103 Unusual Renal Recovery After Severe Lupus Nephritis (LN) Complicated by Thrombotic Microangiopathy (TMA)

2020 ◽  
Vol 75 (4) ◽  
pp. 565 ◽  
Keyword(s):  
Lupus Nephritis ◽  
Thrombotic Microangiopathy ◽  
Renal Recovery ◽  
Severe Lupus Nephritis

scholarly journals SAT0211 RENAL INJURY IN SYSTEMIC LUPUS ERYTHEMATOSUS CHARACTERIZED BY THROMBOTIC MICROANGIOPATHY

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

scholarly journals SAT0210 FACTORS ASSOCIATED WITH TIME TO SEVERE LUPUS NEPHRITIS IN A COHORT OF COLOMBIAN PATIENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1048.2-1048
Author(s):  
S. Herrera ◽  
J. C. Diaz-Coronado ◽  
D. Rojas-Gualdrón ◽  
L. Betancur-Vasquez ◽  
D. Gonzalez-Hurtado ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Renal Involvement ◽  
Classification Criteria ◽  
P Value ◽  
Systemic Lupus ◽  
P Values ◽  
Interval Censored ◽  
Severe Lupus Nephritis ◽  
Age And Sex

Background:Systemic lupus erythematosus (SLE) clinical manifestations, and their severity, vary according to age, ethnicity and socioeconomic status. Both Hispanic and Afro-Americans have a higher incidence and more sever presentation when compared to Caucasian patients with SLEObjectives:To analyze clinical and immunological characteristics associated with time to severe renal involvement in patients with Systemic Lupus Erythematous in a Colombian cohort followed for one year, between January 2015 and December 2018Methods:Retrospective follow-up study based in clinical records. Patients with SLE diagnosis that fulfilled either 1987 American College of Rheumatology Classification Criteria for SLE or 2011 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE. We included patients with diagnosis of lupus nephritis according to Wallace and Dubois criteria. Patients who did not have at least two follow-up measurements or had a cause of nephritis other than lupus were excluded. The main outcome was defined as time from diagnosis to sever renal involvement defined as creatinine clearance ≤50 ml/min, 24-hour proteinuria ≥3.5 grams o end stage renal disease.We analyzed clinical and immunological characteristics. Descriptive statistical analyses of participant data during the first evaluation are reported as frequencies and percentages for categorical variables, and as medians and interquartile ranges (IQR) for quantitative variables. Age and sex adjusted survival functions and Hazard ratios (HR) with 95% confidence intervals and p-values were estimated using parametric Weibull models por interval-censored data. P values < 0.05 were considered statistically significantResults:548 patients were analyzed: 67 were left-censored as they presented renal involvement at entry, 6 were interval censored as outcome occurred between study visits, and 475 were right-censored as involvement was not registered during follow-up. 529 (96.5%) patients were female, median age at entry was 46 (IQR = 23) and median age to diagnosis was 29.5 (IQR = 20.6). 67% were mestizo, 13% Caucasian and 0.3% Afro-Colombian. Age and sex adjusted variables associated with time to severe lupus nephritis were high blood pressure HR = 3.5 (95%CI 2.2-5.6; p-value <0.001) and Anti-RO (per unit increase) HR = 1.002 (95%CI 1.001-1.004; p-value = 0.04). Figure 1 shows age and sex adjusted survival function.Conclusion:In our cohort the appearance of severe lupus nephritis occurs in less than 15% of patients at 10 years. Both high blood pressure and elevated anti-Ro titers were associated with a higher rate of onset in the presentation of severe lupus nephritis, as seen in some polymorphs of anti Ro.References:Disclosure of Interests:Sebastian Herrera Speakers bureau: academic conference, Juan camilo Diaz-Coronado: None declared, Diego Rojas-Gualdrón: None declared, Laura Betancur-Vasquez: None declared, Daniel Gonzalez-Hurtado: None declared, Juanita Gonzalez-Arango: None declared, laura Uribe-Arango: None declared, Maria Fernanda Saavedra Chacón: None declared, Jorge Lacouture-Fierro: None declared, Santiago Monsalve: None declared, Sebastian Guerra-Zarama: None declared, Juan david Lopez: None declared, Juan david Serna: None declared, Julian Barbosa: None declared, Ana Sierra: None declared, Deicy Hernandez-Parra: None declared, Ricardo Pineda.Tamayo: None declared

scholarly journals Severe Lupus Nephritis: Racial Differences in Presentation and Outcome

2006 ◽  
Vol 18 (1) ◽  
pp. 244-254 ◽  
Author(s):  
Stephen M. Korbet ◽  
Melvin M. Schwartz ◽  
Joni Evans ◽  
Edmund J. Lewis
Keyword(s):  
Lupus Nephritis ◽  
Racial Differences ◽  
Severe Lupus Nephritis

Mycophenolate Mofetil for Remission Induction in Severe Lupus Nephritis

2005 ◽  
Vol 100 (3) ◽  
pp. c92-c100 ◽  
Author(s):  
Jane Cross ◽  
Adu Dwomoa ◽  
Peter Andrews ◽  
Aine Burns ◽  
Caroline Gordon ◽  
...  
Keyword(s):  
Mycophenolate Mofetil ◽  
Lupus Nephritis ◽  
Remission Induction ◽  
Severe Lupus Nephritis

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 Acute Kidney Injury Relevant to Tubulointerstitial Nephritis with Late-Onset Uveitis Superimposed by Thrombotic Microangiopathy: A Case Report and Review of the Literature

2020 ◽  
Vol 6 (6) ◽  
pp. 414-421
Author(s):  
Youlu Zhao ◽  
Junwen Huang ◽  
Tao Su ◽  
Zhikai Yang ◽  
Xizi Zheng ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Thrombotic Microangiopathy ◽  
Tubulointerstitial Nephritis ◽  
Late Onset ◽  
Drug Hypersensitivity ◽  
Oral Prednisone ◽  
Kidney Injury ◽  
Renin Angiotensin System ◽  
Renal Recovery

<b><i>Background:</i></b> The syndrome of tubulointerstitial nephritis and uveitis (TINU) is an uncommon and multisystemic autoimmune disorder. This review reports a rare case of TINU being superimposed on thrombotic microangiopathy (TMA) and, by comparing with the available literature, also summarizes the clinical features, associated conditions, treatment, and outcome of patients with TINU. <b><i>Summary:</i></b> Herein, we report the case of a 37-year-old male patient with acute kidney injury (AKI) clinicopathologically identified as malignant hypertension-induced TMA superimposed by acute tubulointerstitial nephritis, which was suspected to be related to drug hypersensitivity. After treatment with oral prednisone combined with a renin-angiotensin system inhibitor, the patient achieved partial renal recovery and was withdrawn from hemodialysis. Recurrent AKI concomitant with new-onset asymptomatic uveitis was detected during routine clinical follow-up after cessation of prednisone. TINU was then diagnosed, and prednisone followed by cyclophosphamide was prescribed. The patient achieved better renal recovery than in the first round of treatment and maintained stable renal function afterward. By reviewing the literature, 36 cases were reported as TINU superimposed on other conditions, including thyroiditis, osteoarthropathy, and sarcoid-like noncaseating granulomas. <b><i>Key messages:</i></b> TINU could be complicated by many other conditions, among which TMA is very rare. When presented as AKI, kidney biopsy is important for differential diagnosis. The case also shows that recurrent AKI with concomitant uveitis after prednisone withdrawal strongly suggested the need for long-term follow-up and elongated prednisone therapy for TINU syndrome.

Evidence of concurrent immunopathological mechanisms determining the pathology of severe lupus nephritis

Lupus ◽  
2009 ◽  
Vol 18 (2) ◽  
pp. 149-158 ◽  
Author(s):  
MM Schwartz ◽  
SM Korbet ◽  
RS Katz ◽  
EJ Lewis
Keyword(s):  
Lupus Nephritis ◽  
Severe Lupus Nephritis
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