scholarly journals Treatment outcomes in refractory lupus nephritis: Data from an observational study

Lupus ◽  
2021 ◽  
pp. 096120332110339
Author(s):  
Subodh Gururani ◽  
Phani Kumar Devarasetti ◽  
Megha Uppin ◽  
Liza Rajasekhar
Keyword(s):  
Lupus Nephritis ◽  
Serum Creatinine ◽  
Treatment Outcomes ◽  
Univariate Analysis ◽  
Response To Therapy ◽  
Outcome Analysis ◽  
Class Iii ◽  
Renal Response ◽  
Significant Difference

Objectives Despite current advances in treatment, refractory lupus nephritis (RLN) continues to pose a challenge. The present paper studies the clinical profile and treatment outcomes in patients with RLN. Methods This observational, bidirectional study enrolled consecutive lupus nephritis (LN) patients from August 2018 to January 2019, who either failed to improve within three months, did not achieve partial renal response (PR) at six months, or did not achieve complete renal response (CR) after two years of treatment. Patients were followed every three months; treatment details and outcomes [CR, PR, no renal response (NR)], doubling serum creatinine, and death were recorded. Group comparisons were made using ANOVA and chi-square test. Factors affecting renal response were studied using linear regression. Results Forty-five of forty-eight enrolled patients completed at least nine months of follow-up and were included in outcome analysis. The median (IQR) SLE duration was three years (2-6 years). The majority of patients (n = 25) had proliferative LN (ISN/RPS class III/IV), with nine patients having pure membranous LN (class V). The mean activity and chronicity indices were 8 and 0. Over a median (IQR) follow-up period of 15 (12-27) months, 28 had CR, 9 had PR, and 8 showed no response to a switch in an immunosuppressive (IS) agent. Repeat renal biopsy (n = 8) with a mean (±SD) biopsy interval of 2 (±1) years showed histological class transformation in more than half of the patients. There was no significant difference in treatment outcome and time to attain response based on individual IS agent or sequence of IS agents used. None of the variables (duration of SLE or nephritis, baseline SLEDAI, leukopenia, hypertension, elevated anti-dsDNA, low complements, serum albumin, 24-hour urinary protein, biopsy class) predicted renal response on univariate analysis. No patient had a doubling of serum creatinine or progression to end-stage renal disease. There were three deaths, all related to infection. Conclusion A change in immunosuppression produces response in most RLN patients while a fifth of them showed no response to therapy. No predictor of renal response was identified. Histologic class switch was frequent. Renal function did not decline over a year of follow-up.

scholarly journals Treatment Outcomes in Refractory Lupus Nephritis: Data From an Observational Study

2021 ◽  
Author(s):  
Subodh Gururani ◽  
Phani Kumar Devarasetti ◽  
Megha Uppin ◽  
Liza Rajasekhar
Keyword(s):  
Lupus Nephritis ◽  
Serum Creatinine ◽  
Treatment Outcomes ◽  
Univariate Analysis ◽  
Response To Therapy ◽  
Outcome Analysis ◽  
Class Iii ◽  
Renal Response ◽  
Significant Difference

Abstract Objectives Despite current advances in treatment, refractory lupus nephritis (RLN) continues to pose a challenge. The present paper studies the clinical profile and treatment outcomes in patients with RLN. Methods This was an observational, bidirectional study enrolling consecutive patients of lupus nephritis from August 2018 to January 2019, who either failed to improve within three months, did not achieve partial renal response (PR) at six months, or did not achieve complete renal response (CR) after two years of treatment. Patients were followed every three months; treatment details and outcomes [CR, PR, no renal response (NR)], doubling serum creatinine, and death were recorded. Group comparisons were made using ANOVA and chi-square test. Factors affecting renal response were studied using linear regression. Results Forty-five of forty-eight enrolled patients completed at least nine months of follow-up and were included in outcome analysis. The median (IQR) SLE duration was three years (2–6 years). The majority of patients (n = 25) had proliferative lupus nephritis (LN) (ISN/RPS class III/IV), with nine patients having pure membranous LN (class V). The mean (SD) activity and chronicity indices were 8 (3) and 0. Over a median (IQR) follow-up period of 15 (12–27) months, 28 had CR, 9 had PR, and 8 showed no response to a switch in an immunosuppressive agent. Repeat renal biopsy (n = 8) with a mean (± SD) biopsy interval of 2 (± 1) years showed histological class transformation in more than half of the patients. There was no significant difference in treatment outcome and time to attain response based on individual immunosuppressive (IS) agent or sequence of IS agents used. None of the variables (duration of SLE or nephritis, baseline SLEDAI, leukopenia, hypertension, elevated anti-dsDNA, low complements, serum albumin, 24-hour urinary protein, biopsy class) predicted renal response on univariate analysis. No patient had a doubling of serum creatinine or progression to end-stage renal disease. There were three deaths, all related to infection. Conclusion The present study shows that a change in immunosuppression produces response in most RLN patients while a fifth of them showed no response to therapy. No predictor of renal response was identified. Histologic class switch was frequent. Renal function did not decline over a year of follow-up. Mortality was the direct cause of infection.

scholarly journals P0266PREDICTIVE FACTORS OF COMPLETE RENAL RESPONSE IN PROLIFERATIVE LUPUS NEPHRITIS: A COLLABORATIVE STUDY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Beatriz Sanchez Alamo ◽  
Clara Maria Cases Corona ◽  
Serena Gatius ◽  
Patricia Dominguez Torres ◽  
Elena Valdes ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Lupus Nephritis ◽  
Kidney Biopsy ◽  
Univariate Analysis ◽  
Immunosuppressive Treatment ◽  
Collaborative Study ◽  
Class Iii ◽  
Renal Response ◽  
Proliferative Lupus Nephritis

Abstract Background and Aims Proliferative lupus nephritis (class III and IV) is the most severe form of lupus nephritis and requires prompt diagnosis and treatment with immunosuppressive therapy. Since it represents the most serious entity and has the greatest functional consequences there is a need to determine which factors in proliferative lupus nephritis are most predictive of good long-term renal function. Method Methods We analysed the data of 49 biopsy-proven proliferative lupus nephritis (18,4% class III and 81,6% class IV) of three different Spanish hospitals to find prognostic factors for complete renal response (CRR), defined as loss of <25% of eGFR and the absence of proteinuria and microhematuria at the end of the follow up. Sociodemographic, clinical, laboratory, and treatment-related data at the time of kidney biopsy and during follow-up were obtained. We performed univariate analysis and logistic regression to identify independent risk factors. Results The median follow-up was 8 years (IQR: 3-12,5), during which time 18 patients (36,7 %) achieved CRR. In the univariate analysis complete renal response was related to: (1) at the diagnosis to: age [40,52 (11,29) years vs 29,92 (11,93) p=0,004]; (2) in kidney biopsy to less leukocyte infiltration (42,3% p=0,05); (3) during the follow up to: less comorbities (27,8% vs 64,3% p=0,02), less infections (27,8% vs 58,6% p=0,04) and less hospitalizations due to infections (0% vs 33,3% p=0,010), less prevalence of high BP (22,2% vs 60,7% p=0,01), (4) at the end of follow up to : serum albumin [3,97 (0,59) vs 4,31(0,19) mg/dL p=0,03]. In the logistic regression comorbidies (HR : 5,71 95%IC: 1,56-20,93 p=0,008) and age at the moment of diagnosis (HR : 1,046 95%IC:1,001-1,071 p=0,04) were related to complete renal response. We didn´t find any differences concerning treatments. Conclusion Proliferative lupus nephritis is one of the most severe manifestations of lupus nephritis, resulting in increased morbidity and mortality. Traditionally it has been thought that older patients have a worst prognosis, however we demonstrated that they achieved more frequently CRR. In the management of the patients traditional reno protective measures like strict control of BP must be considered since it is a predictive factor of CRR. We shouldn´t forget about the implications of an aggressive immunosuppressive treatment such as hospitalizations due to infections and comorbities.

scholarly journals Baseline HDAC6 and TFF3 proteins overexpression as prognostic markers of lupus nephritis relapse

2020 ◽  
Vol 9 (2) ◽  
pp. e14-e14
Author(s):  
Eman Hassan Abdelbary ◽  
Noha Farouk Ahmed ◽  
Adel Abdelmohsen Ghorab
Keyword(s):  
Lupus Nephritis ◽  
Serum Creatinine ◽  
Lupus Erythematosus ◽  
Kidney Diseases ◽  
Activity Index ◽  
Kidney Injury ◽  
Class Iii ◽  
Clinicopathologic Characteristics ◽  
Chronicity Index

Introduction: Lupus nephritis (LN) is a substantial manifestation of systemic lupus erythematosus (SLE). HDAC6 is overexpressed in various kidney diseases, and its inhibition slows kidney injury progression. Urinary TFF3 increases in chronic kidney diseases (CKDs) and may be associated with patient’s outcome. Objectives: This study aimed to examine the relationship between renal HDAC6 and TFF3 proteins expression and with clinicopathologic characteristics and outcome of LN. Patients and Methods: HDAC6 and TFF3 proteins’ expression was immunohistochemically detected in 56 cases of LN. They were correlated to patients’ age, gender, urinary 24 hours protein and serum creatinine levels at baseline and during follow up. Additionally, they were correlated to LN classes, activity index (AI) and chronicity index (CI) and relapse free survival (RFS). Results: HDAC6 overexpression was significantly associated with serum creatinine and 24 hours proteinuria levels at baseline (P = 0.041 and P =0.026 respectively) and during follow up (P < 0.001). It was associated with AI and CI of class III and IV LN (P = 0.047 and 0.003 respectively). TFF3 overexpression was associated with higher serum creatinine and more proteinuria at baseline (P = 0.015 and 0.001 respectively) and during follow up (P < 0.001). It was significantly associated with higher CI (P = 0.001). Both markers were associated with shorter RFS (P < 0.001). Conclusion: HDAC6 and TFF3 proteins are associated with clinicopathologic features of renal damage in LN. They are reliable predictors of patients’ RFS, which makes them good candidates for risk stratification of patients and targeted therapy.

P0451TEN-YEAR OUTCOME DIFFERENCES IN LUPUS NEPHRITIS PATIENTS TREATED WITH CYCLOPHOSHAMIDE AND MYCOPHENOLATE- BASED TREATMENT REGIMEN

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Narayan Prasad ◽  
Jithu Kurian ◽  
Vikas Agarwal ◽  
Dharmendra Bhadauria ◽  
Amit Gupta
Keyword(s):  
Lupus Nephritis ◽  
Serum Creatinine ◽  
Cause Of Death ◽  
Patient Survival ◽  
Class Iii ◽  
Renal Survival ◽  
Common Cause ◽  
Class Iv

Abstract Background and Aims Lupus nephritis (LN) poses a considerable impact on the morbidity and mortality of SLE patients. Long term comparative outcome data with cyclophosphamide (CYP) and mycophenolate mofetil (MMF) based regimen from the Indian subcontinent is sparse. We assessed the renal and patient survival of these patients for the types of induction CYP or MMF and the two maintenance therapies – MMF or Azathioprine. We determined the predictors of death and dialysis dependency in the study population. Method In this retrospective study, we analysed outcomes of 100 LN patients, total 67 (26 class III, 25 class IV, 6 class III+V, and 10 class IV+V)) treated with CYP (euro lupus-40 and NIH- Dharmendra Bhadauria 27), and 33 with MMF based regimen with the steroid between July 2008 to June 2018. The class distribution of the patients in the two groups was similar. The data were archived regarding demography, clinical, histopathological features, and the treatment given of all 100 biopsy-proven LN patients. Outcomes between two regimens CYP and MMF in terms of remission, dialysis dependency, and patient survival were compared. The renal survival and patient survival at the end of follow-up between two groups were also analysed. Results The clinical characteristics were similar in both groups, except the activity index was high in CYP patients (6.13 ±4.48 Vs. 4.61 ± 2.80); however, the chronicity index was similar. The overall remission was 70% at the end of induction. The CR, PR, and NR in the CYP group was 46.2%, 23.9 %, 29.9% respectively; however, in the MMF group was 57.6%, 12.1%, and 30.3%, respectively. More patients died in CYP (14.9%) than those in MMF (9.1 %) patients. The 1-, 2-, 3-, 4-, 5- and 10-years patient survival in the CYP induction was 89.5%, 86.2%, 86.2%,83.8%, 83.8% and 83.8% however in MMF was 93.9%, 93.9%, 89%, 89%, 89% and 89% respectively. The most common cause of death was sepsis 9/13(69.2%), followed by uremia. The high serum creatinine, low Hb, male, thrombocytopenia, microscopic haematuria, leukocyturia, nephrotic proteinuria, lack of remission in 12 months, dialysis, doubling of creatinine on follow-up were significant predictors of mortality. The 1-, 2- 3-, 4-, 5- and 10- years renal survival (event death-censored, but dialysis dependency) in CP group was 98.5%, 96.7%, 94.7%, 92.4%, 92.4% and 84 % respectively however in the MMF was 96.8 %, 96.8%, 91.9%, 91.9%, 91.9%, and 78.8% respectively. (Figure 1)At the end of the study, dialysis dependency in the MMF group and CYP group was 7.5% and 12.1 %, respectively (NS). In the maintenance therapy, 3/56(5.3%) had to double of creatinine in MMF, and 7/34 (20.5%) in the AZA group (p=0.03). Conclusion Long term outcomes in terms of patient and renal survival of LN patients treated with CP and MMF based induction is similar. Serum creatinine doubling was more with MMF than AZA based maintenance. The majority of death occurred during induction, and sepsis was the most common cause of death.

scholarly journals POS0693 EFFICACY AND SAFETY OF BELIMUMAB IN PATIENTS WITH LUPUS NEPHRITIS IN REAL-LIFE SETTING: RESULTS FROM A LARGE, NATIONWIDE, MULTICENTRIC, PROSPECTIVE COHORT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 594-595
Author(s):  
F. Saccon ◽  
M. Gatto ◽  
M. Zen ◽  
M. Fredi ◽  
F. Regola ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Independent Predictor ◽  
Rescue Therapy ◽  
Real Life ◽  
Multivariate Logistic Regression Analysis ◽  
Class Iii ◽  
Baseline Serum ◽  
Renal Response ◽  
Real Life Setting

Background:LN is still a severe manifestation of Systemic lupus erythematosus (SLE) and multitarget therapy is needed to control the disease especially in refractory cases.Objectives:To evaluate renal response in SLE patients with glomerulonephritis (GN) treated with Belimumab in real-life setting.Methods:Patients with proteinuria >0.5 g/24 h and/or active sediment at baseline enrolled in a multicentre Italian cohort of SLE patients (BeRLiSS study), treated with monthly iv Belimumab 10 mg/kg plus standard of care were considered in this study. Complete renal response (CRR) was defined as proteinuria <0.5 g/24 h, estimated glomerular filtration rate (eGFR)≥90ml/min/1.73m2 and no rescue therapy. Primary efficacy renal response (PERR) was defined as proteinuria ≤0.7 g/24 h, eGFR ≥60ml/min/1.73m2 and no rescue therapy. Prevalence and predictive factors of CRR and PERR at 12 and 24 months after Belimumab initiation were analyzed by multivariate logistic regression analysis.Results:A total of 91 patients were considered in this study, 79 female, mean age 40.51±9.03 years, mean disease duration 12.18±8.15 years, median follow-up time after Belimumab initiation 22 months. Twenty patients had baseline proteinuria ≥0.5 <1 g/day, 17 ≥1 <2 g/day, 13 ≥2 g/day. Belimumab was started at GN onset in 20 (22%) patients and at the time of a renal flare in all other cases. Seventy-five patients underwent a renal biopsy: 1 class I, 4 class II, 14 class III, 47 class IV and 9 class V. Baseline serum creatinine was 82.44±29.26 umol/L; 15 patients showed eGFR<60ml/min/1.73m2 at baseline. Immunosuppresants were taken by 70 (76.9%) patients: 47 micofenolate, 15 azathioprine and 5 ciclosporine. Sixty patients (65.9%) were on antimalarials. During follow-up 34 (37.4%) patients achieved CRR. Among them 5 (14.7%) patients relapsed and 29 (85.3%) patients maintained remission. Mean time to achieved CRR was 9.71±5.91 months.High levels of baseline proteinuria were a negative independent predictor of CRR and PERR at 6 months (OR 0.044 CI95% 0.006-0.320 p=0.002 and OR 0.232 CI95% 0.091-0.596 p=0.002) and 12 months (OR 0.029 CI95% 0.002-0.556 p=0.019 and OR 0.056 CI95% 0.009-0.327 p=0.001). High levels of baseline creatinine were a negative independent predictor of renal response. Renal response at 6 months was a strong predictive factor of renal response at 12 and 24 months.Conclusion:Belimumab is an effective add-on therapy in the treatment of GN in real-life practice setting.Disclosure of Interests:None declared

scholarly journals FRI0181 ORGAN DAMAGE FREE SURVIVAL IN SOUTHERN CHINESE PATIENTS WITH ACTIVE LUPUS NEPHRITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 674.1-674
Author(s):  
C. C. Mok ◽  
C. S. Sin ◽  
K. C. Hau ◽  
T. H. Kwan
Keyword(s):  
Lupus Nephritis ◽  
Regression Model ◽  
Cox Regression ◽  
Organ Damage ◽  
Renal Survival ◽  
Cox Regression Model ◽  
Free Survival ◽  
Renal Response

Background:The goals of treatment of lupus nephritis (LN) are to induce remission, retard the progression of chronic kidney disease, prevent organ complications and ultimately reduce mortality. Previous cohort studies of LN have mainly focused on the risk of mortality and development of end stage renal failure (ESRF) (renal survival). The cumulative frequency of LN patients who survive without organ damage, which correlates better with the balance between treatment efficacy and toxicity, as well as quality of life, has not been well studied.Objectives:To study the organ damage free survival and its predictive factors in patients with active LN.Methods:Consecutive patients who fulfilled ≥4 ACR/SLICC criteria for SLE and with biopsy proven active LN between 2003 and 2018 were retrospectivey analyzed. Those with organ damage before LN onset were excluded. Data on renal parameters and treatment regimens were collected. Complete renal response (CR) was defined as normalization of serum creatinine (SCr), urine P/Cr (uPCR) <0.5 and inactive urinary sediments. Partial renal response (PR) was defined as ≥50% reduction in uPCR and <25% increase in SCr. Organ damage of SLE was assessed by the ACR/SLICC damage index (SDI). The cumulative risk of having any organ damage or mortality since LN was studied by Kaplan-Meier’s analysis. Factors associated with a poor outcome were studied by a forward stepwise Cox regression model, with entry of covariates with p<0.05 and removal with p>0.10.Results:273 LN patients were identified but 64 were excluded (organ damage before LN onset). 211 LN patients were studied (92% women; age at SLE 30.4±13.5 years; SLE duration at LN 1.9±3.1years). 47 (22%) patients had nephrotic syndrome and 60 (29%) were hypertensive. Histological LN classes was: III/IV±V (75.1%), I/II (7.8%) and pure V (17.1%) (histologic activity and chronicity score 7.0±4.2 and 1.8±1.5, respectively). Induction regimens were: prednisolone (33.1±17.5mg/day) in combination with intravenous cyclophosphamide (CYC) (21.4%; 1.0±0.2g per pulse), oral CYC (8.6%; 96.4±37.8mg/day), azathioprine (AZA) (14.3%; 78.6±25.2mg/day), mycophenolate mofetil (MMF) (22.8%; 1.9±0.43g/day) and tacrolimus (TAC) (17.1%; 4.3±1.1mg/day). After a follow-up of 8.6±5.4 years, 94(45%) patient developed organ damage (SDI≥1) and 21(10%) patients died. The commonest organ damage was renal (36.3%) and musculoskeletal (17.9%), and the causes of death were: infection (38.1%), malignancy (19.0%), cardiovascular events (9.5%) and ESRF complications (9.5%). At last visit, 114 (55%) patients survived without any organ damage. The cumulative organ damage free survival at 5, 10 and 15 years after renal biopsy was 73.5%, 59.6% and 48.3%, respectively. The 5, 10 and 15-year renal survival rate were 95.2%, 92.0% and 84.1% respectively. In a Cox regression model, nephritic relapse (HR 3.72[1.78-7.77]), proteinuric relapse (HR 2.30[1.07-4.95]) and older age (HR 1.89[1.05-3.37]) were associated with either organ damage or mortality, whereas CR (HR 0.25[0.12-0.50]) at month 12 were associated with organ damage free survival. Baseline SCr, uPCR and histological LN classes were not significantly associated with a poor outcome. Among patients with class III/IV LN, the long-term organ damage free survival were not significantly different in users of MMF (reference) from CYC (IV/oral) (HR 1.45[0.76- 2.75]) or TAC (HR 1.03[0.26-1.62]) as induction therapy.Conclusion:Organ damage free survival is achieved in 55% of patients with active LN upon 9 years of follow-up. CYC/MMF/TAC based induction regimens did not differ for the long-term outcome of LN. Targeting complete renal response and preventing renal relapses remain important goals of LN treatment.Acknowledgments:NILDisclosure of Interests:None declared

scholarly journals POS1189 THE IMPACT OF TELEMEDICINE AND COVID-19 ON A TERTIARY RHEUMATOLOGY SERVICE: A RETROSPECTIVE AUDIT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 876-877
Author(s):  
W. Zhu ◽  
T. De Silva ◽  
L. Eades ◽  
S. Morton ◽  
S. Ayoub ◽  
...  
Keyword(s):  
Immunosuppressive Therapy ◽  
Univariate Analysis ◽  
Diagnostic Delay ◽  
Cohort Differences ◽  
Phone Calls ◽  
Retrospective Audit ◽  
Common Diagnosis ◽  
Significant Difference ◽  
The Impact

Background:Telemedicine was widely utilised to complement face-to-face (F2F) care in 2020 during the COVID-19 pandemic, but the impact of this on patient care is poorly understood.Objectives:To investigate the impact of telemedicine during COVID-19 on outpatient rheumatology services.Methods:We retrospectively audited patient electronic medical records from rheumatology outpatient clinics in an urban tertiary rheumatology centre between April-May 2020 (telemedicine cohort) and April-May 2019 (comparator cohort). Differences in age, sex, primary diagnosis, medications, and proportion of new/review appointments were assessed using Mann-Whitney U and Chi-square tests. Univariate analysis was used to estimate associations between telemedicine usage and the ability to assign a diagnosis in patients without a prior rheumatological diagnosis, the frequency of changes to immunosuppression, subsequent F2F review, planned admissions or procedures, follow-up phone calls, and time to next appointment.Results:3,040 outpatient appointments were audited: 1,443 from 2019 and 1,597 from 2020. There was no statistically significant difference in the age, sex, proportion of new/review appointments, or frequency of immunosuppression use between the cohorts. Inflammatory arthritis (IA) was a more common diagnosis in the 2020 cohort (35.1% vs 31%, p=0.024). 96.7% (n=1,444) of patients seen in the 2020 cohort were reviewed via telemedicine. In patients without an existing rheumatological diagnosis, the odds of making a diagnosis at the appointment were significantly lower in 2020 (28.6% vs 57.4%; OR 0.30 [95% CI 0.16-0.53]; p<0.001). Clinicians were also less likely to change immunosuppressive therapy in 2020 (22.6% vs 27.4%; OR 0.78 [95% CI 0.65-0.92]; p=0.004). This was mostly driven by less de-escalation in therapy (10% vs 12.6%; OR 0.75 [95% CI 0.59-0.95]; p=0.019) as there was no statistically significant difference in the escalation or switching of immunosuppressive therapies. There was no significant difference in frequency of follow-up phone calls, however, patients seen in 2020 required earlier follow-up appointments (p<0.001). There was also no difference in unplanned rheumatological presentations but significantly fewer planned admissions and procedures in 2020 (1% vs 2.6%, p=0.002). Appointment non-attendance reduced in 2020 to 6.5% from 10.9% in 2019 (OR 0.57 [95% CI 0.44-0.74]; p<0.001), however the odds of discharging a patient from care were significantly lower in 2020 (3.9% vs 6%; OR 0.64 [95% CI 0.46-0.89]; p=0.008), although there was no significance when patients who failed to attend were excluded. Amongst patients seen via telemedicine in 2020, a subsequent F2F appointment was required in 9.4%. The predictors of needing a F2F review were being a new patient (OR 6.28 [95% CI 4.10-9.64]; p<0.001), not having a prior rheumatological diagnosis (OR 18.43 [95% CI: 2.35-144.63]; p=0.006), or having a diagnosis of IA (OR 2.85 [95% CI: 1.40-5.80]; p=0.004) or connective tissue disease (OR 3.22 [95% CI: 1.11-9.32]; p=0.031).Conclusion:Most patients in the 2020 cohort were seen via telemedicine. Telemedicine use during the COVID-19 pandemic was associated with reduced clinic non-attendance, but with diagnostic delay, reduced likelihood of changing existing immunosuppressive therapy, earlier requirement for review, and lower likelihood of discharge. While the effects of telemedicine cannot be differentiated from changes in practice related to other aspects of the pandemic, they suggest that telemedicine may have a negative impact on the timeliness of management of rheumatology patients.Disclosure of Interests:None declared.

scholarly journals Eculizumab discontinuation in atypical hemolytic uremic syndrome: TMA recurrence risk and renal outcomes

2021 ◽  
Author(s):  
Gema Ariceta ◽  
Fadi Fakhouri ◽  
Lisa Sartz ◽  
Benjamin Miller ◽  
Vasilis Nikolaou ◽  
...  
Keyword(s):  
Family History ◽  
Hemolytic Uremic Syndrome ◽  
Univariate Analysis ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Renal Response ◽  
Pathogenic Variants ◽  
Increased Risk ◽  
History Of ◽  
Uremic Syndrome

ABSTRACT Background Eculizumab modifies the course of disease in patients with atypical hemolytic uremic syndrome (aHUS), but data evaluating whether eculizumab discontinuation is safe are limited. Methods Patients enrolled in the Global aHUS Registry who received ≥1 month of eculizumab before discontinuing, demonstrated hematologic or renal response prior to discontinuation and had ≥6 months of follow-up were analyzed. The primary endpoint was the proportion of patients suffering thrombotic microangiopathy (TMA) recurrence after eculizumab discontinuation. Additional endpoints included: eGFR changes following eculizumab discontinuation to last available follow-up; number of TMA recurrences; time to TMA recurrence; proportion of patients restarting eculizumab; and changes in renal function. Results We analyzed 151 patients with clinically diagnosed aHUS who had evidence of hematologic or renal response to eculizumab, before discontinuing. Thirty-three (22%) experienced a TMA recurrence. Univariate analysis revealed that patients with an increased risk of TMA recurrence after discontinuing eculizumab were those with a history of extrarenal manifestations prior to initiating eculizumab, pathogenic variants, or a family history of aHUS. Multivariate analysis showed an increased risk of TMA recurrence in patients with pathogenic variants and a family history of aHUS. Twelve (8%) patients progressed to end-stage renal disease after eculizumab discontinuation; 7 (5%) patients eventually received a kidney transplant. Forty (27%) patients experienced an extrarenal manifestation of aHUS after eculizumab discontinuation. Conclusions Eculizumab discontinuation in patients with aHUS is not without risk, potentially leading to TMA recurrence and renal failure. A thorough assessment of risk factors prior to the decision to discontinue eculizumab is essential.

MO661COMPARISON OF CLINICAL EFFICACY OF CENTRIFUGAL-MEMBRANOUS DOUBLE FILTRATION PLASMAPHERESIS AND MEMBRANOUS DOUBLE FILTRATION PLASMAPHERESIS ON SEVERE LUPUS NEPHRITIS

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Jianhua Dong ◽  
Li Huang ◽  
Chuan Li ◽  
Ling Kong ◽  
Lixuan Huang ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Adverse Events ◽  
Blood Cell ◽  
Serum Creatinine ◽  
Clinical Efficacy ◽  
Vascular Access ◽  
Double Filtration Plasmapheresis ◽  
Severe Lupus Nephritis ◽  
Double Filtration

Abstract Background and Aims The Study delves into the clinical efficacy and advantages of centrifugal double filtration plasmapheresis on severe lupus nephritis (LN) by comparing it with membranous double filtration plasmapheresis (DFPP). Method A retrospective analysis was performed on 56 patients who were diagnosed with severe LN and had received DFPP treatment from May 2016 and January 2020. Of them, 38 were given centrifugal DFPP and had their plasma centrifuged in a blood cell separator, and 18 were given membranous DFPP and had their plasma centrifuged in an MPS07 plasma separator. An EC20W plasma component separator was used as the secondary filter to reprocess the separated plasma of all of them. The two DFPPs were compared for differences in clinical efficacy, vascular access, dosage of anticoagulant, treatment cost and adverse events in patients with severe LN. Results Of the 56 severe LN patients (including 43 females and 13 males), the median of age of onset was 29 years old, the SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) was 18.6±6.0 points and the serum creatinine was 402(294,553) umol/L, and all patients had acute kidney injury and 51 of them (91.1%) required renal replacement therapy (RRT). A total of 142 DFPPs were performed, including 97 centrifugal DFPPs and 45 membranous DFPPs. After treatment and at Month 3 of follow-up visit, patients in both the centrifugal DFPP group and the membranous DFPP group had ANA, AdsDNA titer, quantitative urinary protein, urinary red blood cell count and serum creatinine significantly dropped and hemoglobin significantly increased over those before treatment, the differences in which between the two groups, however, were not statistically significant. The centrifugal DFPP group had a more significant drop in complements C3 and C4 after treatment. Comparison of the data before and after a single DFPP treatment showed that the membranous DFPP group had a more significantly longer prothrombin time, but there were no differences in partial prothrombin time, fibrinogen and platelet change between the two groups. At Month 3 of follow-up visit, 31 of the 51 RRT patients (60.8%) (including 34 given centrifugal DFPP and 17 given membranous DFPP) were released from dialysis, including 23 given centrifugal DFPP and 8 given membranous DFPP. In the membranous DFPP group, all patients had the vascular access built via the central venous catheter, while in the centrifugal DFPP group, 6 patients (15.8%) had the vascular access built by puncturing into the artery or vein. The dosage of the anticoagulant, the low molecular weight heparin, to the centrifugal DFPP group was significantly lower than that to the membranous DFPP group (1174±243 vs 4106±399IU, P&lt;0.001), and in the centrifugal DFPP group, 29 patients (76.3%) were given 4% citric acid alone for anti-coagulation. No blood coagulation occurred. In terms of treatment consumables, the membranous DFPP group had a significantly higher cost than the centrifugal DFPP group (RMB4340.2±237.0 vs 5677.0±0.0, P&lt;0.001). Two patients (4.4%) in the membranous DFPP group developed skin ectasis, epistaxis or aggravated alveolar hemorrhage after treatment, and four patients (4.1%) in the centrifugal DFPP group developed perioral numbness, numbness in distal extremities or tetany during treatment, which was alleviated after calcium supplementation. Conclusion Centrifugal DFPP differed little from membranous DFPP in clinical efficacy in severe LN patients, but had lower anti-coagulation requirements, cost less on treatment consumables, and caused no severe adverse events, so it can be used as an important means to treat severe LN.

scholarly journals Lupus Nephritis: Role of Serum Complement Levels as Prognostic Marker

2020 ◽  
Vol 6 (1) ◽  
pp. 01-05
Author(s):  
Richmond Gomes
Keyword(s):  
Lupus Nephritis ◽  
Treatment Response ◽  
Lupus Erythematosus ◽  
Serum Complement ◽  
Class Iii ◽  
Proliferative Lupus Nephritis ◽  
Significant Difference ◽  
Before And After ◽  
Assess Treatment Response ◽  
Serological Biomarkers

Background: Lupus Nephritis (LN) is one of the most common and serious manifestations in Systemic Lupus Erythematosus (SLE) patients that causes significant morbidity and mortality. Certain biomarkers for LN are sometimes able to assess treatment response of lupus nephritis. Objective: To compare serum complement levels (C3 & C4) as markers of treatment response of LN and their relation to the LN class in renal biopsy. Methods: This prospective observational study was conducted in the Department of Nephrology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from July 2018 to August 2019. Twenty seven patients who were diagnosed with lupus nephritis after kidney biopsy were included in this study. Serum complement levels (C3 & C4), 24 hours urinary total protein (24-hr UTP) and anti-double-stranded DNA (anti-ds DNA) were measured in all patients at baseline, 3 months and 6 months after treatment. These biomarker values before and after treatment were compared between the treatment response and non response groups. Results: Serum C3 levels were significantly different in patients of proliferative lupus nephritis (Class III & Class IV) than non proliferative lupus nephritis (Class V) at baseline (0.47 ± 0.32 vs0.89 ± 0.43g/l, p = 0.009) and levels changed significantly 6 months after treatment (p <0.001) and likewise for Serum C4 levels (0.10 ± 0.06 vs0.24 ± 0.26g/l, p = 0.040). Serum C3 levels were also found to correlate significantly with SLEDAI and renal SLEDAI. No significant difference was observed for 24-hr UTP levels at baseline between remission and non-remission groups. Conclusion: Serum C3 & C4 levels may be utilized as serological biomarkers to predict and monitor the treatment response of lupus nephritis.

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