nephritis and compared it with 18 Korean age- and gender-matched recipients without lupus nephritis who were diagnosed with end-stage renal disease caused by renal diseases other than lupus nephritis in a single centre. We reviewed histological findings of kidneys and calculated cumulative dose of immunosuppressive agents. We assessed renal flare of systemic lupus erythematosus, recurrence of lupus nephritis and graft failure as prognosis. The mean age of recipients with lupus nephritis was 43.5 years and all patients were female. Six patients had class III, 10 had class IV and three had class V. There were no meaningful differences in demographic data, renal replacement modality, cumulative doses of immunosuppressants and prognosis between recipients with and without lupus nephritis. Eight patients experienced renal flare of systemic lupus erythematosus, but there were no cases of recurrence of lupus nephritis or graft failure in recipients with lupus nephritis. Kidney-recipients with class IV lupus nephritis exhibited a lower cumulative renal flare of systemic lupus erythematosus free survival rate than those with class III lupus nephritis. In conclusion, renal outcome of kidney-transplantation in patients with lupus nephritis is similar to that in those without lupus nephritis, and class IV was associated with renal flare of systemic lupus erythematosus.

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Histopathological findings of renal biopsy in systemic lupus erythematosus

Journal of Patan Academy of Health Sciences ◽

10.3126/jpahs.v3i2.20268 ◽

2016 ◽

Vol 3 (2) ◽

pp. 10-14

Author(s):

Sanjit Karki ◽

Roshan Shrestha ◽

Buddhi Paudyal ◽

Bimal Pandey ◽

Nora Ranjitkar ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Renal Biopsy ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Definitive Treatment ◽

Class Iii ◽

Systemic Lupus ◽

Morphological Pattern ◽

Class Iv

Introductions: Classifying morphological pattern of renal involvement is important in systemic lupus erythematosus (SLE) for definitive treatment and prognosis. This study aims to analyse the histopathological pattern of glomerula in SLE patients.Methods: This was a retrospective chart review of patients diagnosed with SLE who had renal biopsy during October 2013 to September 2015 at Patan Hospital.Results: There were 38 patients of SLE. Antinuclear antibody (ANA) was positive in all 38 (100 %), Anti-dsDNA seen in 18 (47.4%). Active urinary sediment & proteinuria was seen in 25 (65.8%) patients and proteinuria in 13 (34.2%) patients. Histopathological patterns were of glomerular involvement, ISN Class II in 2 (5.3%), Class III in 2 (5.3%), class IV in 20 (52.5%), Class V in 6 (15.8%) and mixed IV-V in 8 (21.1%).Conclusions: The diffuse proliferative lupus nephritis (ISN Class IV) was the most common pattern of lupus nephritis encountered in our study followed by mixed pattern (ISN Class IV & V) and membranous lupus nephritis (ISN class IV). Journal of Patan Academy of Health Sciences. 2016 Dec;3(2):10-14

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O004. Assessment of the impact of lupus nephritis on clinical, biological parameters, disease activity and mortality in pediatric Systemic Lupus Erythematosus

Rheumatology ◽

10.1093/rheumatology/keab723.003 ◽

2021 ◽

Vol 60 (Supplement_5) ◽

Author(s):

O Gacem ◽

L Labboun ◽

N Ayad ◽

Z Zeroual ◽

Z Arrada ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Renal Failure ◽

Chronic Renal Failure ◽

Lupus Nephritis ◽

Disease Activity ◽

Lupus Erythematosus ◽

Systemic Lupus Erythematosus Disease ◽

Systemic Lupus ◽

Pediatric Systemic Lupus Erythematosus ◽

The Impact

Abstract Background Systemic lupus erythematosus (SLE) is an autoimmune disorder affecting multiple organ systems. Kidney involvement is one of the most frequent and severe manifestations of pediatric systemic lupus erythematosus (pSLE), seriously affecting the prognosis. It usually manifests as glomerulonephritis of varying severity. Objective: Knowledge of the correlation of lupus nephritis (LN) with clinical, biological, immunological parameters, disease activity and mortality in pediatric systemic lupus erythematosus is limited. This study aims to describe the impact of renal involvement with these different determinants. Methods This was a prospective, multicenter, descriptive 36-month study (January 2015 - December 2018) including patients less than 16 years of age with LN. The presence of LN was defined according to the American College of Rheumatology classification SLE criteria. The LN class was determined by renal biopsy and was classified according to the Morphology in Kidney International Society of Nephrology (ISN)/Renal Pathology Society (RPS) 2004 classification of lupus nephritis. The disease activity was estimated by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), the use of which has been validated in children. Means, percentages and Chi-square tests were specified. P values less than 0.05 were considered statistically significant. Results We included 83 patients in this study. 37/83 patients (44.6%) developed LN with the following urinary signs: 92% of proteinuria (mean 3366.147 mg ± 2785.93 / 24h) including 2/3 of cases of nephrotic syndrome, 81% of hematuria, 14% of acute renal failure with significant reduction in glomerular filtration rate (average creatinine clearance of 32.42 ml / min) and 12% high blood pressure. Out of a total of 30 renal biopsies interpreted at disease diagnosis, 73.4% diffuse proliferative glomerulonephritis forms were observed: (III (30%), IV (36.7%) and VI (6, 7%)). Lupus nephritis were significantly correlated with hypocomplementemia in its C3 (P = 0.00002) and C4 (P = 0.00005) fraction, lymphopenia (P = 0.02), anti-DNA antibodies (P = 0.026), SLEDAI (P = 0.00001) and mortality (P = 0.03). The most frequently used induction drugs for LN classes III, IV and VI were pulsed intravenous methylprednisolone (500 mg daily for 3 doses) in combination with low dose intravenous cyclophosphamide (23%) in the short term (500 mg/m2/15 days X 6) followed by mycophenolate mofetil (28%) (600mg/ m2 in two daily doses) as maintenance treatment associated with a daily dose of oral glucocorticoids with a gradual decrease until reaching the minimum amount necessary to control the disease. All of our SLE patients with nephritis were treated with HCQ with a significant correlation with the decrease in SLEDAI. During the first two years of disease progression, the frequency of LN increased to 43/83 (51.8%) mainly in these severe forms: (IV (41.7%), V (2.8 %). The progression to chronic renal failure had a prevalence of 6, 9% (3/43) of cases; these were mainly patients with severe lupus nephritis (III, and IV) Conclusion Nephritis is a major risk factor for morbidity and mortality in pSLE; LN in children is most often proliferating and more active. The early diagnosis and management of kidney damage are the only guarantee of a good course and prevention of the progression of chronic renal failure. Keywords lupus nephritis; child; systemic lupus erythematosus; disease activity, mortality.

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Particularities of the Evolution and Therapeutic Response in Men with Systemic Lupus Erythematosus and Lupus Nephritis

Internal Medicine ◽

10.2478/inmed-2019-0089 ◽

2019 ◽

Vol 16 (6) ◽

pp. 7-17

Author(s):

Anna Mirela Stroie ◽

Mircea Niculae Penescu ◽

Nicoleta Petre ◽

Geanina Beldea

Keyword(s):

Systemic Lupus Erythematosus ◽

Side Effects ◽

Lupus Nephritis ◽

Renal Biopsy ◽

Lupus Erythematosus ◽

Therapeutic Response ◽

Immunosuppressive Agents ◽

Systemic Lupus ◽

Male Patients ◽

Class Iv

AbstractAim. The prevalence ratio of systemic lupus erythematosus in men compared to women is 1:10, with more aggressive forms in men and in children. The incidence of lupus nephritis is of 50-60% in patients with lupus.In this paper, we aim to report on a series of cases that include male patients who had lupus nephritis via renal biopsy and were admitted between January 2011 - December 2017, with or without other SLE manifestations. The aim is to review the particularities and the therapeutic response: age at onset, disease duration, lupus nephritis class, extra-renal organ involvement of lupus disease, paraclinical findings – proteinuria, inflammatory syndrome, renal function, therapeutic response – immunosuppressive regimens used for induction and maintenance, remission onset, remission type, number of flares, side effects of immunosuppressive therapy.Materials and method. We have reviewed the histopathology database of male patients with lupus nephritis revealed via renal biopsy, medical charts and the Hippocrates information system in order to collect patient data.Outcomes. Out of 68 renal biopsies positive for lupus nephritis, 9 were from male patients, which reveals a 6.5:1 ratio. The average age at the time of the renal biopsy is 37. 33 years old. Lupus nephritis class - 8 out of 9 patients were class IV, 1 sample was class IV+V. The duration of the corticosteroid therapy is 6.6 years. In addition, we also reviewed the immunosuppressive agents used, the number of flares, and the side effects of the medication.Conclusions. Our data are similar to the literature data.

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Lupus Nephritis: Prognostic Factors in Children

PEDIATRICS ◽

10.1542/peds.89.2.240 ◽

1992 ◽

Vol 89 (2) ◽

pp. 240-246

Author(s):

Deborah K. McCurdy ◽

Thomas J. A. Lehman ◽

Bram Bernstein ◽

Virgil Hanson ◽

Karen K. King ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Renal Failure ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Elevated Serum ◽

Chronic Dialysis ◽

World Health ◽

Treatment Modalities ◽

Glomerular Sclerosis ◽

Systemic Lupus

As newer treatment modalities become available for patients with severe lupus nephritis, it becomes increasingly important to identify patients at risk for renal failure. In this study, the records of 90 children presenting with systemic lupus erythematosus over a 13-year period were reviewed. Nineteen were lost to follow-up prior to completion of the study. Of the 71 remaining children, 16 (22%) progressed to chronic renal failure. Persistent hypertension lasting greater than 4 months, anemia, abnormalities of the urinalysis, and elevated serum creatinine level were significantly associated with progression to renal failure. Sex, race, age, abnormalities of creatinine clearance, and 24-hour urine protein collection were not associated with progression to renal failure. Renal biopsies were obtained in 45 children. Biopsies were initially classified according to World Health Organization criteria. Diffuse proliferative glomerulonephritis was significantly associated with progression to renal failure. The 45 biopsies available were reviewed by one of the authors and categorized by activity and chronicity indices. Both the active lesions of fibrinoid necrosis, synechiae, tubular casts, and vasculitic lesions and the chronic lesion of glomerular sclerosis correlated with progression to renal failure. Of the 16 children who progressed to renal failure, 2 had cadaver kidney transplants and are well 5 years posttransplant; 4 had fulminant lupus and died within 1 month of commencing dialysis; 10 began chronic dialysis. Five of the 10 children on chronic dialysis died from sepsis. These data suggest that children with systemic lupus erythematosus who undergo dialysis do poorly. This alters the risk-to-benefit ratio of newer, more aggressive treatment protocols which should be considered in children whose clinical, laboratory, and renal biopsy findings suggest eventual progression to renal failure.

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