Renal-limited Vasculitis Research Articles

Aneurysmal rupture in microscopic polyangiitis: a case-based review.

Microscopic polyangiitis (MPA) affects small and medium vessel, which sometimes leads to arterial aneurysms. In English database, only 15 reports refer to ruptured aneurysms in MPA. We experienced a fatal case with MPA who developed multiple visceral aneurysms, resulting in rupture of the hepatic aneurysm. For the better knowledge of aneurysmal rupture in MPA, we reviewed the feature of 16 cases, including our case. Organ involvement observed was glomerulonephritis 100%, pulmonary involvement 25%, peripheral neuropathy 25%, and purpura 12.5%. Locations of ruptured aneurysms were left gastric artery 31.25%, renal and hepatic artery 18.75% each, intracranial and splenic artery 12.5% each, and gastroepiploic and mesenteric artery 6.25% each. Median time to rupture was 45days after systemic symptom onset, and 15days after immunosuppressive treatment induction. Symptoms at rupture were visceral pain 68.75% and hemodynamic instability 62.5%. Pathological findings of ruptured aneurysms were acute vasculitis in 5, no evidence of active inflammation in 3. Causes of death were aneurysmal rupture in 5, treatment complications in 3, and total mortality rate was 50%. In conclusion, the initial presentation of MPA resulting in ruptured aneurysms tends to be renal-limited vasculitis. Aneurysms of abdominal medium-sized arteries tend to rupture, from 4weeks after systemic symptom onset to 2weeks after immunosuppressive treatment induction. Most aneurysms are less than 10mm in diameter, develop asymptomatically in a few days, and are recognized when they rupture. Early induction of immunosuppressive treatment has the potential to shrink aneurysms and prevent rupture.

Glucocorticoid doses according to tissue/organ involvement in ANCA-associated vasculitis

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are a group of disorders that include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), renal-limited vasculitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Infections and cardiovascular diseases are the main causes of death in patients with AAV. New treatment regimens with low-dose glucocorticoidsare proposed to reduce the frequency of side effects. Studies related to the use of glucocorticoids in AAV patients were searched in the literature and results were summarized. Low-dose steroid protocols are not inferior to standard dose treatment and have fewer side effects. Although their effectiveness appears to be similar and their side effects appear to be less, the long-term results of low-dose regimens should also be evaluated.New studies are needed for alternative treatment regimens.

First Reported Case of Rapidly Progressive Glomerulonephritis (RPGN) Due to Combined Renal-Limited ANCA-Associated Vasculitis and Anti-glomerular Basement Membrane (GBM) Disease in a Young Man with Koolen-de Vries Syndrome

First Reported Case of Rapidly Progressive Glomerulonephritis (RPGN) Due to Combined Renal-Limited ANCA-Associated Vasculitis and Anti-glomerular Basement Membrane (GBM) Disease in a Young Man with Koolen-de Vries Syndrome

Myeloperoxidase-ANCA IgG induces different forms of small vessel vasculitis based on type of synergistic immune stimuli

Anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis has diverse patterns of injury including microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Necrotizing and crescentic glomerulonephritis (NCGN) occurs in all syndromes and as renal limited vasculitis (RLV). Single dose intravenous ANCA IgG-specific for mouse myeloperoxidase (MPO) causes RLV in mice. Although multiple mouse models have elucidated ANCA-IgG induced necrotizing and crescentic glomerulonephritis (NCGN), pathogenesis of ANCA-induced granulomatosis and vasculitis outside the kidney has not been clarified. To investigate this, we used intravenous MPO-ANCA IgG in the same strain of mice to induce different patterns of lung disease mirroring patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Repeated intravenous MPO-ANCA IgG induced GPA with NCGN, lung capillaritis, arteritis and granulomatosis. Lung leukocyte phenotypes were evaluated by immunohistochemical image analysis and by flow cytometry. ANCA lung capillaritis and microabscesses began within one day and evolved into granulomas in under seven days. Influenza plus single dose MPO-ANCA IgG induced MPA with NCGN, lung capillaritis and arteritis, but no granulomatosis. Allergic airway disease caused by house dust mites or ovalbumin plus single dose intravenous MPO-ANCA IgG induced EGPA with eosinophilic bronchiolitis, NCGN, capillaritis, arteritis, and granulomatosis. Thus, our study shows that the occurrence and pattern of lung lesions are determined by the same ANCA IgG accompanied by different synergistic immune factors.

A case of renal limited myeloperoxidase anti-neutrophil cytoplasmic antibody-positive vasculitis treated with maintenance avacopan monotherapy.

A 76-year-old woman was admitted with progressive renal function decline. A kidney biopsy was performed because of myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA; 333IU/mL), proteinuria (1.21g/d), and urinary erythrocyte sediment (10-19/high-power field). Renal-limited ANCA-positive vasculitis with pauci-immune necrotizing crescentic glomerulonephritis (ANCA-associated vasculitis, AAV) was diagnosed. Glucocorticoid therapy was started, and the patient responded well. About 1year later, avacopan treatment was started and glucocorticoid therapy was discontinued. Avacopan did not normalize ANCA levels and did not make urinary findings negative. However, further progression of renal function decline is prevented. Factors attributed to the development of AAV in this case were investigated; AAV developed after the second dose of the COVID-19 vaccine and ANCA levels re-elevated after the fifth dose. This suggests that the COVID-19 vaccine may have contributed to the development of AAV in this elderly patient. Avacopan monotherapy has been shown to be effective as maintenance therapy to control the progression of renal failure although not sufficient for complete remission of AAV.

#1782 Clinicopathological study of renal limited pauci-immune vasculitis and its short-term outcome: a single centre observational study

Abstract Background and Aims Pauci immune crescentic glomerulonephritis, is a leading cause of RPGN. The majority of patients with PICGN have glomerular diseases as a part of a systemic small vessel vasculitis, a minority may present as renal-limited vasculitis. In north-east India, there are still no major studies in this group of patients. Therefore, our study involving north-east Indian population constitutes an effort to prospectively study the clinico-pathological feature of patients with PICGN and assess the short-term outcome. Method This single-centre, prospective, observational, study was conducted over 12 months at the Department of Nephrology, Gauhati Medical College. All renal biopsy-proven patients with pauci-immune renal vasculitis were included, excluding those with secondary vasculitis. Baseline characteristics and laboratory data were recorded, and disease activity was assessed using the Birmingham Vasculitis Activity Score. The ANCA tests were performed using a standardised essay for both indirect immunofluorescence assay and antigen-specific ELISA. The renal biopsy specimen obtained was evaluated by light microscopy and direct immunofluorescence. The sample size was calculated based on the prevalence of pauci-immune glomerulonephritis, and data were analysed using SPSS. Results The study involved 24 patients, predominantly females (70.83%), aged 14-65 years, presenting predominantly with pedal oedema and oliguria. Laboratory findings showed varying levels of haemoglobin, total count, platelet count, creatinine, and 24-hour urine protein. 50% of the patients were (MPO) positive, 33.3% were (PR3) positive while 16% were ANCA negative. Renal biopsy analysis showed a majority with mixed class according to the Berden classification. Treatment involved induction with Methylprednisolone and Cyclophosphamide, with dialysis administered to 58.3% of patients. At six months, 36.3% had a stable GFR, and 50% were on maintenance hemodialysis. Factors predicting renal loss included oliguria, dialysis at presentation, and fibrous/fibrocellular crescents. The best predictor of renal outcome was serum creatinine at presentation. Conclusion Pauci-immune CrGN is a severe disease affecting a wide age range. Early diagnosis and treatment initiation are crucial for better outcomes, including lower ESRD risk and relapse rates. Prognostic factors include baseline serum creatinine levels and fibrous crescents. Optimising treatment regimens may improves outcomes.

#2291 ANCA negative vasculitis—the unmet need within small vessel vasculitis

Abstract Background and Aims ANCA negative small vessel vasculitis with renal involvement has been described in a number of cohorts. Though comprehensive studies comparing these individuals to ANCA positive patients are lacking and they are routinely excluded from clinical trials. Whilst the numbers are smaller than those with ANCA positivity, they still make up a large proportion of small vessel vasculitidies (Fig. 1). The absence of detectable circulating ANCA is thought to indicate a distinct spectrum of the disease. There remains unanswered questions regarding the pathophysiology, diagnosis and management of patients with seronegative AAGN. We present data from Croatian referral center. Method Study included 106 AAV patients with biopsy proven renal involvement in the period from 2007-2017. Category variables were analysed with Fisher Exact test and continuous with Kruskal-Wallis test. Statistical difference was then analysed post hoc 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 106 AAV patients with renal involvement were included: 66 (61.1%) MPA, 20 (18.5%) GPA, 20 (18.5%) RLV. 14 (13%) were PR3-ANCA positive, 57 (52.8%) MPO ANCA positive, 5 (4.6%) PR3-ANCA+MPO-ANCA and 32 (29.6%) ANCA negative patients. ANCA negative patients were comparatively younger (p = 0.02) and presented with more renal limited vasculitis (RLV; p < 0.001), lower BVAS (p = 0.003) and lower CRP (p = 0.001). ANCA negative patients usually presented with nephrotic syndrome compared to positive ones which presented with rapid progressive nephritic syndrome or acute nephritic syndrome (p = 0.027). 24-hour proteinuria levels were higher in ANCA negative group (p = 0.003). Though statistical difference was not reached, there was tendency for ANCA negatives to receive less plasmapheresis (p = 0.074). Histologically there were no differences between ANCA positive and ANCA-negative patients. On sub analysis ANCA negatives versus individual seropositive groups, there was tendency that ANCA negatives and MPO-ANCA patients had lower percentage of glomerular crescents (p = 0.092) but more acute tubular damage (p = 0.087). There were no differences in clinical outcomes between the groups. Conclusion ANCA negative vasculitis form a large proportion of small vessel vasculitidies and present with different clinical laboratory and histological (renal) characteristics than ANCA positive ones. More often than not disease seems to be renal limited and patients tend to be younger. Due to the rarity of the disease, there remains unanswered questions regarding the pathophysiology, diagnosis and management of these patients. This study highlights noteworthy distinctions between the two groups. Whether these differences warrant adjustments in treatment algorithms or a reconsideration of the classification of small vessel vasculitis remains to be determined. Further investigation into the pathogenetic mechanisms of seronegative AAVs is essential.

The predictive performance of the ANCA renal risk score in patients over 65 years of age with renal ANCA-associated vasculitis.

The anti-neutrophil cytoplasmic antibody (ANCA) renal risk score (ARRS) for predicting renal survival in ANCA-associated vasculitis (AAV) had not previously been validated in adults over 65 years of age and presenting impairments associated with an aging kidney, a high cardiovascular comorbidity burden and prevalent microscopic polyangiitis. We retrospectively studied a cohort of 192 patients over 65 years of age [median (interquartile range) age: 73 (68-78) years], including 17.2% with renal-limited vasculitis, 49.5% with microscopic polyangiitis and 33.3% with granulomatosis with polyangiitis, at six centres in northern France. The primary study endpoint was the cumulative incidence of end-stage kidney disease (ESKD, maintenance of dialysis for at least 3 months) at 12 months, with death considered as a competing event. The median serum creatinine concentration at diagnosis was 300 (202-502) µmol/L, and 48 (25.0%) patients required dialysis at presentation. The ARRS was high in 43 (22.4%) patients, medium in 106 (55.2%) and low in 43 (22.4%). The cumulative incidence of ESKD at 12 months was 0% in the low-risk group, 13.0% (interquartile range 7.6-20.0) in the medium-risk group and 44.0% (29.0-58.0) in the high-risk group (P<.001). In the subgroup of 149 patients presenting a medium or high score, the ARRS had a C-index of 0.66 (0.58-0.74) for the prediction of ESKD at 12 months; this rose to 0.86 (0.80-0.90) when dialysis status at diagnosis was included. The ARRS was a poor predictor of kidney survival at 12 months among patients over 65 years of age with renal AAV involvement-especially in the high ARRS group. The addition of dialysis status at diagnosis as an additional clinical parameter might improve the predictive performance of the ARRS.

Renal Limited Anti-Neutrophil Cytoplasmic Antibody Associated Vasculitis: A Case Report

Background: Renal Limited Vasculitis (RLV) is a localized autoimmune vascular inflammatory disorder that is part of the Pauci Immune Glomerulonephritis (PIGN) spectrum. Over 90% of PIGN patients have circulating Anti-Neutrophil Cytoplasmic Antibodies (ANCA Ab). Anti-Neutrophil Cytoplasmic Antibody (ANCA) associated vasculitides are a heterogeneous group of multisystemic autoimmune disorders with distinct pathological findings. They are characterized by widespread inflammation of vessels according to their size, their location, and their serotypes based on the presence or absence of ANCA antibodies, namely Myeloperoxidase (MPO-ANCA), Proteinase-3 (PR3-ANCA) or simply ANCA negative, if no antibodies are found. RLV is characterized by antibody positivity to myeloperoxidase in the majority of cases. Case Report: A 77-year-old Caucasian female was admitted with altered mental status and laboratory evidence of renal function compromise consistent with non-oliguric acute renal failure. Imaging and renal biopsy resulted in a diagnosis of pauci-immune glomerulonephritis consistent with MPO-ANCA vasculitis with a subtype that was associated with necrotizing arteritis, a severe form of the disease. She was placed on hemodialysis, intravenous cyclophosphamide, and oral prednisone, and entered remission. Conclusion: A high level of suspicion and familiarity with clinical signs and symptoms are critical in distinguishing primary RPGN subtypes from other types of ANCA-associated small vessel vasculitis. If left untreated, renal limited vasculitis can progress to widespread systemic involvement and become fatal.

Vasculite restrita aos rins em criança de 8 anos: Causa rara de glomerulonefrite rapidamente progressiva evoluindo para doença renal crônica terminal

Glomerulonephritis is one of the main renal involvements in the pediatric age group, usually secondary to infection with group A beta hemolytic streptococcus. However, although rare, ANCA-related vasculitis can also be a cause of glomerulonephritis. The aim of this report is to describe a case of glomerulonephritis secondary to ANCA-related vasculitis, given its low incidence in childhood and the rapid evolution to chronic nephropathy in this case. L.E.F, 8 years old, female, mixed race, sought care due to macroscopic hematuria, anasarca, headache and arthralgia. On admission, he presented a hypertensive crisis, and in laboratory tests, a significant increase in urea and creatinine; Positive P-ANCA and anti-MPO. Hemodialysis, antihypertensives and pulse were started with Methylprednisolone (30mg/kg/day), followed by prednisolone at a dose of 2mg/kg/day. In addition, renal biopsy showed glomerular sclerosis and negative immunofluorescence. Diagnosis of Restricted Kidney Vasculitis was performed. She evolved stable, but with no improvement in her kidney function, requiring her to remain on hemodialysis and being referred to a kidney transplant outpatient clinic. Related ANCA vasculitis are divided into Granulomatosis with Polyangiitis, Eosinophilic Granulomatosis with Polyangiitis, Microscopic Polyangiitis, and Restricted Kidney Vasculitis. They are rare in the pediatric age group, but it is important to know about them, both in relation to their involvement of the upper and lower airways, as well as their renal involvement, so that the diagnosis can be made earlier, making the treatment and prognosis favorable.

Antineutrophil cytoplasmic antibody-associated vasculitis classification by cluster analysis based on clinical phenotypes: a single-center retrospective cohort study.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a group of small vessel inflammatory disorders. Overlapping clinical phenotypes of AAV subgroups continually provoke controversies over their diagnostic and classification criteria. Using the agglomerative hierarchical clustering method, we classified 210 Korean patients diagnosed with AAV into mutually exclusive clusters according to Birmingham Vasculitis Activity Score items, ANCA specificity, sex, and age. We analyzed the resulting clusters' outcomes to investigate the clinical significance of the classification. We proposed a distance-based algorithm of patient assignment and explored its clinically relevant modification. In total, 116 patients (55%) had microscopic polyangiitis, 53 (25%) had granulomatosis with polyangiitis, and 42 (20%) had eosinophilic granulomatosis with polyangiitis. Our model grouped the patients into five clusters, namely, "limited proteinase 3 (PR3)-ANCA vasculitis," "generalized PR3-ANCA vasculitis," "ANCA-negative vasculitis," "renal-limited vasculitis," and "myeloperoxidase-ANCA vasculitis." Patients clustered under "generalized PR3-ANCA vasculitis" had a higher relapse rate (hazard ratio [HR] = 2.12, P = 0.067). The incidence of end-stage renal disease was higher in patients belonging to the "renal-limited vasculitis" cluster (HR=1.50, P=0.03), and those in the "ANCA-negative vasculitis" cluster experienced a relatively milder clinical course of AAV (mortality = 0). Because the clusters were naturally derived from their distinguished phenotypes and have different clinical courses, our clustering method may be a more clinically relevant classification system for AAV, revealing its phenotypic diversity. We also proposed a simple and intuitive distance-based assignment algorithm, which can be easily modified according to specific clinical needs. Key Points • In this study with a single-center AAV cohort, we showed that AAV can be divided into five distinct subclasses with different disease courses based on the clinical and laboratory features of the patients. • Our study revealed ethnic differences in AAV manifestation and suggests that physicians may need to analyze their own AAV patients to assess the disease status of AAV patients. • We proposed a distance-based cluster membership assignment method that can be clinically modified to fit the specific purpose of grouping 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.

Monitoring disease activity in antineutrophil antibody-associated vasculitis.

Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) comprises a group of multisystem disorders with alternating periods of relapse and remission. Beyond that, a smouldering progress during apparently clinically silent phases often develops. AAVs are subgrouped in microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and renal limited vasculitis (RLV). ANCA are hallmark of this disease entity, although they are not always present. Despite the simplification of treatment, fundamental aspects concerning assessment of its efficacy and its adaptation to encountered complications or to the relapsing/remitting/subclinical disease course remain still unknown. Through the advances in pathogenesis and pathophysiology of AAV a reliable biomarker-based monitoring and treatment algorithm has not been established and disease management follows not infrequently a "trial and error" approach. Here, we overviewed the most interesting biomarkers reported so far.

142 A Case of Hydralazine Induced Severe Renal-Limited ANCA Vasculitis Leading to ESRD in a 63-Year-Old Man

142 A Case of Hydralazine Induced Severe Renal-Limited ANCA Vasculitis Leading to ESRD in a 63-Year-Old Man

Patient-reported outcomes in ANCA-associated vasculitis: a cross-sectional study to explore the interactions between patients' and physicians' perspectives.

To evaluate associations between the domains of the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) instrument and clinical variables. Patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), or renal-limited vasculitis (RLV) were recruited from a tertiary care center in Mexico City. Demographic, clinical, serological, and treatment-related data were retrieved. Disease activity, damage, patient and physician global assessments (PtGA and PhGA) were evaluated. All patients completed the AAV-PRO questionnaire, male patients also completed the International Index of Erectile Function (IIEF-5) questionnaire. Seventy patients (44 women and 26 men) were included, with a median age of 53.5years (43-61), and a disease duration of 82months (34-135). Moderate correlations were identified between the PtGA and the AAV-PRO domains: social and emotional impact, treatment side effects, organ-specific symptoms, and physical function. The PhGA correlated with the PtGA and prednisone doses. Subanalyses of the AAV-PRO domains according to sex, age, and disease duration showed significant differences in the treatment side effects domain, with higher scores in women, in patients < 50years, and in patients with disease duration < 5years. The domain of concerns about the future showed a higher score in patients with disease duration < 5years. A total of 17/24 (70.8%) of men who completed the IIEF-5 questionnaire were classified as having some degree of erectile dysfunction. The domains of AAV-PRO correlated with other outcome measures, while differences were found between some of the domains according to sex, age, and disease duration.

Renal Limited PR3-ANCA Vasculitis due to Abiotrophia defectiva Infective Endocarditis

Renal Limited PR3-ANCA Vasculitis due to Abiotrophia defectiva Infective Endocarditis

Dialysis Dependency at Discharge in De Novo ANCA Associated Renal Limited Vasculitis: Should We Avoid Further Immunosuppression?

Introduction: ANCA associated vasculitis commonly affects the kidneys. The intense inflammatory damage disrupts the glomerular architecture. Induction immunosuppressive therapy is responsible for a large part of the morbidity and mortality of these patients, due to infectious complications. This highlights the importance of defining risk factors associated with a worse renal prognosis in order to select the patients who would benefit the most from immunosuppressive therapy when it comes to renal limited vasculitis. Methods: Retrospective collection of data from patients admitted to the Coimbra’s University Hospital Nephrology Department with the diagnosis of de novo ANCA vasculitis, between 01-06-2009 and 01-06-2019. Data were analysed with SPSS v26® using parametric and nonparametric tests, Kaplan Meyer survival and ROC curve analysis. Results: There were 81 cases of de novo vasculitis, mainly due to anti-MPO antibody associated vasculitis (N=69; 85.2%). Mean age at diagnosis was 67.4 ± 15.7 years and most patients were male (N=47; 58%). At admission, patients had a mean serum creatinine (sCr) of 6.8 ± 4.0 mg/dL. Dialysis was required in 23 (28.4%) patients at admission and 44.4% were dependent on renal replacement therapy at discharge. Among dialysis-dependent patients at discharge treated with immunosuppression for at least 3 months (N=15) we observed partial kidney recovery in 2 (13.3%). Deaths were mostly (N=8/11; 72.7%) caused by infections. Discussion: Our results call into question the need for aggressive induction treatment in dialysis-dependent patients without extrarenal manifestations.

Association between renal-limited vasculitis and relapse of antineutrophil cytoplasmic antibody-associated vasculitis: A single-center retrospective cohort study in Japan.

BackgroundSeveral previous studies have evaluated the predictors of relapse in antineutrophil cytoplasmic antibody-associated vasculitis. Nonetheless, the association between renal-limited vasculitis and relapse has not been evaluated.ObjectiveTo assess the association between renal-limited vasculitis and the incidence of relapse in Japan among patients with microscopic polyangiitis/renal-limited vasculitis.MethodsThis retrospective cohort study included consecutive patients in remission at 6 months, with renal-limited vasculitis (n = 24, renal-limited vasculitis group) and microscopic polyangiitis with renal and extra-renal involvement (n = 56, non-renal-limited vasculitis group) between 2004 and 2020.ResultsDuring the median follow-up period of 35 (range, 15‒57) months, 28 (35.0%) patients had a relapse. Multivariable Cox proportional hazards models revealed that the lower estimated glomerular filtration rate (per -10 mL/min/1.73 m2; adjusted hazard ratio = 0.87, 95% confidence interval: 0.76–0.99; P = 0.043), renal-limited vasculitis (adjusted hazard ratio = 0.23, 95% confidence interval: 0.08–0.68; P = 0.008), and glucocorticoid combined with intravenous cyclophosphamide or rituximab (adjusted HR = 0.32, 95% CI: 0.11–0.96; P = 0.042) were associated with a decreased risk of relapse. Glucocorticoid dose during the observation period was lower in the renal-limited vasculitis group than in the non-renal-limited vasculitis group.ConclusionsRenal-limited vasculitis was associated with a lower risk of relapse than non-renal-limited vasculitis. Our data may contribute to the development of optimal management for renal-limited vasculitis, which may assist in minimizing the adverse effects of immunosuppressive therapy.

Rituximab treatment in ANCA-associated vasculitis patients: outcomes of a real-life experience from an observational cohort.

Rituximab is a first-line therapy in patients with anti-neutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV). Among previous studies evaluating its efficacy, the Hispanic/Latino population has been underrepresented. This study aimed to assess the outcomes of AAV patients treated with rituximab in a tertiary care center in Mexico. This is a retrospective cohort study including patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or renal-limited vasculitis (RLV), who received at least one dose of rituximab (induction or maintenance therapy) from January 2014 to October 2020. Demographic, clinical, serological, histopathological, and treatment-related variables were retrieved. Outcomes were the rate of remission at 6 months during induction and the rate of relapses during maintenance. Damage, serious infections, and death were assessed. Differences between patients with and without remission were analyzed. Forty-two patients received rituximab, 34 of them as induction to remission. Twenty-two patients (65%) achieved remission after 6 months. Patients who achieved remission were younger than those who did not (50 vs. 60 years, p = 0.03). During induction, severe infections, most frequently pneumonia, occurred in 9 (26%), and one patient died. Twenty-four patients received rituximab as maintenance; of them, 23 (96%) achieved complete response, and 8 (33%) experienced relapses (median follow-up time 19 months). During maintenance, severe infections (pneumonia) occurred in 5 patients (21%), and 3 of them (13%) died. In this observational cohort study, the outcomes were similar to the ones reported in other populations, whereas severe infections were frequent and associated with mortality. Key Points• In this study, the outcomes of 42 Mexican patients with ANCA-associated vasculitis treated with rituximab were assessed in a real-life setting.• At 6 months, 65% of the patients achieved remission with rituximab, especially those younger than 50 years of age.• During maintenance therapy with rituximab, 96% of the patients achieved complete response, and 33% experienced relapses.• Severe infections, mostly pneumonia, occurred in 26% of patients during induction and 21% of patients during maintenance therapy with rituximab.Supplementary InformationThe online version contains supplementary material available at 10.1007/s10067-022-06192-1.

A past medical history of autoimmune disease predicts a future with fewer relapses in patients with ANCA-associated vasculitis

To explore the frequency and impact of an autoimmune disease past-medical history (PMH) in the clinical picture and outcomes of patients with antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV). This was a retrospective study of patients with biopsy-proven AAV, >16 years old, with detailed information about their PMH. Outcomes of interest included remission, treatment resistance, relapse, end-stage kidney disease (ESKD), and death. 206 patients with biopsy-proven AAV and available information regarding their PMH were studied. 63(30.6%) of them had a history of autoimmune disease prior to AAV diagnosis. The mean age overall was 54.1 years. One hundred and five patients (51%) were positive for PR3-ANCA, 101 (49%) for MPO-ANCA. Granulomatosis with polyangiitis was diagnosed in 79 (38.3%), microscopic polyangiitis in 97 (47.1%) and renal-limited vasculitis in 30 (14.6%) individuals. Remission rate was similar among patients with and without a PMH of autoimmune disease. Time-to-event analysis indicated that the relapse-free survival was significantly longer in patients with PMH of autoimmune disease (148.2 vs. 61.9 months, p-value <0.001). After adjusting for covariates, autoimmune disease history was associated with significantly lower risk of relapse (HR: 0.33, 95% CI: 0.15-0.72), which remained significant in males, patients ≥60 years old and those with C/PR3-ANCA, kidney and lung involvement. Patients with a PMH of autoimmune disease, prior to AAV diagnosis, experienced significantly fewer relapses after achievement of remission, compared to patients without such a history, underlining the importance of individualisation of maintenance immunosuppressive therapy, given the different aetiopathogenetic settings the disease was developed.