scholarly journals Prognostic Factors for Survival and Relapse in ANCA-Associated Vasculitis with Renal Involvement: A Clinical Long-Term Follow-Up Study

2018 ◽  
Vol 2018 ◽  
pp. 1-11 ◽  
Author(s):  
Anna Salmela ◽  
Tom Törnroth ◽  
Tuija Poussa ◽  
Agneta Ekstrand
Keyword(s):  
Prognostic Factors ◽  
Patient Survival ◽  
Renal Involvement ◽  
Multivariable Analysis ◽  
Induction Treatment ◽  
Renal Survival ◽  
Histopathological Classification ◽  
Anca Associated Vasculitis

Aim. We describe the clinical pattern of ANCA-associated vasculitis (AAV) and assess long-term prognostic factors of patients and renal survival and relapse. Methods. Data from 85 patients with renal biopsy-proven AAV at a single center with up to 20-year [median 16.2 years (95% CI 14.9-17.7)] follow-up were retrospectively collected. Results. Overall, 55% of the patients had microscopic polyangiitis (MPA) and 45% had granulomatosis with polyangiitis (GPA). The histopathological classes were focal in 35%, crescentic in 26%, mixed in 20%, and sclerotic glomerulonephritis in 19% of the patients. As induction treatment, a combination of cyclophosphamide and corticosteroids was given to 82%, while a combination of azathioprine and corticosteroids was maintenance therapy in 79%. The twenty-year patient survival was 45%. In a multivariable analysis, age ≥58 years [hazard ratio (HR) 7.64, 95% CI 3.44-16.95] and myeloperoxidase (MPO) ANCA (HR 2.12, 95% CI 1.08-4.17) were associated with shorter patient survival time. Renal survival was 68% overall: 88% in focal, 71% in crescentic, 56% in mixed, and 37% in sclerotic class (p=0.01). Female sex (HR 0.26, 95% CI 0.10-0.73) was a predictor of improved renal survival, whereas GFR <30 ml/min and MPO-ANCA were associated with worse renal survival (HR 4.10, 95% CI 1.35-12.49 and HR 3.10, 95% CI 1.21-7.95, respectively). Relapse-free survival at 20 years was 10%. MPA was associated with a lower risk for relapse (HR 0.48, 95% CI 0.28–0.82). Conclusion. We confirmed the improved patient and renal survival in AAV patients with glomerulonephritis, while relapse remained the primary challenge. Histopathological classification may be relevant for survival.

scholarly journals SAT0245 RENAL INVOLVEMENT AT ONSET IN ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED VASCULITIS: A MAJOR INDEPENDENT RISK FACTOR FOR RENAL RELAPSE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1065.2-1065
Author(s):  
M. Felicetti ◽  
A. Ortolan ◽  
A. C. Frigo ◽  
R. Padoan ◽  
M. Gasparotto ◽  
...  
Keyword(s):  
Renal Involvement ◽  
Multivariable Analysis ◽  
Low Frequency ◽  
Information Criterion ◽  
Remission Induction ◽  
Induction Treatment ◽  
Inclusion Criteria ◽  
Treatment Regimens ◽  
Anca Associated Vasculitis

Background:In ANCA-associated vasculitis (AAV), renal relapses are cause for concern as they are unpredictable and predictors of end-stage renal disease (ESRD).Objectives:We aimed to assess the frequency of major renal (MR) relapses in AAV in our cohort and identify independent predictors of the first MR relapse at diagnosis.Methods:We performed a retrospective monocentric observational study in our Vasculitis clinic from January 2000 to August 2019. Inclusion criteria were: 1) granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and limited kidney disease (LKD) diagnosis fulfilling EMA algorithm criteria; 2) achievement of a stable remission, defined as absence of vasculitis symptoms or signs and adherence to the prednisone taper during remission-induction treatment. We excluded patients who developed ESRD before remission and those with incomplete data during the follow-up. Major renal (MR) relapses were defined as occurrence of at least one major item of renal Birmingham Vasculitis Activity Score version 3 (BVASv3).All remitted patients were allocated in two subgroups: patients without MR relapse and patients with MR relapse. Univariate and multivariable analysis of first MR relapse predictors was performed with Fine and Gray (F&G) sub distribution hazards model to assess all competitive risks (progression to ESRD without MR relapse and death before MR relapse). Due to the relatively low frequency of events and the risk of overfitting, we performed several multivariable models with three variables, as recommended by Peduzzi e al1. The best multivariable model was selected accordingly to the Akaike information criterion (AIC).Results:96 (53% females) patients met the inclusion criteria: 74 GPA, 21 MPA and 1 LKD. Median age at diagnosis was 54 (44-64) years. ANCA testing was present in 94 patients, 85 were ANCA positive: 56 c-ANCA/PR3, 28 p-ANCA/MPO and 1 double positivity.During a median follow-up (FU) of 54.5 months (29.3-96.5), we observed 19 MR relapses in 17 patients while 2 patients progressed to ESRD, 3 died without events and 76 reported no MR relapse. Density-incidence of MR relapses since remission was 3.6/100 person-year (CI 95% 2.2-5.6). Median time to first MR relapse after remission was 33 months (14-67.5).At first MR relapse, 8 (53.3%) patients were on steroids while 10 (66.7%) were on immunosuppressant (5 azathioprine, 5 mycophenolate). In 2 cases, data about remission-maintenance treatment was not available.MR relapses were observed only in ANCA positive patients with a significantly higher frequency of skin, kidney and nerve involvement at diagnosis (41.2% vs 17.7%, p=0.034, 94.1% vs 57.0% p=0.004, and 52.9% vs 25.3% p=0.024, respectively); while Ear, Nose and Throat (ENT) involvement was significantly lower (35.3% vs 62.0% p=0.043). Mean BVASv3 at diagnosis scored significantly higher in MR relapse group (24.1±6.2 vs 18.1±8.1. p=0.007).At multivariable analysis with F&G model, renal involvement and induction treatment without cyclophosphamide and/or Rituximab at diagnosis were independent predictors of MR relapse (sHR 20.4 (2.6-158.2), p=0.004 and sHR 4.2 (1.5-12.0), p=0.007, respectively). Moreover, there was a trend of higher risk of MR relapse in PR3-ANCA (sHR 2.5 (0.9-7.1), p=0.091).Conclusion:Renal involvement at diagnosis and milder remission-induction treatment regimens resulted in a significantly higher risk of MR relapse during the FU in our cohort. PR3-ANCA specificity was not an independent predictor of MR relapse, even if we observed a trend of higher MR relapse risk with this covariate.References:[1]Peduzzi P et al. A simulation study of the number of events per variable in logistic regression analysis. J Clin Epidemiol. 1996;49(12):1373-9.Disclosure of Interests:Mara Felicetti: None declared, Augusta Ortolan: None declared, Anna Chiara Frigo: None declared, Roberto Padoan: None declared, Michela Gasparotto: None declared, Mariagrazia Lorenzin: None declared, Andrea Doria Consultant of: GSK, Pfizer, Abbvie, Novartis, Ely Lilly, Speakers bureau: UCB pharma, GSK, Pfizer, Janssen, Abbvie, Novartis, Ely Lilly, BMS, Roberta Ramonda Speakers bureau: Novartis, Celgene, Janssen, Pfizer, Abbvie, Lilly, Franco Schiavon: None declared

Prognostic Factors and Long-Term Outcome with ANCA-Associated Kidney Vasculitis in Childhood

2021 ◽  
pp. CJN.19181220
Author(s):  
Marta Calatroni ◽  
Filippo Consonni ◽  
Marco Allinovi ◽  
Alessandra Bettiol ◽  
Natasha Jawa ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Kidney Failure ◽  
Prognostic Significance ◽  
Remission Induction ◽  
Long Term Outcome ◽  
Anca Associated Vasculitis ◽  
Kidney Involvement ◽  
Ckd Stage

Background and objectives. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare in children. We report the clinico-pathological features, long-term outcomes, and prognostic factors of a large paediatric cohort of patients with ANCA-associated kidney vasculitis. Design, setting, participants, and measurements. This retrospective study included 85 consecutive patients with kidney biopsy-proven ANCA-associated vasculitis followed at tertiary referral centres in Italy and Canada. Kidney biopsies were categorised as focal, crescentic, sclerotic or mixed following Berden's classification. The prognostic significance of baseline clinical, laboratory and histological findings was analysed with respect to kidney failure or chronic kidney disease (CKD) 3-5/kidney failure. Results. Fifty-three patients had microscopic polyangiitis (62%) and 32 granulomatosis with polyangiitis (38%). Rapidly progressive glomerulonephritis was the most frequent presentation (39%); one third of the patients also had nephrotic-range proteinuria. Kidney biopsies were classified as focal in 21% of the patients, crescentic in 51%, sclerotic in 15% and mixed in 13%. Remission-induction therapies included cyclophosphamide in 78% of cases. Twenty-five patients (29%) reached kidney failure. The median time to kidney failure or last follow-up was 35 months (6-89) in the whole cohort, and 73 months (24-109) among the patients who did not reach this outcome. Cases with sclerotic histology showed significantly shorter kidney survival [HR 11.80 (95% CI 2.49-55.99)] and CKD 3-5-free survival [HR 8.88 (95% CI 2.43-32.48)] as compared with focal/mixed ones. Baseline eGFR, low serum albumin, hypertension, central nervous system complications and sclerotic histology, which reflected severe kidney involvement, were associated with both kidney failure or CKD stage 3-5/kidney failure at unadjusted analysis; no independent prognostic factors emerged at multivariate analysis. Conclusions. Children with ANCA-associated kidney vasculitis often have aggressive presentation; one-third of them progress to kidney failure and usually do so early during the follow-up. A severe renal presentation is associated with the development of CKD or kidney failure.

Long-term patient survival in ANCA-associated vasculitis

2010 ◽  
Vol 70 (3) ◽  
pp. 488-494 ◽  
Author(s):  
Oliver Flossmann ◽  
Annelies Berden ◽  
Kirsten de Groot ◽  
Chris Hagen ◽  
Lorraine Harper ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Causes Of Death ◽  
Patient Survival ◽  
First Year ◽  
Term Survival ◽  
Risk Of Death ◽  
Anca Associated Vasculitis ◽  
Increased Risk ◽  
Age And Sex

BackgroundWegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.ObjectiveTo describe the long-term patient survival and possible prognostic factors at presentation in an international, multicentre, prospectively recruited representative patient cohort who were treated according to strictly defined protocols at presentation and included the full spectrum of ANCA-associated vasculitis disease.MethodsOutcome data were collected for 535 patients who had been recruited at the time of diagnosis to four randomised controlled trials between 1995 and 2002. Trial eligibility was defined by disease severity and extent, covered the spectrum of severity of ANCA-associated vasculitis and used consistent diagnostic criteria. Demographic, clinical and laboratory parameters at trial entry were tested as potential prognostic factors in multivariable models.ResultsThe median duration of follow-up was 5.2 years and 133 (25%) deaths were recorded. Compared with an age- and sex-matched general population there was a mortality ratio of 2.6 (95% CI 2.2 to 3.1). Main causes of death within the first year were infection (48%) and active vasculitis (19%). After the first year the major causes of death were cardiovascular disease (26%), malignancy (22%) and infection (20%). Multivariable analysis showed an estimated glomerular filtration rate <15 ml/min, advancing age, higher Birmingham Vasculitis Activity Score, lower haemoglobin and higher white cell count were significant negative prognostic factors for patient survival.ConclusionPatients with ANCA-associated vasculitis treated with conventional regimens are at increased risk of death compared with an age- and sex-matched population.

scholarly journals FC 033LONG-TERM FOLLOW-UP OF PATIENTS WITH ANCA-ASSOCIATED VASCULITIS INCLUDED IN EUROPEAN VASCULITIS SOCIETY RANDOMIZED CLINICAL TRIALS SINCE 1995: A SURVIVAL ANALYSIS

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Beatriz Sanchez Alamo ◽  
Laura Moi ◽  
Mikkel Faurschou ◽  
Thomas Hauser ◽  
Ingeborg M Bajema ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Patient Outcomes ◽  
Causes Of Death ◽  
Randomized Clinical Trials ◽  
Patient Survival ◽  
Anca Associated Vasculitis ◽  
Ckd Stage ◽  
End Stage

Abstract Background and Aims ANCA-associated vasculitis (AAV) is the most common cause of rapidly progressive glomerulonephritis globally and the most common finding in renal biopsies for those above 75 years of age. Patients with AAV have had an improved outcome after the introduction of immunosuppressive therapy. However, there is still an increased risk of end stage renal failure, complications to therapy, comorbidities and death. The European Vasculitis Society (EUVAS) has run several prospective randomized clinical trials (RCT) since late -90’ies as an attempt to improve the outcome for patients with a broad spectrum of AAV. The aim of the current study was to analyze results from a 10-year follow-up of patients who have participated in EUVAS’s randomized clinical trials to report on patient outcomes using current standard of care immunosuppressive treatment. Method Data on patient outcomes were collected from questionnaires to the principal investigators of the original RCTS (74 centers from 17 countries in Europe): MEPEX, NORAM, CYCAZAREM, CYCLOPS, IMPROVE, RITUXVAS and MYCYC. All patients were well characterized at inclusion in the trials respectively, as was the type and duration of induction therapy. Long-term follow-up data regarding cumulative duration and type of immunosuppressive therapy, end stage renal failure (ESRF), renal transplantation, patient survival and comorbidities as cardiovascular events, infections were recorded. Efforts have been made to control and validate the collected data. Results The current dataset comprises 858 patients, of whom 708 were eligible to include in this study. We received questionnaires regarding patient outcomes from 70% of the cohort. The median age was 60 years at time of the diagnosis of AAV; 487 patients had GPA (57%) and 371 MPA (43%). The median follow-up time was 8.0 years (IQR: 2.9-13.6). During the observation period, there were 305 deaths: 78 patients died within the first year of follow up, 69 during the following 5 years, and another 158 patients after 5 years. The main causes of death were infections (25%), cardiovascular disease (14%) and malignancies (13%), followed by pulmonary diseases, vasculitis, others, kidney disease and gastrointestinal disease. Vasculitis were a major contributor to the causes of death in 18% of the patients. Median survival time for the whole cohort was 17.8 years (95%CI: 15.6 – 20.1). Survival rate at 5 and 10 years was 81.6% and 66.5% respectively. Advanced age, low estimated glomerular filtration rate and male sex were negative prognostic factors for patient survival in the Cox model. When we categorized the deaths according to their initial CKD stage, the highest number of deaths was found in the group of patients with low eGFR i.e. an initial CKD stage V (40%). The initial CKD stage, as shown in the Kaplan-Meier curve, was found to have an important impact on the patient survival (LR: 144.4 p&lt;0.001). In our cohort, 159 patients (18.5%) reached ESRF, of whom 110 patients died (69%); the main cause of death in this group was infection. Conclusion In a series of over 800 patients with AAV, ten-year mortality was predominantly associated with impaired renal function at onset, older age and male gender, rather than subtype of disease. The main strength of this study is that we have a well-defined cohort of patients with AAV, all with well-defined induction treatments and a long period of follow-up with the possibility to analyze possible prognostic factors regarding outcomes. Further analyses are ongoing regarding comparative statistics, cumulative incidence of malignancies, renal transplantation and comorbidities. The data supports efforts to diagnose these patients at an earlier stage, when they have a higher eGFR and to tailor therapy for the individual patient. Although newer treatment modalities are available, there is still a considerable morbidity and mortality in the long-term.

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 Renal survival and prognostic factors in patients with PR3-ANCA associated vasculitis with renal involvement

2003 ◽  
Vol 63 (2) ◽  
pp. 670-677 ◽  
Author(s):  
Marjan C. Slot ◽  
Jan Willem Cohen Tervaert ◽  
Casper F.M. Franssen ◽  
Coen A. Stegeman
Keyword(s):  
Prognostic Factors ◽  
Renal Involvement ◽  
Renal Survival ◽  
Anca Associated Vasculitis

Prognostic factors that mandate long term follow up following surgery for appendix neuroendocrine tumours (aNETs)

2016 ◽  
Author(s):  
Edward Alabraba ◽  
Heman Joshi ◽  
Andrea Tufo ◽  
Hassan Malik ◽  
Melissa Banks ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Neuroendocrine Tumours ◽  
Long Term Follow Up

scholarly journals POS0119 RENAL INVOLVEMENT AND NEED OF RENAL REPLACEMENT THERAPY IN ANCA ASSOCIATED VASCULITIS IN A SPANISH SINGLE-CENTER STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 270.2-271
Author(s):  
J. Álvarez Troncoso ◽  
J. C. Santacruz Mancheno ◽  
A. Díez Vidal ◽  
S. Afonso Ramos ◽  
A. Noblejas Mozo ◽  
...  
Keyword(s):  
Renal Replacement Therapy ◽  
Replacement Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Age At Diagnosis ◽  
Systemic Involvement ◽  
Risk Of Death ◽  
Anca Associated Vasculitis ◽  
The Mean

Background:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EPGA). Renal involvement is frequent in AAV and is an important factor for morbidity and mortality.Objectives:The main objective of this study was to analyze the demographic, clinical, histological and therapeutic characteristics of renal involvement in patients with AAV and the risk of renal replacement therapy (RRT) or death.Methods:Retrospective observational study of 56 patients with AAV fulfilling classificatory criteria and renal involvement diagnosed between 1995 and 2020 from a Spanish tertiary centre. We studied the histological involvement (according to the 2010 classification in focal, crescentic, mixed or sclerotic), immunofluorescence (IF) and the treatment received with the risk of RRT or death.Results:We included 56 patients diagnosed with AAV and renal involvement. The mean age was 61.08±4.05 years; 58.9% were women. The mean follow-up time of these patients was 16.14± 8.80 years. Only 57.1% of patients presented systemic involvement.Most frequent non-renal AAV manifestations were lung involvement (39.3%), central nervous system (30.4%), otorhinolaryngology (ORL) (14.3%), skin (8.9%) and cardiac involvement (8.9%). Main immunological findings were ANCA-MPO+ (69.6%), ANCA-PR3+ (23.2%), ANCA-negative (5.4%). Low C3 was found in 19.6% patients. Histologic classification (HC) and need of RRT is described in table 1. Main HC in renal AAV was crescentic, mixed, focal and sclerotic respectively. Eight patients had not biopsy performed. IF was positive for C3 deposits in 20 patients (35.7%). Half of the patients presented <50% normal glomeruli.The treatment of renal involvement in AAV in our cohort was as follows: 83.9% (47) corticosteroids (CS) and cyclophosphamide (of which 40 received intravenous and 7 oral cyclophosphamide; and 12 patients associated plasma exchange (PE) with this treatment), 5.36% CS alone, 2 patients received CS and mycophenolate; 1 CS and rituximab, 1 CS and PE, and 2 patients received no treatment. A total of 13 patients received PE and 18 RRT. The mean time to RRT was 65.44±32.72 months. Relapses were not uncommon, 33.93% of the patients presented ≥1 relapse and 10.71% presented ≥2.Infections were very frequent since they were present in 91.07% of the patients. Other frequent non-immunological complications observed in the follow-up of these patients were thrombosis in 31.14%, cardiovascular events in 28.57% and cancer in 19.64%.Patients with ANCA-PR3+ were younger at diagnosis (p<0.001), were more likely to present cardiac (p=0.045) and ORL involvement (p<0.001). However, neither ANCA-PR3+ nor ANCA-MPO+ were specifically associated with the need of RRT or higher risk of death in our cohort. Use of CS alone for the treatment of renal AAV was associated with higher mortality (p=0.006) but CS and cyclophosphamide with lower mortality (p=0.044). ANCA-negative patients were more likely to receive no treatment. Having <50% normal glomeruli and C3 deposits on IF were associated with an increased need for RRT. Presenting focal disease on HC was protective for the need of RRT. Older age at diagnosis, systemic involvement of AAV and need of RRT was associated with higher mortality.Conclusion:AAVs are complex vasculitides with frequent renal involvement. Increased C3 deposition, non-focal histological forms, and <50% normal glomeruli were related to the need for RRT. In turn, the need for RRT, a later age at diagnosis, and systemic involvement were associated with higher mortality. Holistic and multidisciplinary early management of AAVs in experience centers can help improve renal prognosis and decrease mortality.References:[1]Binda et al. ANCA-associated vasculitis with renal involvement. J Nephrol. 2018 Apr;31(2):197-208.[2]Kronbichler et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev. 2020 Apr;19(4):102495.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

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