Maintenance Of Remission Research Articles

Modificación de la dosis de mesalazina en función de los niveles de calprotectina fecal en pacientes con colitis ulcerosa en remisión clínica

BackgroundFaecal calprotectin (FC) shows an excellent correlation with endoscopic and histological activity of ulcerative colitis (UC) and it is the best predictor of clinical relapse. Our aim was to evaluate the usefulness of modifying the dose of mesalazine based on FC levels, in clinical practice. MethodsRetrospective, single-centre study in UC patients in clinical remission while treated with mesalazine which dosage was decreased (DOWN) or increased (UP) according to FC levels. The main endpoint was the long-term maintenance of clinical remission. ResultsA total of 56 patients were included (39 DOWN, 17 UP). In the DOWN group, the median baseline dose of mesalazine was 3.6g/day and the median baseline FC was 36μg/g. After a median follow-up of 22 months, 28% required rescue therapy. The cumulative relapse-free survival after tapering was 91% and 82% at 12 and 24 months, respectively. In the UP group, the median baseline dose of mesalazine was 2.4g/day, with a median baseline FC of 524μg/g. After a median follow-up of 12 months, 29% required rescue therapy. The cumulative relapse-free survival after dose increase was 86% and 72% at 12 and 24 months, respectively. ConclusionsMesalazine dose modification based on FC monitoring seems to be a safe strategy in patients with UC in clinical remission, with a probability of clinical relapse around 20% at two years.

The use of belimumab in three cases of refractory lupus nephritis.

In recent trials for the treatment of systemic lupus erythematosus (SLE), belimumab (BLM), in addition to standard immunosuppression, has been shown to improve renal and nonrenal outcomes. We report our experience using BLM in three cases of refractory lupus nephritis (LN), where renal remission was not achieved using cyclophosphamide, mycophenolatemofetil and other immunosuppressive medications. In two of the three cases, BLM therapy led to a partial remission of LN, improvement in serological markers of SLE and disease activity, which permitted a reduction in prednisolone dosing. Treatment with efficacious therapies early in the course of LN is a desirable therapeutic strategy, to achieve early remission of proteinuria and curtail the development of irreversible chronic renal damage. Further studies are needed to provide information on the effectiveness of BLM for maintenance of remission, prevention of flares and monitoring for long-term complications of B-cell modulation.

P16 The wood for the trees

P16 The wood for the trees

Real-world effectiveness and safety of ustekinumab in bio-naive patients with moderate-to-severe Crohn's disease: A Canadian multi-center study

BackgroundClinical practice guidelines recommend ustekinumab as a first-line biological treatment option for moderately-to-severely active Crohn's disease (CD). However, there is limited real-world effectiveness and safety data in bio-naïve patients. AimsTo assess ustekinumab effectiveness and safety in bio-naïve CD patients. MethodsMedical charts were reviewed retrospectively at seven Canadian centers. The primary outcome was the proportion of patients achieving clinical remission at Month 6 following ustekinumab initiation. Secondary outcomes included clinical, biochemical, and endoscopic response, and remission at Months 4, 6 and 12. Ustekinumab safety was assessed over the one-year follow-up period. Results158 charts were reviewed. Clinical remission was achieved by 50.0% (36/72), 67.7% (105/155), and 73.7% (84/114) of patients at Months 4, 6, and 12, respectively. At these study timepoints, biochemical remission was observed in 65.2% (43/66), 71.6% (63/88), and 73.9% (68/92) of patients. At Months 6 and 12, endoscopic remission was observed in 40.5% (15/37) and 56.3% (27/48) of patients, respectively. Most participants (93.5%; 145/155) persisted on ustekinumab through Month 12. No serious adverse drug reactions were reported. ConclusionIn this real-world study, ustekinumab presents as an effective first-line biologic for induction and maintenance of remission among bio-naïve Canadian patients with moderately-to-severely active CD.

Long-Term Prognosis of Antimelanoma Differentiation-Associated Gene 5-Positive Dermatomyositis With Interstitial Lung Disease.

Antimelanoma differentiation-associated gene 5 (anti-MDA5)-positive dermatomyositis with interstitial lung disease (DM-ILD) progresses rapidly and has a poor prognosis. Previously, we reported the efficacy of a combination therapy comprising high-dose glucocorticoids (GCs), calcineurin inhibitors (CNIs), and intravenous cyclophosphamide (IV CYC) in a multicenter clinical trial (UMIN000014344). In the present study, we evaluated the long-term outcomes and effects of induction therapy on the maintenance of remission. All participants from our previous trial were followed up for > 5 years. Seventy-three other patients with anti-MDA5-positive DM-ILD from our institute were retrospectively integrated into the previous trial for further analysis. Sixty-eight patients achieved remission and survived for > 6 months. Based on the induction treatment, we classified the patients into 2 groups: (1) group T (n = 56), with triple combination therapy (GCs, CNIs, and IV CYC), and (2) group C (n = 12), with monotherapy/dual therapy. The recurrence-free and drug-withdrawal rates of immunosuppressive agents were compared. The overall survival and recurrence-free survival rates at 5 years were 100% for the participants in the previous trial. The 5-year cumulative withdrawal rates for CNIs and GCs were 70% and 53%, respectively. In a comprehensive analysis, the recurrence-free rates in group T were higher than those in group C (90% vs 56%; P < 0.05). The drug-withdrawal rates of CNIs and GCs at 10 years in group T were also higher than those in group C (79% vs 0% and 43% vs 0%, respectively; P < 0.05). Triple combination therapy in the induction phase can reduce the risk of recurrence and facilitate drug withdrawal in anti-MDA5-positive DM-ILD.

Sustained post-rituximab B-cell depletion is common in ANCA-associated vasculitis and is affected by sex and renal function.

Despite the increasing use of rituximab in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), it remains unclear what the optimal dosing is, especially for maintenance of remission. A deeper understanding of post-rituximab B-cell repopulation patterns may aid better-tailored treatment. This is a monocentric, retrospective study including ANCA-positive AAV patients receiving a single course of rituximab induction. CD19+ B cells were longitudinally monitored with flow cytometry. B-cell repopulation was defined as CD19+ >10 cells/μL. Seventy-one patients were included, the majority with microscopic polyangiitis (75%), myeloperoxidase-ANCA positivity (75%) and with renal involvement (79%). During a median follow-up of 54months since the first rituximab infusion, 44 patients (62%) repopulated B cells, with a median time to repopulation of 39months (range 7-102). Patients experiencing B-cell depletion lasting longer than the overall median time to repopulation (39months) exhibited a lower risk of flare and higher risk of serious infection. In multivariate Cox regression, higher estimated glomerular filtration rate (eGFR) [hazard ratio (HR) 1.84, 95% confidence interval (CI) 1.13-2.98 per 30mL/min/1.73 m2 eGFR] and female sex (HR 2.70, 95% CI 1.37-5.31) were independent predictors of increased rate of B-cell repopulation. A subset of AAV patients develop sustained post-rituximab B-cell depletion, which associates with reduced risk of flare and increased risk of serious infection in the long term. Preserved renal function and female sex are associated with faster B-cell repopulation. These observations further highlight the need to personalize immunosuppression to improve clinical outcomes.

Efficacy and safety of fecal microbiota transplantation in the treatment of ulcerative colitis: a systematic review and meta-analysis

To explore the efficacy and safety of fecal microbiota transplantation (FMT) as a treatment approach for ulcerative colitis (UC), a comprehensive systematic review and meta-analysis of randomized controlled trials was conducted. To collect and evaluate randomized controlled trials of high quality on FMT for UC, we searched a number of databases, including PubMed, Web of Science, Cochrane, Embase, and Medline, for studies published between the establishment of the databases and March 2023. We conducted a meta-analysis of the studies using Review Manager software (version 5.4.1) to determine the differences in rates of remission and adverse reactions between the FMT group and the control group, utilizing the risk ratio (RR) and 95% confidence interval (CI) to combine our findings. A total of 13 randomized controlled trials (RCTs) on the efficacy of FMT in patients with UC were included in the study, in which 580 patients participated, including 293 patients treated with FMT and 287 control subjects. Meta-analysis revealed that clinical remission was significantly better in the FMT group than in the control group [RR = 1.73; 95% CI = (1.41, 2.12); P < 0.00001]; endoscopic remission was significantly better in the FMT group than in the control group [RR = 1.74; 95% CI = (1.24, 2.44); P = 0.001]. Additionally, there were no significant differences in the incidence of adverse reactions between the two groups [RR = 1.00; 95% CI = (0.86, 1.15); P = 0.96]. Fecal microbiota transplantation has shown potential as a therapeutic intervention for inducing clinical remission in ulcerative colitis UC; nevertheless, the attainment of endoscopic remission and the maintenance of long-term remission continue to present challenges. Safety concerns persist throughout the treatment process, necessitating the implementation of measures to augment both safety and success rates.

The use of methotrexate in rheumatoid arthritis. Recommendations of the All-Russian public organization “Association of Rheumatologists of Russia”

Rheumatoid arthritis (RA) is the most frequent immunoinflammatory (autoimmune) rheumatic disease characterized by chronic erosive arthritis and systemic damage to internal organs. The data obtained in the course of basic research on deciphering the mechanisms of action of methotrexate (MT) and the materials of numerous randomized placebocontrolled trials, observational studies and national registries have strengthened the position of MT as the “gold standard” of RA pharmacotherapy and a key component of the “Treatment to Target” strategy. This was the basis for the development of new recommendations of the Association of Rheumatologists of Russia (ARR) concerning the use of MT in RA, according to which MT is considered as the drug of “choice” for induction and maintenance of remission in patients with early and advanced RA, including those who need combination therapy of MT with glucocorticoids, standard Disease-Modifying Antirheumatic Drugs (DMARDs), biologics and targeted synthetic DMARDs. Special attention is paid to the safety of MT therapy and the impact of MT on comorbid pathology associated with cardiovascular complications and interstitial lung disease. Implementation of the ARR recommendations into clinical practice will reduce the risk of disability and improve life prognosis in patients with RA.

Efficacy and Safety of Probiotic versus Conventional Treatment of Irritable Bowel Syndrome

Abstract Background Irritable bowel syndrome is a gastrointestinal syndrome characterized by chronic abdominal pain and a change in intestinal habits without any organic cause. It affects 11% of the world-wide population. irritable bowel syndrome reduces health-related quality of life and leads to a significant economic healthcare burden. Aim of the Work The aim of this study is to investigate the efficacy of probiotics in irritable bowel syndrome (IBS) patients. Patients and Methods A randomized controlled clinical trial in Gastroenterology and Hepatology outpatient clinic at: Ain Shams University Hospitals for 6 months from November 2020 till April, 2021. 120 Person were included in this study divided into 2 group: Group 1:60 patient with irritable bowel syndrome received conventional treatment and and Lactobacillus Acidophilus probiotics. Group 2: 60 patient with irritable bowel syndrome received conventional treatment. Results There was a significant increase of passage of gas in group 1 than in group 2 but no significant difference in between group 1 and group 2 regarding stool consistency, mucus with stool, abdominal pain, Bloating and passage of stools. There was 46.7% in group 1 and 55% in group 2 had a very good treatment success with no significant difference in between. Regarding to the treatment success in group 1, there were 46.7% in group 1 had a very good treatment success, 33.3% had a good treatment success and 20% had poor response. There was 80% in group 1 had a very good overall tolerability to probiotic treatment. Conclusion This study shows moderate evidence for the use and safety of probiotics in irritable bowel syndrome. Probiotics provide a new strategy as a safer treatment for irritable bowel syndrome. In general, the use of Probiotics has been successful in practice, especially for symptom control and maintenance of remission in these entities. However, it is necessary to take into account the specific condition of the patient and their comorbidities to make informed decisions about management.

Crohn's Disease Exclusion Diet for the Treatment of Crohn's Disease: Real-World Experience from a Tertiary Center.

The Crohn's Disease (CD) exclusion diet (CDED) has been shown to induce remission in pediatric and adult patients with CD. In this retrospective cohort study, we describe our real-world experience with the CDED at the inflammatory bowel disease (IBD) unit of the Tel Aviv Medical Center between 2018-2021. CD patients with multiple clinical presentations and disease phenotypes who initiated the diet were included. Indications for treatment, medical and nutritional data were collected from dietician clinic visits and medical records. Clinical and biomarker responses were determined. The CDED was recommended to 220 CD patients. Seventy-two patients were included in the analysis for a clinically active disease (n = 48) or for remission maintenance (n = 24). Among patients with a clinically active disease, 62.5% of patients achieved clinical remission at week 6 and at week 12. A positive association between high adherence to the CDED and clinical remission at week 12 was observed (adjusted OR = 7.6, 95% CI 1.07-55.2, p = 0.043). Among patients treated for remission maintenance, remission at week 12 was maintained among 83.3% of patients. We conclude that the CDED may be a promising intervention for multiple CD presentations and indications. These findings should be further validated in larger, prospective, controlled studies.

Systematic review with meta-analysis: Medical therapies for treatment of ulcerative proctitis.

Ulcerative proctitis (UP) is a common highly symptomatic form of ulcerative colitis that can be difficult to treat. To assess the efficacy of medical treatments for UP. We searched MEDLINE, EMBASE, and CENTRAL on 23 November 2022 for randomised controlled trials (RCTs) of medical therapy for adults with UP. Primary outcomes included induction and maintenance of clinical remission. Pooled risk ratios (RRs) and 95% confidence intervals (CIs) were calculated for each outcome. We included 53 RCTs (n = 4096) including 46 induction studies (n = 3731) and seven maintenance studies (n = 365). First-line therapies included topical 5-aminosalicylic acid (5-ASA), conventional corticosteroids, budesonide, and oral 5-ASA. Therapy for refractory UP included topical tacrolimus and small molecules. Topical 5-ASA was superior to placebo for induction (RR 2.72, 95% CI 1.94-3.82) and maintenance of remission (RR 2.09, 95% CI 1.26-3.46). Topical corticosteroids were superior to placebo for induction of remission (RR 2.83, 95% CI 1.62-4.92). Topical budesonide was superior to placebo for induction of remission (RR 2.34, 95% CI 1.44-3.81). Combination therapy with topical 5-ASA and topical corticosteroids was superior to topical monotherapy with either agent. Topical tacrolimus was superior to placebo. Etrasimod was superior to placebo for induction (RR 4.71, 95% CI 1.2-18.49) and maintenance of remission (RR 2.08, 95% CI 1.31-3.32). Topical 5-ASA and corticosteroids are effective for active UP. Topical 5-ASA may be effective for maintenance of remission. Tacrolimus may be effective for induction of remission. Etrasimod may be effective for induction and for maintenance of remission. Trials should include UP to expand the evidence base for this under-represented population.

Long-term safety and effectiveness of azathioprine in the management of inflammatory bowel disease: A real-world experience.

Azathioprine (AZA) forms the cornerstone for maintenance of sustained remission in inflammatory bowel disease (IBD). There is apprehension regarding the long-term effectiveness and safety of AZA in IBD. We present our experience with AZA use and outcomes in a cohort of IBD patients followed up over a long period of time. Records of 507 IBD patients under treatment at a single, tertiary care center in south India between 2013 and 2022 were evaluated retrospectively. Long-term compliance, tolerance, clinical outcome at the point of last follow-up, type and duration to the onset of adverse events, and subsequent amendment to treatment with regard to AZA were analyzed. Of 507 patients with IBD, 320 patients (207 Crohn's disease [CD], 113 ulcerative colitis [UC]) who received AZA were included. The median follow-up was 41 months (interquartile range 15.5-77.5). Total duration of exposure was 1359 patient-years with median usage of 33 months. Of the patients, 26.9% received AZA for >5 years. Mean initiation and maximum doses of AZA were 0.97 and 1.72 mg/kg/day. Among the participants, 20.6% experienced side effects, including myelotoxicity (7.2%) and gastrointestinal intolerance (5.6%). Six patients developed malignancy. Among the side effects, 39.4% of side effects were dose-dependent. Among the patients, 38.1% had relapses requiring pulse corticosteroid therapy, and 16.2% had more than one relapse after commencement of AZA. AZA was continued till the last follow-up in 76.5%. Among the patients, 49.7% (UC 51.3, CD 48.8) attained durable remission without biologics, and 5.3% continued to have active disease. AZA is safe and effective in the long-term in IBD. Effectiveness, tolerance, and compliance with AZA are well sustained beyond 5 years of usage and comparable between UC and CD.

Predictors and optimal management of tumor necrosis factor antagonist nonresponse in inflammatory bowel disease: A literature review.

Tumor necrosis factor-α (TNF-α) antagonists, the first biologics approved for treating patients with inflammatory bowel disease (IBD), are effective for the induction and maintenance of remission and significantly improving prognosis. However, up to one-third of treated patients show primary nonresponse (PNR) to anti-TNF-α therapies, and 23%-50% of IBD patients experience loss of response (LOR) to these biologics during subsequent treatment. There is still no recognized predictor for evaluating the efficacy of anti-TNF drugs. This review summarizes the existing predictors of PNR and LOR to anti-TNF in IBD patients. Most predictors remain controversial, and only previous surgical history, disease manifestations, drug concentrations, antidrug antibodies, serum albumin, some biologic markers, and some genetic markers may be potentially predictive. In addition, we also discuss the next steps of treatment for patients with PNR or LOR to TNF antagonists. Therapeutic drug monitoring plays an important role in treatment selection. Dose escalation, combination therapy, switching to a different anti-TNF drug, or switching to a biologic with a different mechanism of action can be selected based on the concentration of the drug and/or antidrug antibodies.

In relapsing AAV with rituximab-induced remission, maintenance rituximab vs. azathioprine reduced relapse at ≥32 mo.

Smith RM, Jones RB, Specks U, et al; RITAZAREM co-investigators. Rituximab versus azathioprine for maintenance of remission for patients with ANCA-associated vasculitis and relapsing disease: an international randomised controlled trial. Ann Rheum Dis. 2023;82:937-944. 36958796.

Clinical practice guidelines ofthe Japan Research Committee ofthe Ministry ofHealth, Labour, andWelfare forIntractableVasculitis forthe management ofmicroscopic polyangiitis andgranulomatosis with polyangiitis: The 2023 update - secondary publication.

To revise the 2017 clinical practice guidelines (CPG) for the management of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) to reflect advancements in the field. Similar to the 2017 CPG, the Grading of Recommendations, Assessment, Development, and Evaluation system was adopted for this revision. The intended users of this CPG include patients diagnosed with MPA or GPA in Japan and their families and healthcare professionals, including specialists and non-specialists. Based on a scoping review, four clinical questions (CQs) of the 2017 guidelines were modified, and six new CQs were added. We suggest a combination of glucocorticoid and cyclophosphamide or rituximab for remission induction therapy. In cases where cyclophosphamide or rituximab is used, we suggest the use of avacopan over high-dose glucocorticoid. Furthermore, we suggest against the use of plasma exchange in addition to the standard treatment in severe cases of MPA/GPA. Finally, we suggest the use of glucocorticoid and rituximab over glucocorticoid and azathioprine for remission maintenance therapy. The recommendations have been updated based on patient preference, certainty of evidence, benefit and risk balance, and cost.

Potential for sustaining remission in ankylosing spondylitis patients upon netakimab discontinuation

Introduction. Biological disease modifying drugs (bDMARD) in the treatment of ankylosing spondylitis (AS) have shown good results with the achievement and long-term preservation of remission. There is a discussion about the withdrawal of drugs without loss of effect in order to reduce the economic burden, drug load, adverse events, the possibility of interrupting therapy during surgical treatment.Aim. To evaluate the potential for sustaining the therapeutic effect of netakimab (NTK) after its discontinuation in patients with AS who have achieved remission.Materials and methods. A cohort of 11 patients diagnosed with ankylosing spondylitis (AS) who had achieved remission was included in this study. The patients were closely observed for 52 weeks after discontinuing NTK treatment. AS exacerbations, pain intensity, disease activity scores (BASDAI, ASDAS), enthesitis evaluations (MASES), functional impairments (BASMI and BASFI), C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) levels were documented, as well as radiographic and MRI assessments of the sacroiliac joints and spine were performed.Results. Out of the 11 patients, 5 (45.5%) experienced AS exacerbations within the 12-month observation period. Patients who developed flare-ups had higher baseline levels of BASDAI, ASDAS, BASMI, and CRP at the time of NTK discontinuation. They also had a longer disease duration and were older compared to patients without relapse (p &lt; 0.05). The presence of flare-ups was significantly associated (p &lt; 0.05) with a history of peripheral arthritis, previous treatment with IFN-alpha, and the number of comorbidities. By week 52 of the observation period, patients demonstrated a deterioration in both activity and functional limitations (p &lt; 0.05). Elevated ASDAS-CRP levels were found to be correlated (p &lt; 0.05) with higher radiographic stages of sacroiliitis, the presence of syndesmophytes, functional limitations based on BASMI at the time of drug discontinuation, and the absence of continuous NSAID use. Significant prolongation of remission was associated with a substantial decline in ASDAS-CRP under NTK treatment (rSp = 0.996; p &lt; 0.05), especially among younger patients (rSp = 0.607; p &lt; 0.05).Conclusions. Approximately half of the patients who discontinued NTK therapy after achieving clinical and laboratory remission were able to sustain it. Maintenance of remission for 1 year was more prevalent in younger patients with shorter duration of AS, achieving inactive disease status based on ASDAS-CRP, fewer functional limitations, absence of peripheral arthritis, and comorbidities. Nevertheless, regular patient monitoring is necessary to promptly identify disease recurrence.

Mirikizumab for the treatment of moderate to severe ulcerative colitis.

Despite a growing number of available therapeutic options for ulcerative colitis (UC), up to 50% of patients do not respond to initial treatment or lose response over time, highlighting the need for novel therapies. The IL-23 pathway has emerged as an important therapeutic target for UC. Mirikizumab is a humanized IgG4 monoclonal antibody against the p19 subunit of IL-23, dosed intravenously during induction and subcutaneously during maintenance. It is effective for the induction and maintenance of remission in moderately to severely active UC, including patients with prior failure of biological or tofacitinib therapy. Like other IL-23 antagonists, mirikizumab has a favorable safety profile. It is the first agent of its class to receive regulatory approval for moderately to severely active UC in Europe.

Systematic literature review informing the 2022 update of the EULAR recommendations for the management of ANCA-associated vasculitis (AAV): part 1—treatment of granulomatosis with polyangiitis and microscopic polyangiitis

ObjectiveTo summarise and update evidence to inform the 2022 update of the EULAR recommendations for the management of antineutrophil cytoplasm antibody-associated vasculitis (AAV).MethodsA systematic literature review (SLR) was performed to...

#6071 RITUXIMAB FOR MAINTENANCE OF REMISSION IN ADULT MINIMAL CHANGE DISEASE

Abstract Background and Aims Maintaining remission in a minority of adult patients with minimal change disease remains challenging. Prolonged corticosteroid use is associated with severe adverse effects. A limited number of observational studies suggest that rituximab may be an alternative therapeutic option for remission maintenance, allowing steroid withdrawal. Method We retrospectively analysed the data of all adult patients with minimal change disease, who received rituximab in our centre between 2017 and 2022. Results A total of 14 adults with minimal change disease were treated with rituximab, six of whom had childhood-onset nephrotic syndrome. Six patients were steroid dependent. The median number of relapses was 6.5 (IQR 4.5-15.2) prior to rituximab. Thirteen patients had received at least ≥ 2 different immunosuppressive agents during their disease. Corticosteroid-related adverse events, including osteopenia, hypertension, cataract and diabetes mellitus were observed in 10 subjects. Rituximab was administered in a median time of 10.6 years (IQR 4.5-16.3) after diagnosis. All patients received 2 doses of 1g rituximab 2 weeks apart. The median age at rituximab administration was 31 years (IQR 18.7-49.0); all patients were in remission. Besides corticosteroids, concomitant immunosuppressive therapy included calcineurin inhibitors (n = 6) and mycophenolate acid analogues (n = 1). The median follow-up time after the first dose of rituximab was 44 months (IQR 9.5-67.2). Relapses occurred in 6/14 patients in a median time of 17.5 months (IQR 10.2-26.5). The number of relapses per year decreased to 0.27 from 0.87 (p &amp;lt; 0.001) after rituximab. Six patients received a repeat course of rituximab. One patient developed Pneumocystis Jirovecii pneumonia, that was successfully treated with cotrimoxazole. At the last follow-up visit, corticosteroids were discontinued in 8 out of 14 patients and calcineurin inhibitors in 3 out of 7 patients. Conclusion Rituximab is a reasonable therapeutic approach for maintaining remission and avoiding steroid toxicity in adult minimal change disease. Randomized controlled studies are needed to further evaluate the safety and efficacy of rituximab in these patients.

#6407 RENAL LIMITED VASCULITIS: DATA FROM A CROATION REFERRAL CENTER

Abstract Background and Aims Small vessel vasculitis and in particular ANCA associated vasculitis (AAV) are diseases with variable clinical presentation. Renal limited vasculitis (RLV) can vary in presentation and characteristics compared to systemic disease. There is a tendency to categorise RLV as a part of microscopic polyangiitis (MPA) spectrum though there seem to be differences in terms of both histology and serology between RLV and MPA. We present data on patients with renal limited vasculitis from our referral center. Method This study included 106 consecutive AAV patients with biopsy proven renal involvement in the period from 2007-2017. Category variables were analysed with Fisher Exact testom and continuous with Kruskal-Wallis testom. Statistical difference was then analysed posthoc with Chi-square test. Kaplan Meyer survival analysis and multivariate Cox proportional hazard regression analysis were used to explore difference between clinical phenotypes and finding significant predictors regarding outcomes which were defined as combined outcome end-stage renal disease and death (ESRDD), ESRD alone, the death outcome alone and relapse rate. Results Out of 106 AAV patients with renal involvement in our database: 66 (61,1%) MPA, 20 (18,5%) GPA (granulomatosis with polyangiitis), 20 (18,5%) RLV. In RLV group 14 (70%) were female and average age was 55 (IQR 47-60). Average SCr in RLV group was 234 μmol/l (IQR 168-376,5) and median proteinuria was 3,3g/24h (IQR 0,9-3,8) and most of these patients presented with either nephrotic syndrome (30%) or RPGN (30%). Serologicaly 90% of RLV patients were ANCA negative and 10% were MPO-ANCA positive. Histologicaly (Berden classification) 60% had mixed class, 25% crescentic, 10% focal and 1% sclerotic. Average BVAS score was 14 (IQR 12-17). Compared to MPA and GPA, RLV patients were younger (p=0,058), had less expressed constitutional symptoms (p= 0,013) with lower average BVAS (p=0,0001) and lower CRP levels (p=0,001). Though there was no significant difference of average SCr level RLV patients presented more often with nephrotic syndrome (p=0,014), had histological predominantly mixed class (p=0,006) and higher interstitial fibrosis and tubular atrophy (IFTA) percentage (p=0,086). Interestingly, compared to MPA and GPA majority of RLV patients were ANCA negative (p=0,001). There were no statistically significant differences in treatment used both in induction (including need for dialysis and/or PLEX) or remission maintenance treatment between RLV, MPO and GPA group. In survival analysis there was no statistical differences for all of the outcomes between RLV group, MPA and GPA group. Conclusion RLV can be challenging disease considering it's clinical presentation but also characteristics of renal involvement in terms of more often nephrotic proteinuria and higher IFTA percentage. This might suggest that these patients could have slower disease course (so called slower progressors). Also interestingly in our cohort most of these patients were ANCA negative which also makes diagnosis predominantly a combination of clinical presentation and histology thus prompting a question of how should we classify RLV within a spectrum of small vessel vasculitis. More research is needed to better understand the RLV.