P0471DIALYSIS TREATMENT AT THE TIME OF DIAGNOSIS PREDICTS OUTCOMES IN ANCA ASSOCIATED VASCULITIS PATIENTS - DATA FROM CROATIAN REFERRAL CENTER

Abstract Background and Aims Dialysis dependence and ESRD are known complications of ANCA associated vasculitis (AAV) with renal involvement. What is not so often discussed is the role of dialysis treatment at the time of diagnosis and how it affects patient outcomes as well as characteristics of such patients. We present data showing the importance of dialysis treatment at the time of diagnosis as the predictor of clinical outcomes. Method This study included 106 consecutive AAV patients with renal involvement in the period from 2007-2017. We performed renal biopsy on patients using automatic 16 Gauge needle. Light, immunofluorescent and electronic microscopy were performed. Primary outcomes were combined outcome progression to end-stage renal disease, defined as persistent (more than three months) need for renal replacement therapy or permanent reduction of EGFR to <15ml/minute (according to CKD EPI formula) and/or death (ESRDD), death (D) and ESRD alone, and disease relapse. Kaplan Meyer survival analysis and multivariate Cox proportional hazard regression analysis were used to explore difference between phenotypes and finding significant predictors regarding outcomes. Results Out of 106 patients (55,6% female, median age 61; IQR 51-70) there were 66 (61,1%) microscopic poliangitiis (MPA), 20 (18,5%) granulomatosis with angitiis and 20 (18,5%) with renal limited vasculitis (RLV). Out of those 14 (13%) were PR3-ANCA positive patients, 57 (52,8%) MPO ANCA positive, 5 (4,6%) PR3-ANCA+MPO-ANCA positive and 32 (29,6%) ANCA negative patients. Average serum creatinine (SCr) levels was 316,5 μmol/l (IQR 207,0-548,5), 24-hour proteinuria median was 1,7g/24h (IQR 0,8-2,8). According to the Berden classification 43 (39,8%) patients had crescentic, 19 (17,6%) focal, 34 (31,5%) mixed and 12 (11,1%) sclerotic class. Follow up time ranged from 1 to 127 months. Median follow up time was 21 months (IQR = 7-44). Median time to diagnosis was 3 months (IQR 2,0-6,0). Patients requiring dialysis treatment at the time of diagnosis were more often MPO – (p=0,04), had more severe anemia (p=0,001), higher CRP (p=0,003), and more pronounced hypoalbuminemia (serums albumin <30g/l; p=0,006).Such patients were older than those not requiring dialysis (p=0,055) na had shorter time to diagnosis (p=0,001). Clinically such patient s presented more often with RPGN (p<0,001) which is in a way expected thus having higher SCr levels (p=<0,001). Histologically dialysis treated patients predominantly had crescentic class, while non-dialysis group had focal class (p<0,001). Of note dialysis group had more acute tubular damage (p=0,007). Interestingly enough there was slightly more positive C3 deposition in dialysis group (p=0,09). In univariate analysis the need for acute dialysis at the time of diagnosis of AAV was significant predictor for combined ESRDD, D, ESRD and relapse rate. In multivariate analysis the need for acute dialysis at the time of diagnosis of AAV remained significant predictor for ESRD (HR = 4,674, 95% CI =1,996-10,946; p = < 0,001) and relapse rate (HR = 59,545, 95% CI =3,467-1022,665; p = 0,005). Conclusion The need for dialysis at the time of AAV diagnosis is a strong predictor for ESRD and relapse rate. It is also interesting to further study differences between patients needing dialysis at the time of diagnosis and those who don’t need it.

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MO290CLINICAL DIFFERENCES AND OUTCOMES IN ANCA ASSOCIATED VASCULITIS PATIENTS ACCORDING TO SEROLOGICAL PHENOTYPE – EXPERIENCE FROM CROATIAN REFERRAL CENTER

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab104.0048 ◽

2021 ◽

Vol 36 (Supplement_1) ◽

Author(s):

Matija Crnogorac ◽

Ivica Horvatić ◽

Patricia Kacinari ◽

Miroslav Tišljar ◽

Ana Brechelmacher ◽

...

Keyword(s):

Relapse Rate ◽

Renal Involvement ◽

Strong Tendency ◽

Tubular Damage ◽

Constitutional Symptom ◽

Lung Involvement ◽

Double Positive ◽

Anca Associated Vasculitis ◽

Significant Difference

Abstract Background and Aims ANCA associated vasculitis (AAV) are usually classified according to clinical presentation (Chapel-Hill consensus conference). There is however suggestion by some authors that AAVs could be classified according to ANCA specificity. We aimed to compare AAV patients in our cohort according to serological phenotype. Method This study included 106 consecutive AAV patients with renal involvement in the period from 2007-2017. We performed renal biopsy on patients using automatic 16 Gauge needle. Light, immunofluorescent and electronic microscopy were performed. Category variables were analysed with Fisher Exact testom and continuous with Kruskal-Wallis testom. Statistical difference was then analysed posthoc with Chi-square test. Primary outcomes were combined outcome progression to end-stage renal disease, defined as persistent (more than three months) need for renal replacement therapy or permanent reduction of EGFR to <15ml/minute (according to CKD EPI formula) and/or death (ESRDD), death (D) and ESRD alone, and disease relapse. Kaplan Meyer survival analysis and multivariate Cox proportional hazard regression analysis were used to explore difference between phenotypes and finding significant predictors regarding outcomes. Results The study included 106 AAV patients with renal involvement: 66 (61,1%) MPA, 20 (18,5%) GPA, 20 (18,5%) RLV. There were 14 (13%) PR3-ANCA positive patients, 57 (52,8%) MPO ANCA positive, 5 (4,6%) PR3-ANCA+MPO-ANCA and 32 (29,6%) ANCA negative patients. Average SCr was 316,5 μmol/l (IQR 207,0-548,5), 24-hour proteinuria median was 1,7g/24h (IQR 0,8-2,8). Clinicaly PR3 positive AAVs had significantly more ENT (p<0,001) and skin (p=0,001) involvement, and ANCA negatives had significantly less lung involvement (p<0,001), and less expressed constitutional symptom (p=0,031). Interestingly both MPO and PR3 positive AAV patients had approximately equal percentage of lung involvement. Both PR3 (p=0,021) and MPO (p=0,009) positive AAVs had higher BVAS score compared to ANCA negatives, while on average there was no significant difference between MPO, PR3 and double positives. PR3 (p=0,007) and MPO (p=0,003) positive AAVs had higher CRP levels than ANCA negatives, and PR3 AAVs had on average higher CRP than MPO AAVs though not statistically significant. There was strong tendency (p=0,087) to PR3 AAVs having more acute tubular damage than other groups and also strong tendency (p=0,092) of having more crescentic formations than MPO AAVs and ANCA negatives but similar to double positives. Though it was not statistically significant ANCA negatives had higher median of IFTA compared to other groups. PR3 AAVs and double positives required significantly more often treatment with PLEX (p=0,042) and dialysis (p=0,04) compared to MPO positive AAVs and ANCA negatives. We then grouped patients into ANCA positives and ANCA negatives. ANCA negative patients were younger (p=0,02), expressed clinicaly more as RLV (p<0,001). BVAS score was lower in ANCA negative group (p= 0,003). ANCA positive patients presented more often with RPGN (p=0,027) and ANCA negatives with nephrotic syndrome. There was tendency of ANCA positives being treated with PLEX more often (p=0,074). In the primary outcome analysis there were no statistically significant differences between serological phenotypes though for relapse rate (p=0,155) curve dynamics through follow up time seems to show higher relapse rate for PR3 and double positive AAVs after 2 years of follow up. Conclusion Serological classification of AAVs is an interesting way for overcoming the limitations of clinical classification. Apart from differences between MPO, PR3 and double positive AAVs, it appears there are even more significant differences between ANCA positive and negative ones.

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P0456LONG TERM PATIENT SURVIVAL AND RELAPSE RATE IN ANCA ASSOCIATED PATIENTS WITH RENAL INVOLVMENET - DATA FROM CROATIAN REFERRAL CENTER

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0456 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Matija Crnogorac ◽

Ana Brechelmacher ◽

Ivica Horvatić ◽

Patricia Kacinari ◽

Miroslav Tišljar ◽

...

Keyword(s):

Relapse Rate ◽

Interstitial Fibrosis ◽

Renal Involvement ◽

Induction Treatment ◽

Tubular Atrophy ◽

Free Survival ◽

Acute Dialysis ◽

Significant Difference ◽

Stage Renal Disease

Abstract Background and Aims The aim of the research was to evaluate patient and renal as well as relapse free survival in ANCA associated vasculitis (AAV) patients in our center. Despite the advances in understanding pathogenesis of AAVs and advances in treatment, the outcomes of AAV patient differ in various centers. Method This study included 106 consecutive AAV patients with renal involvement in the period from 2007-2017. We performed renal biopsy on patients using automatic 16 Gauge needle. Light, immunofluorescent and electronic microscopy were performed. All the patients were treated with cyclophosphamide and steroids in induction treatment with adjuvant PLEX and dialysis depending on renal function and lung manifestations. Primary outcomes were combined outcome progression to end-stage renal disease, defined as persistent (more than three months) need for renal replacement therapy or permanent reduction of EGFR to <15ml/minute (according to CKD EPI formula) and/or death (ESRDD), death (D) and ESRD alone, and disease relapse. Kaplan Meyer survival analysis and multivariate Cox proportional hazard regression analysis were used to explore difference between phenotypes and finding significant predictors regarding outcomes. Out of 106 patients (55,6% female, median age 61; IQR 51-70) there were 66 (61,1%) microscopic poliangitiis (MPA), 20 (18,5%) granulomatosis with angitiis and 20 (18,5%) with renal limited vasculitis (RLV),There were 14 (13%) PR3-ANCA positive patients, 57 (52,8%) MPO ANCA positive, 5 (4,6%) PR3-ANCA+MPO-ANCA positive and 32 (29,6%) ANCA negative patients. Histologically (Berden classification) 43 (39,8%) patients had crescentic, 19 (17,6%) focal, 34 (31,5%) mixed and 12 (11,1%) sclerotic class. Follow up time ranged from 1 to 127 months. Median follow up time was 21 months (IQR = 7-44). Median time to diagnosis was 3 months (IQR 2,0-6,0). Results During follow up 21 (19,8%) patients died, 26 (24,5%) patients reached ESRD and 10 (9,4%) patients relapsed. There was no significant difference in outcomes between clinical, serological or histological phenotypes. In multivariant analysis independent predictors for death were age (HR = 1,059, 95% CI =1,001-1,120; p = 0,046), anemia (HR = 0,952, 95% CI =0,908-0,998; p = 0,040) and BVAS (HR = 1,093, 95% CI =1,030-1,159; p = 0,003), for ESRD. the need for acute dialysis (HR = 4,674, 95% CI =1,996-10,946; p = < 0,001), and interstitial fibrosis and tubular atrophy (IFTA) percentage over 50% (HR = 2,652, 95% CI =1,157-6,081; p = 0,021). and for relapse rate younger age (HR = 0,924, 95% CI = 0,870-0,981; p =0,010), lower serum creatinine levels (HR = 0,996, 95% CI = 0,992-1,000; p = 0,033), and the need for acute dialysis (HR = 59,545, 95% CI =3,467-1022,665; p = 0,005). Event free survival after 12, 24, 36 and 60 months was for death 83,9, 81,2, 79 and 74,7%, for ESRD 80,6, 77,9, 76,1 and 71% and for relapse 95,3, 88,4, 88,4 and 85%. Conclusion Timely diagnosis and treatment can ensure better outcomes in AAV patients. Though there is an overlap in predictive factors between different cohorts, there are still distinctive differences especially between cohorts from clinical trials and those from observational studies. Our study is among few to show significance of anemia as clinical predictor and IFTA percentage as pathohistological predictor.

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

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.345 ◽

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

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All-cause and cause-specific mortality in ANCA-associated vasculitis: overall and according to ANCA type

Rheumatology ◽

10.1093/rheumatology/kez589 ◽

2019 ◽

Vol 59 (9) ◽

pp. 2308-2315 ◽

Cited By ~ 4

Author(s):

Zachary S Wallace ◽

Xiaoqing Fu ◽

Tyler Harkness ◽

John H Stone ◽

Yuqing Zhang ◽

...

Keyword(s):

Causes Of Death ◽

Cox Regression ◽

Renal Involvement ◽

Inception Cohort ◽

Vital Status ◽

Anca Associated Vasculitis ◽

Common Causes ◽

Mortality Incidence ◽

Standardized Mortality Ratios

Abstract Objective The objective of this study was to evaluate causes of death in a contemporary inception cohort of ANCA-associated vasculitis patients, stratifying the analysis according to ANCA type. Methods We identified a consecutive inception cohort of patients newly diagnosed with ANCA-associated vasculitis from 2002 to 2017 in the Partners HealthCare System and determined vital status through the National Death Index. We determined cumulative mortality incidence and standardized mortality ratios (SMRs) compared with the general population. We compared MPO- and PR3-ANCA+ cases using Cox regression models. Results The cohort included 484 patients with a mean diagnosis age of 58 years; 40% were male, 65% were MPO-ANCA+, and 65% had renal involvement. During 3385 person-years (PY) of follow-up, 130 patients died, yielding a mortality rate of 38.4/1000 PY and a SMR of 2.3 (95% CI: 1.9, 2.8). The most common causes of death were cardiovascular disease (CVD; cumulative incidence 7.1%), malignancy (5.9%) and infection (4.1%). The SMR for infection was greatest for both MPO- and PR3-ANCA+ patients (16.4 and 6.5). MPO-ANCA+ patients had an elevated SMR for CVD (3.0), respiratory disease (2.4) and renal disease (4.5). PR3- and MPO-ANCA+ patients had an elevated SMR for malignancy (3.7 and 2.7). Compared with PR3-ANCA+ patients, MPO-ANCA+ patients had a higher risk of CVD death [hazard ratio 5.0 (95% CI: 1.2, 21.2]; P = 0.03]. Conclusion Premature ANCA-associated vasculitis mortality is explained by CVD, infection, malignancy, and renal death. CVD is the most common cause of death, but the largest excess mortality risk in PR3- and MPO-ANCA+ patients is associated with infection. MPO-ANCA+ patients are at higher risk of CVD death than PR3-ANCA+ patients.

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Evaluation of Exercise Capacity In Children with SCD by Six Minute Walk Test

Blood ◽

10.1182/blood.v116.21.2664.2664 ◽

2010 ◽

Vol 116 (21) ◽

pp. 2664-2664

Author(s):

Caterina Minniti ◽

Shoaib Alam ◽

Gregory J. Kato ◽

Mehdi Nouraie ◽

Craig Sable ◽

...

Keyword(s):

Multivariate Analysis ◽

Exercise Capacity ◽

Conflicts Of Interest ◽

Strong Predictor ◽

Univariate Analysis ◽

History Of ◽

Rate Of Decline ◽

Changes Over Time ◽

Minute Walk

Abstract Abstract 2664 Background: The six-minute walk (6MW) test is used in pediatrics in clinical practice and research to determine cardiopulmonary functional status. A change in 6MW may be affected by factors not strictly related to cardiopulmonary function, which may be different in different patient populations. In children and adolescents, age and height has been found to be a strong predictor of 6MW distance. We set out to study the effects of hematological and echocardiographic variables on 6MW distance in children with sickle cell disease (SCD) and its changes over time. Methods: We reviewed prospectively collected hematological, 6MW distance, and echocardiographic data from four hundred children with SCD (including 311 Hb SS or β0) and 69 controls (including 21 Hb AS) enrolled in PUSH (Pulmonary Hypertension and the Hypoxic Response in SCD). Subjects were evaluated at baseline and after 18–24 months, as per protocol. An un-encouraged 6MW was performed in children 5 years or older by trained personnel as per the guidelines of the American Thoracic Society. Subjects were studied at steady state, at least three weeks after any acute exacerbation of SCD. We used ANOVA for univariate analysis and stepwise linear regression for multivariate analysis. Results: Median (interquartile range) 6MW in severe SCD genotype (SS and S-β0) was 444 (399-508) and in controls was 495 meters (435-539) (P = 0.0002), while it was 461 meters (408-518) in milder SCD genotypes (P=0.2 for comparison with severe genotypes) (Table 1). In multivariate analysis, Hb, WBC and history of frequent pain episodes were significantly associated to distance of 6MW. Follow up 6MW obtained in 174 SCD subjects revealed a decline of 10% or more in distance in 22% of subjects with severe genotypes and 33% of other genotypes. The decline was more frequent in the subset of SS subjects with TRV>2.59 (40% vs 19%). CONCLUSION: Six minute walk distance is significantly shorter in children with SCD, even as young as 5 years of age, when compared to age and race appropriate controls, indicating early compromise of exercise capacity. SS and S-β-0 genotype subjects have more impairment of exercise capacity compared to milder genotypes. Predictors of 6MW distance are similar in SCD and non SCD subjects, which validates the use of this test in this patient population. Longitudinal changes in subjects with SCD are similar, with declines in about a quarter of the subjects. Patients with SS who have an elevated TRV have the highest rate of decline in 6MW. These results validate the use of 6MW as a tool for assessing exercise capacity in children with SCD. Disclosures: No relevant conflicts of interest to declare.

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Longer Follow-Up Confirms a Low Relapse Rate after Non-Myeloablative Allogeneic Transplantation (NMT) for Non-Hodgkin’s Lymphoma (NHL), Including Patients with PET or Gallium-Avid Disease.

Blood ◽

10.1182/blood.v106.11.44.44 ◽

2005 ◽

Vol 106 (11) ◽

pp. 44-44 ◽

Cited By ~ 3

Author(s):

Issa F. Khouri ◽

Rima M. Saliba ◽

Ming-Sheng Lee ◽

Grace-Julia Okoroji ◽

Luis Fayad ◽

...

Keyword(s):

Disease Progression ◽

Relapse Rate ◽

Chronic Gvhd ◽

Univariate Analysis ◽

Autologous Transplantation ◽

Conditioning Regimen ◽

Large Cell ◽

Allogeneic Transplantation ◽

Study Entry

Abstract We have used the combination of fludarabine/cyclophosphamide/rituximab (FCR) as a conditioning regimen for NMT in patients (pts) with relapsed NHL (Blood989:3595, 2001). Seventy-eight consecutive pts were treated. Median age was 53 yrs (range, 21–68). Median # of prior chemoregimens was 3. Twenty-one (27%) had failed a prior autologous. Histologies included follicular (FL)=47 pts (60%), diffuse large cell (DLCL)=16 pts (21%) and mantle cell (MCL)=15 pts (19%). Thirty-nine (50%) were in partial remission (PR), five (6%) had stable or progressive disease (SPD) and 34 (44%) were in complete remission (CR) at study entry. Sixty-nine (88%) had a matched sibling, 6 a matched unrelated, and three a mismatched sibling donor. Ten pts had either PET or Gallium-avid disease at transplant: six were in clinical PR and 4 were in SPD. Peripheral blood was the source of graft in 71 pts (91%). Tacrolimus/Methotrexate were used for GVHD prophylaxis. Median time for ANC >500 was 10 days. Sixty pts did not require any platelet transfusion. All but 2 pts who had SPD, converted to CR post transplant. All pts engrafted donor cells. Six had a secondary graft failure (GF), two of which occurred in the setting of disease progression. With a median follow-up time of 34 months (mos) (range, 3 to 70 mos), only 6 pts (8%) relapsed: two pts were in SPD/PET or Gallium(+), 3 were in PR/PET or Gallium(−) and one was in CR at transplant. None of the 6 pts who were PR/PET or Gallium (+) at study entry relapsed. Overall the risk of relapse in PET/Gallium (+) or (−) disease was not significant (P=0.15). Five pts received donor lymphocyte infusion (DLI) for disease progression: one achieved CR; the 4 other pts (two were in GF) did not respond. DLI was also given successfully to two other pts because of cytopenia related to a viral infection (1), and because of decreasing chimerism (1). Overall survival (OS) for all pts was 88%, 82% and 74% at 1,2 and 3-yr, respectively. By univariate analysis, OS was 95% at 3-yr for those who had failed a prior autologous transplant (P = 0.04). Patients with FL histology had also a better 3-yr survival than DLCL [88 % vs 51% (P = 0.008)]; the difference with MCL (65% at 3-yr) was not statistically significant (P = 0.2). Non-relapse related mortality was 11%, 13%, and 19% at 1, 2 and 3-yr, respectively. The incidence of acute II-IV GVHD was 17%. Patients with FL disease had lower incidence of acute II-IV and III-IV GVHD than DLCL [11% vs 31%, P = 0.04; and 2% vs 25%, P = 0.02; respectively] which may have contributed to the higher OS rate in FL. Both FL and DLCL pts had similar age and donor type distribution. Two pts developed chronic GVHD post DLI. The incidence of chronic extensive and limited GVHD was 51% and was not statistically different between various histologies. We conclude that NMT with FCR for NHL is an effective treatment even in pts who failed a prior autologous transplantation and is associated with a low incidence of acute GVHD. The lower than expected relapse rate observed may be indicative that NMT could be curative for these diseases.

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

International Journal of Nephrology ◽

10.1155/2018/6369814 ◽

2018 ◽

Vol 2018 ◽

pp. 1-11 ◽

Cited By ~ 8

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.

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A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature

Case Reports in Rheumatology ◽

10.1155/2017/2421760 ◽

2017 ◽

Vol 2017 ◽

pp. 1-7 ◽

Cited By ~ 4

Author(s):

Richard A. Lau ◽

Ramandeep Bains ◽

Duminda Suraweera ◽

Jane Ma ◽

Emil R. Heinze ◽

...

Keyword(s):

Granulomatosis With Polyangiitis ◽

English Literature ◽

Renal Involvement ◽

Antineutrophil Cytoplasmic Antibody ◽

High Dose ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Proteinase 3 ◽

Anca Associated Vasculitis ◽

Digital Ischemia

This paper describes one patient with Antineutrophil Cytoplasmic Antibody- (ANCA-) associated vasculitis who initially presented with multiple ischemic fingers and toes. On further evaluation, the patient was also found to have pulmonary-renal involvement and episcleritis. The diagnosis was supported with a positive cANCA (anti-proteinase 3) and a bronchoscopy consistent with diffuse alveolar hemorrhage. Although the patient refused a tissue biopsy, clinical presentation including nasal ulceration, sinus congestion, and epistaxis and anti-proteinase 3 antibody were more consistent with Granulomatosis with Polyangiitis (GPA) rather than Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) based on the recently presented ACR/EULAR Provisional 2017 Classification Criteria for GPA (Luqmani et al., 2016). The patient responded well to therapy including high dose steroids and cyclophosphamide, with improvement of all organs involved and had no further digital ischemia or gangrene on follow-up. We include a review of the English literature summarizing presentation, management, and outcome of 16 similar cases.

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Supraventricular arrhythmia in tetralogy of Fallot repair

EP Europace ◽

10.1093/europace/euab116.308 ◽

2021 ◽

Vol 23 (Supplement_3) ◽

Author(s):

V Vincze ◽

A Kardos ◽

L Kornyei ◽

H Balint

Keyword(s):

Atrial Fibrillation ◽

Tetralogy Of Fallot ◽

Supraventricular Tachycardia ◽

Strong Predictor ◽

Univariate Analysis ◽

Supraventricular Arrhythmia ◽

Supraventricular Arrhythmias ◽

Tetralogy Of Fallot Repair ◽

Prospective Database

Abstract Funding Acknowledgements Type of funding sources: Public hospital(s). Main funding source(s): Gottsegen National Cardiovascular Center BACKGROUND With aging morbidity related to arrhythmias in adult patients with Tetralogy of Fallot repair (TOFr) is increasing. OBJECTIVE We aimed to analyze the prevalence of supraventricular tachycardia in these patients using our prospective database. METHODS TOFr data were collected from our prospective database conducted since 2010. Supraventricular arrhythmias (intraatrial reentrant tachycardia (IART), atrial fibrillation, AFib) related complications and therapies were documented. RESULTS Among those with TOFr (n = 296, mean age 34 ± 11) supraventricular tachyarrhythmias (SVT) were present in 41 patients (14%), as following: n = 12 AFib, and n = 29 IART. At the univariate analysis predictors of atrial fibrillation and IART were: age at last follow-up (p < 0,0001), age at first repair (p < 0,0001), number of surgeries (p = 0,014), and tricuspid regurgitation (p = 0,013). Supraventricular tachycardia was a strong predictor of death (OR 3.0). Twenty-five patients had radiofrequency ablation, and after a mean follow-up of 61 ± 56 months, the rate of recurrence for SVT was 32 %. In the non-ablated cohort (treated with amiodarone) 73 % recurrence was detected. CONCLUSION Supraventricular arrhythmias are common in TOFr patients and are associated with increased mortality risk, but arrhythmia control with catheter ablation is superior to anti-arrhythmic drug therapy in this patient population.

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Abstract 19766: Prediction of Clinical Atrial Fibrillation induced during Typical Atrial Flutter Ablation

Circulation ◽

10.1161/circ.130.suppl_2.19766 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Jorge Romero ◽

Rodolfo Estrada ◽

Anthony Holmes ◽

David Goodman ◽

Norman Roth ◽

...

Keyword(s):

Atrial Fibrillation ◽

Atrial Flutter ◽

Multivariate Analyses ◽

Strong Predictor ◽

Univariate Analysis ◽

Anticoagulation Management ◽

Drug Induced ◽

Typical Atrial Flutter ◽

History Of

Background: Atrial fibrillation (AF) and isthmus dependent atrial flutter (AFL) are two separate entities that in many patients coexist. We sought to investigate whether AF inducibility (spontaneous or drug induced) during isthmus AFL ablation predicted the occurrence of AF at follow up after successful AFL ablation. Methods: Two hundred seventy three consecutive patients with isthmus dependent AFL undergoing ablation of AFL at our institution were enrolled in this study. 119 (43%) patients were excluded since they had evidence of AF prior to AFL ablation. Univariate and multivariate analyses were performed. Results: A total of 154 patients (male: 72%, age: 61 ±13) with AFL and without history of AF composed our patient population. All patients underwent successful AFL ablation. During ablation, AF was induced in 28 (18%) patients. After a mean follow up of 34 ± 23.5 months a total of 50 (32%) experienced AF. Univariate and multivariate analyses showed that only age and AF inducibility during AFL ablation were predictors of AF. Univariate analysis (age: p=0.038 and inducible AF p=0.032 and multivariate analysis (age: p=0.011 inducible AF: p=0.016) ) with and adjusted odds ratio of 3.3 [95% CI (1.250-8.676)] (Table 1). A total of 169 (62%) patients experienced AF before or after AFL ablation. Conclusion: AF inducibility in patients undergoing isthmus dependent AFL without history of AF is a strong predictor of AF recurrence. This has an important clinical relevance on anticoagulation management of these patients.

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