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

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.

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

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Matija Crnogorac ◽  
Ana Brechelmacher ◽  
Ivica Horvatić ◽  
Patricia Kacinari ◽  
Miroslav Tišljar ◽  
...  
Keyword(s):  
Relapse Rate ◽  
Strong Predictor ◽  
Univariate Analysis ◽  
Renal Involvement ◽  
Tubular Damage ◽  
Dialysis Treatment ◽  
Acute Dialysis ◽  
Anca Associated Vasculitis ◽  
Time To Diagnosis

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.

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

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.

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

Rheumatology ◽  
2019 ◽  
Vol 59 (9) ◽  
pp. 2308-2315 ◽  
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.

scholarly journals Efficacy and Safety of Eltrombopag with or without Immunosuppressant in Patients with Relapsed/Refractory Acquired Aplastic Anemia: A Real-World Experience

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 1124-1124
Author(s):  
Jiang Ji ◽  
Ziqi Wan ◽  
Jing Ruan ◽  
Yali Du ◽  
Miao Chen ◽  
...  
Keyword(s):  
Adverse Events ◽  
Aplastic Anemia ◽  
Median Time ◽  
Relapse Rate ◽  
Real World ◽  
Reticulocyte Count ◽  
Efficacy And Safety ◽  
Overall Response ◽  
Significant Difference

Abstract Background: Eltrombopag (EPAG) with or without immunosuppressant (IST) has been applied in acquired aplastic anemia (AA), yet data of EPAG+IST in relapsed/refractory AA was limited, and no study has compared efficacy and safety between EPAG+IST and EPAG monotherapy in relapsed/refractory AA patients. Aims: To evaluate and compare the efficacy and safety between EPAG+IST and EPAG monotherapy in relapsed/refractory AA patients in a real-world setting. Methods: Data from patients diagnosed as acquired AA in our center were retrospectively collected. All the enrolled patients were refractory/relapsed to the standard IST for at least 6 months before EPAG. All patients had been treated with EPAG, which was started at 25 mg/day and increased every 2 weeks to a maximum of 150 mg/day until a best response was achieved. Meanwhile, some patients were treated with cyclosporin A (CsA) or tacrolimus (FK506) at the same time. EPAG had to be prescribed for at least 6 months before evaluation. Complete response (CR), overall response (OR) and relapse rate, as well as adverse events and factors which could affect efficacy were analyzed. Results: Totally 99 patients (83 non-severe AA (NSAA) and 16 SAA) were included in the study. The median age at EPAG initiation was 46 (13-88) years old, the median time of EPAG treatment was 11 (6-41) months and the median time of follow-up was 18 (6-41) months. 72 patients were treated with EPAG+IST, including 41 (56.9%) treated with EPAG+FK506 and 31 (43.1%) treated with EPAG+CsA. 27 patients were treated with EPAG alone. No significant difference was found between EPAG+IST group and EPAG group in patient baseline characteristics like age, male proportion, NSAA proportion, presence of PNH clone, proportion of previous ATG+CsA / CsA treatment, previous IST duration and dosage. With compatible follow-up time, EPAG exposure duration and dosage, there was no significant difference in OR/CR rate at 3 rd/6 th/12 th month between patients who was treated with EPAG+FK506 and EPAG+CsA. Under similar compatible baseline conditions, the OR rate was 33.3% vs 22.2% (P=0.284) at 3 rd month, 61.1% vs 37.0% (P=0.032) at 6 th month, and 67.2% vs 42.1% (P=0.051) at 12 th month for patients treated with EPAG+IST and EPAG alone, respectively, but no significant difference was found in time to response (3 (1-12) vs 3 (1-7) months, P=0.679) or CR rate at 3 rd/6 th/12 th month (6.9%/12.5%/20.7% vs 3.7%/7.4%/5.3%, P&gt;0.05) between the two groups. Relapse occurred at 6 th to 12 th month of EPAG treatment, and the relapse rate at 12 th month was 9.8% and 27.3% (P=0.154) for patients treated with EPAG+IST and EPAG alone, respectively. For patients treated with EPAG+IST, responders had a significantly higher baseline reticulocyte count (60.25 (11.5-230.5)×10 9/L vs 16.7 (6.6-56.6)×10 9/L, P=0.040) compared with non-responders. No predictive factors for the overall response were found for patients treated with EPAG alone. Adverse events which led to dosage regulation were gastrointestinal disorders (2.8% vs 3.7%, P=1.000), elevated creatinine (2.8% vs 0, P=0.599), elevated ALT (1.4% vs 0, P=1.000) and arthralgia (0 vs 3.7%, P=0.280) for patients with EPAG+IST and EPAG, respectively. No deaths were found in either group, while the clone evolution rate was 2.8% and 3.7% (P=1.000) in EPAG+IST and EPAG monotherapy group, respectively. Conclusion: EPAG+IST had higher OR rate than EPAG monotherapy with similar side effects for patients with relapsed/refractory acquired AA. Those with higher baseline reticulocyte count were more likely to respond to EPAG+IST. Key words: relapsed/refractory, aplastic anemia, eltrombopag, immunosuppressant, efficacy Disclosures No relevant conflicts of interest to declare. OffLabel Disclosure: In the presented study, eltrombopag was prescribed in relapsed/refractory aplastic anemia patients.

scholarly journals Significance of Linked Color Imaging for Predicting the Risk of Clinical Relapse in Ulcerative Colitis

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Shuji Kanmura ◽  
Akihito Tanaka ◽  
Kazuki Yutsudou ◽  
Kosuke Kuwazuru ◽  
Fukiko Komaki ◽  
...  
Keyword(s):  
Ulcerative Colitis ◽  
Relapse Rate ◽  
Additional Treatment ◽  
Chronic Inflammatory Bowel Disease ◽  
Unknown Etiology ◽  
Clinical Relapse ◽  
Color Imaging ◽  
Significant Difference ◽  
Linked Color Imaging

Ulcerative colitis (UC) is a chronic inflammatory bowel disease with unknown etiology. Recently, mucosal healing has emerged as an important therapeutic endpoint in UC. Linked color imaging (LCI) is a novel endoscopic system that enhances the color differences of the gastrointestinal mucosa. Our previous study emphasized the redness and yellowness of the lesion using LCI observation, which was useful for the evaluation of histological mucosal activity in UC. In this study, we aimed to evaluate the correlation between LCI observation and clinical relapse rate in UC patients. We retrospectively analyzed UC patients who underwent total colonoscopy between August 2016 and October 2018 at our facility with Mayo endoscopic scores of 0 or 1. We assessed the correlation between orange-like color lesion (defined as LCI-scarlet color lesions) and clinical relapse rate (requiring additional treatment for UC) during the 1-year follow-up period. Fifty-eight patients (22 female, 36 male; median age at diagnosis, 47.2 (18–80) years) who underwent colonoscopy were analyzed. During the 1-year follow-up period, clinical relapse was observed in 12 patients (20.1%) among which ten patients (83.3%) had an LCI-scarlet color lesions recognized by LCI. By contrast, 29 patients (63%) had no LCI-scarlet color lesions in the clinical remission group (n=46). There was a significant difference in LCI-scarlet color between the clinical relapse and remission groups, remaining significantly associated with clinical relapse. LCI findings, including an orange-like color lesion, have diagnostic implications for predicting the risk of clinical relapse in UC during the 1-year follow-up period.

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 SO058IGG4-RELATED KIDNEY DISEASE : A FRENCH NATIONWIDE RETROSPECTIVE COHORT STUDY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Anis Chaba ◽  
Mohamad Zaidan ◽  
BOFFA Jean Jacques ◽  
KARRAS Alexandre ◽  
Audard Vincent ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Serum Creatinine ◽  
Corticosteroid Therapy ◽  
Renal Involvement ◽  
Renal Lesion ◽  
Serum Igg4 ◽  
Significant Difference ◽  
The Mean ◽  
Glomerular Involvement

Abstract Background and Aims IgG4 disease is a systemic fibroinflammatory disorder that may affect virtually any organ. IgG4-related kidney disease (IgG4-RKD) is a major manifestation, occurring in almost one third of patient. The present study addresses the diagnosis, management and outcome of French patients with IgG4-RKD. Method We conducted a retrospective observational study including patients with renal impairment due to IgG4-RKD from 32 centers. Clinical, biological, and imaging data, as well as management modalities and outcome, were retrieved from medical records. Results 74 patients, 64 males and 10 females, were diagnosed with IgG4-RKD between 1997 and 2019 and were included in the present study. The mean age at diagnosis was 65.2 ± 15-year-old. Renal involvement was present at diagnosis in 63% of cases. Nine (12%) patients had isolated renal involvement, whereas extra-renal involvement included retroperitoneal fibrosis (20%), auto-immune pancreatitis (49%), cholangitis (27%), and lung disease (22%). Renal imaging lesions were observed in 48 (64%) patients, and included renal hypertrophy, patchy lesions and pseudo-tumor solitary renal lesion. PET-CT scan was performed in 47 (65%) patients and showed hypermetabolic renal lesion(s) in 36% of cases. The presence of extra-renal hypermetabolic lesions was observed in 35 cases (74%). 43 %, 25% and 14% of patients presented with AKI, AKI-on-CKD, and isolated CKD, respectively. The mean serum creatinine level at diagnosis was 244 ± 140 µmol/L, corresponding to an eGFR of 69 ± 22ml/min/1.73m. Urinary sediment was most often bland. Mean urine protein-to-creatinine ratio was 1.27 g/g. of note, 26% of patients had more than 1 g/g. 82% of patients underwent a kidney biopsy showing tubulointerstitial involvement in all cases, 12 (16%) had additional glomerular involvement, mainly membranous nephropathy. The major additional laboratory abnormalities included polyclonal hypergammaglobulinemia, and increased serum IgG4 increase in 83% and 86%, respectively. Complement levels were decreased in 27 (37%) patients. Two patients were lost for follow-up and 69 patients were treated with corticosteroid therapy in 66 (89%) patients and 10 received Rituximab as first line therapy. The mean duration of follow-up was 28.8 ± 30 months. During follow-up, 22 (30%) patients relapsed, while 45 (62%) patients had persistent CKD at last follow-up, with a mean eGFR of 47±27 ml/min/1.73m. Seven (9%) patients progressed to ESRD and 7 died. A significant response was achieved in 46 (62%) patients. Patients who received steroids higher than 0.5mg/kg/day had lower serum IgG4 levels (1.8 g/l versus 5.7 g/l, p=0.007) at last follow up but there was no significant difference observed in terms of renal function between the two groups (45.5 vs 48.8 ml/min/1.73m, p= 0.72). By univariate analysis, predictors of relapse were low C3 or C4 serum level (p=0.01), ANCA positivity (p= 0.02), renal radiological lesions (p=0.05). At last follow-up the eGFR was worse in patients with a serum creatinine diagnosis greater than 300 µmol/L (30.25 vs 57 ml/min/1.73m p=0.0001) and more progressed to ESRD (p=0.03). Conclusion IgG4-RKD has been recently described and may affect middle-aged males. The disease presents as tubulointerstitial nephritis with glomerular involvement in 16% of cases. Disease response to corticosteroid therapy is favorable but is characterized by a high rate of relapses and a significant risk of persistent CKD in the majority of patient. Risk of ESRD and death in almost 20% of patients.

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

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
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.

scholarly journals Kidney Pathology and Outcomes in Anca-Associated Vasculitis: Retrospective Analysis of 85 Patients

2021 ◽  
Author(s):  
Elena Zakharova ◽  
Anastasiia Zykova ◽  
Tatyana Makarova ◽  
Eugenia Leonova ◽  
Ekaterina Stolyarevich
Keyword(s):  
Interstitial Fibrosis ◽  
Significant Risk ◽  
High Risk Group ◽  
Additional Parameter ◽  
Tubular Atrophy ◽  
Anca Associated Vasculitis ◽  
Pathology Classification ◽  
Ckd Stage ◽  
Significant Difference

ANCA-associated vasculitis (AAV) pose a significant risk of kidney failure, kidney biopsy remains a key prognostic tool. Pathology classification of the AAV glomerulonephritis (GN) developed by Berden et al showed correlation between GN classes and kidney outcomes; ANCA Renal Risk Score (ARRS) included tubular atrophy and interstitial fibrosis (TA/IF) as an additional parameter for risk assessment. We aimed to evaluate kidney survival across AAV GN classes and ARRS groups. A single-center retrospective study included 85 adult patients with biopsy-proven AAV kidney disease followed in 2000-2020. Primary outcome was kidney survival at the end of 18 [5; 66] months follow-up, kidney death considered as CKD stage 5. We found significant difference in the kidney survival for sclerotic, mixed, crescentic and focal AAV GN classes: 19%, 76.2%, 91.7% and 100% respectively (p=0.009). Kidney survival was 0%, 75.6% and 100% for the high, median and low risk ARRS groups respectively (p&amp;lt;0.001); TA/IF analysis showed kidney survival 49.6% vs 87.7% for widespread and mild TA/IF respectively (р=0.003). Kidney survival was significantly lower in anti-MPO-ANCA versus anti-PR3-ANCA carriers (50.3% and 78.1% respectively, р=0.045). We conclude that unfavorable AAV kidney outcomes associated with sclerotic GN class by Berden&rsquo;s classification, ARRS high risk group, and anti-MPO-ANCA subtype.

Sign in / Sign up

Export Citation Format

Share Document