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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 45 days after systemic symptom onset, and 15 days 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 4 weeks after systemic symptom onset to 2 weeks after immunosuppressive treatment induction. Most aneurysms are less than 10 mm 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. [ABSTRACT FROM AUTHOR]

Introduction: Multiple sclerosis (MS) is a chronic, disabling neurodegenerative disease, leads to reduced quality of life. The increasing prevalence of MS around the world and its comorbidities increase its burden. Primary vasculitis subtypes, one of autoimmune diseases with different prevalence in different ages and genders, should be considered one of the important differential diagnosis in patients with MS. This study aims to verify the relationship between MS and primary vasculitis by conducting a systematic review. Method: We searched PubMed, Scopus, EMBASE, Web of Science, and Google Scholar, from January 1974 to July 2023. We included original articles that reported characteristics of patients involved with any type of Primary Vasculitis with MS. Result: From an initial 816 publications, 18 studies consisting of 18 individual patients from 14 countries with confirmed MS and one of different subtypes of primary vasculitis met the inclusion criteria. The female/male ratio was 0.38:1, the mean (SD) age was 40.44 (14.37) years with the range of 16 to 70 years old, and the relapsing/progressive ratio was 1.57:1. Most of them, 14 (77%) experienced MS before vasculitis, and mostly received Corticosteroids, interferon, cyclophosphamide, Glatiramer acetate as MS treatment. The concurrence of Takayasu Arteritis (2 cases), Polyarteritis Nodosa (2 cases), Churg-Strauss Syndrome (1 case), Wegener's Granulomatosis (2 cases), Microscopic Polyangiitis (1 case), Cutaneous leukocytoclastic vasculitis (5 cases), Good pasture's disease (5 cases) were reported with MS. Conclusion: Our study suggested that different primary vasculitis can be an important comorbidity of MS and can mimic its symptoms and MRI. Any atypical syndrome for PwMS, whether clinical or radiological, must be evaluated in terms of other differential diagnoses including vasculitis. [ABSTRACT FROM AUTHOR]

Objectives: Pneumocystis jirovecii pneumonia (PJP) is a serious complication of autoimmune and inflammatory diseases. This study aimed to describe the characteristics of PJP in patients with various systemic vasculitides and explore potential prognostic factors. Method: Data on 62 enrolled PJP patients with systemic vasculitis were analyzed. Patients were stratified based on the outcomes. Prognostic factors were investigated using Cox-regression models. Characteristics of patients with and without interstitial lung disease (ILD) were compared. Results: Among 62 vasculitis-PJP patients, 48 had anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), with microscopic polyangiitis (MPA) being the most common subtype (28 patients). MPA (HR 4.33, p = 0.001), concomitant aspergillosis (HR 2.68, p = 0.019), and higher D-dimer at PJP diagnosis (HR 1.07, p = 0.004) were independent adverse prognostic factors for overall survival. Stable disease activity of vasculitis was an independent favorable prognostic factor (HR 0.28, p = 0.027). Patients with MPA were older than non-MPA patients (median age: 69 vs. 58 years, p = 0.001); both ILD and fibrotic ILD were more prevalent in MPA patients (ILD: 78.6% vs. 35.3%, p = 0.001; fibrotic ILD: 57.1% vs. 11.8%, p < 0.001). At the diagnosis of PJP, patients with preexisting ILD had higher counts of white cells, lymphocytes, and neutrophils, as well as higher levels of immunoglobulin (Ig) G and IgA, than patients without preexisting ILD. Conclusions: MPA was associated with a higher risk of death in patients with vasculitis-PJP, possibly due to a higher prevalence of ILD. In clinical practice, we should pay more attention to the prophylaxis and management of PJP in patients with systemic vasculitis-associated ILD and/or MPA. Key Points • Data from this study showed that MPA was the most common subtype of vasculitis among vasculitis-PJP patients. • Compared with non-MPA patients in this study, patients with MPA were older, had more ILD and fibrotic ILD, and had a poorer prognosis. • In clinical practice, we should pay more attention to the prophylaxis and management of PJP in patients with systemic vasculitis-associated ILD and/or MPA. [ABSTRACT FROM AUTHOR]

Microscopic polyangiitis (MPA) is a form of necrotizing vasculitis affecting the small vessels. Our study is the first study with the objective of describing the difference in clinical presentation of MPA and response to treatment at 6 months based on the age of disease onset. All patients seen at a tertiary vasculitis clinic between 2015 and 2023 with a diagnosis of MPA were included. Patients were divided into an elderly group (age > = 65 years) and a younger group (age < 65). Comparative analysis was conducted to characterize differences amongst the elderly and younger patients, including differences in organ involvement and clinical presentation, Birmingham Vasculitis Activity Score (BVAS) scores, Vasculitis Damage Index(VDI) scores, and response to treatment at 6 months. Thirty-one patients were included in our study. Younger MPA patients (n = 18) with mean age at diagnosis of 53.17 years were compared with older MPA patients(n = 13) with mean age at diagnosis of 76.08 years. The younger patients had statistically significant higher BVAS scores (p = 0.009), along with higher incidence of renal (p = 0.028), pulmonary (p = 0.0069), and cutaneous (p = 0.026) manifestations at disease onset. Furthermore, amongst the younger population, there was noted statistically significant clinical improvement at 6 months following treatment induction, particularly in the domains of general symptoms (p = 0.011), MSK (p = 0.019), cutaneous (p = 0.011), and pulmonary symptoms (p = 0.04). In contrast, the elderly population presented with a predominant of non-specific constitutional symptoms, with statistically significant improvement in the domain of non-specific general symptoms at 6 months (p = 0.00008). All MPA patients responded well to treatment, with statistically significant improvement amongst younger patients (p = 0.0032), but no statistically significant improvement amongst elderly patients (p = 0.67). Our study findings concluded that MPA's clinical presentation differed by age group. Younger patients had more aggressive vasculitis disease presentation with better response to treatments, whereas, elderly patients had less severe disease presentation with predominant of general symptoms at disease onset. Key Points • MPA clinical presentation differed by age at disease onset. Younger patients presented with more aggressive and classic vasculitis-like presentations, with multi-system organ involvement and statistically significant higher incidence of renal, pulmonary, and cutaneous manifestations. In contrast, elderly patients had a predominant of constitutional and non-specific symptoms with often delayed diagnosis. • All MPA patients responded well to treatment. Amongst the younger population, there was statistically significant improvement in BVAS scores after treatment induction at 6 months; however, there was no statistically significant improvement of BVAS scores in the elderly population. • Future studies are required to further understand the difference in the clinical presentation of MPA based on the age at disease onset, and the implication on disease diagnosis and management. [ABSTRACT FROM AUTHOR]

ANCA-associated vasculitides (AAV) comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis. All forms may involve different organ systems, yet kidney and lung involvement are common and fatal in many cases. Here, we aimed to determine the predictive value of pulmonary disease manifestation and individual CT findings in AAV patients. Available CT scans and clinical information on mortality, renal outcomes, occurrence of relapses and damage scores were analysed retrospectively from a tertiary rheumatology center in Germany. We included a total of 94 AAV patients (49 with GPA, 41 with MPA). Forty-four patients had lung involvement with available CT scans, 70.5% of which with GPA and 72.7% with renal involvement. Nodule formation and cavities were more frequent among GPA patients, whereas ground-glass opacities (GGO), ILD and pleural effusion were observed predominantly in MPA patients. Over a median follow-up of 37 months, GPA patients had a slightly higher overall mortality, whereas end-stage kidney failure rates were significantly increased in MPA patients. Relapse frequencies were comparable between both entities. The presence of GGO and pleural effusion were associated with higher relapse rates, whereas nodules were negatively correlated with relapses. Notably, RTX-treated patients had less infections as compared to individuals under different therapies. Our data demonstrate the outstanding importance of characteristic CT patterns in AAV diagnosis assessment. Especially certain CT patterns including GGO and pleura effusion may help to identify patients who are at higher risk for relapsing disease. [ABSTRACT FROM AUTHOR]

We aimed to clarify the long-term safety and efficacy of rituximab (RTX) as a remission induction therapy following severe relapse in patients with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV). We retrospectively collected the data of patients with severely relapsed AAV from a Japanese multicentre cohort. The primary exposure was RTX use; the primary outcome was complete remission (CR) proportions at week 24. Baseline characteristics were compared between the RTX and non-RTX groups. We performed multivariate logistic regression analysis and one-to-one propensity score matching analysis as a sensitivity analysis. Totally, 100 patients were enrolled: 52 in the RTX group and 48 in the non-RTX group. Baseline characteristics were comparable between the two groups, except for age, AAV subtype and ANCA serotype. The median age was 71 vs. 75 years, and the PR3-ANCA positivity rate was 44.2% vs. 18.8% in the RTX and non-RTX groups, respectively. No significant difference was observed in CR proportions at week 24 between the two groups (79.2% vs. 68.1%, p = 0.321), with an adjusted odds ratio of 1.27 (95% confidence interval [CI] 0.47–3.51). At week 48, CR proportions were significantly higher in the RTX group (91.7% vs. 64.9%, p = 0.005), with an adjusted odds ratio of 2.95 (95% CI 0.97–9.91). Serious infection rates were lower in the RTX group than in the non-RTX group, with no statistically significant difference. RTX was not superior to conventional immunosuppressive therapies at week 24 but showed significantly favourable results at week 48 for severely relapsed AAV. Key Points • RTX might be superior to traditional treatments as an induction therapy for severely relapsed AAV. • RTX has the potential to sustain long-term remission with fewer occurrences of infections in the treatment of severely relapsed AAV. [ABSTRACT FROM AUTHOR]

This article discusses a study that examined the frequency of positive antineutrophil cytoplasmic antibody (ANCA) tests during the COVID-19 pandemic compared to before the pandemic. The study found that both PR3-ANCA and MPO-ANCA tests were more commonly ordered during the pandemic. Positive results for PR3-ANCA were more frequently detected during the pandemic, while positive results for MPO-ANCA were more commonly detected before the pandemic. The study suggests a potential link between COVID-19 and the development of autoimmune diseases, but further research is needed to understand the underlying mechanisms. The study also emphasizes the importance of educating physicians about ANCA and autoimmune vasculitis. [Extracted from the article]

Introduction: This study aimed to explore ocular manifestations in ANCA-associated vasculitis (AAV), focusing on granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA) and to examine the associations with laboratory parameters and other systemic manifestations. Methods: This retrospective study reviewed data from 533 AAV patients across two major Chinese medical centers from January 2016 to November 2023. Data including diagnosis, cranial manifestations of disease, ocular complications, and laboratory parameters were analyzed. Univariate and multivariable logistic regression analyses assessed associations across disease manifestations. Machine learning models were also utilized to predict the risk of retinal/eye involvement in AAV patients. Results: Among 533 patients (210 GPA, 217 MPA, 99 EGPA, and 7 unclassified AAV), ocular complications were observed in 20.64% of them, with a distribution of 36.67% in GPA, 7.37% in MPA, and 18.18% in EGPA. The most common ocular manifestations included scleritis and retro-orbital mass/dacryocystitis, which were notably prevalent in GPA patients. Retinal involvement was observed in 9.09% of EGPA cases. The machine learning models yielded that eosinophil percentage (EOS%), high-sensitivity C-reactive protein (hsCRP), and CD4 + T cell/CD8 + T cell ratio (T4/T8) can predict retinal involvement. Furthermore, the white blood cell, EOS%, APTT, IgA, hsCRP, PR3-ANCA, and T4/T8 can predict eye involvement. Conclusion: Ocular manifestations are a prevalent complication across all forms of AAV. Predictive models developed through machine learning offer promising tools for early intervention and tailored patient care. This necessitates a multidisciplinary approach, integrating rheumatology and ophthalmology expertise for optimal patient outcomes. Key Points • This study provides new insights into the ocular manifestations of ANCA-associated vasculitis across different subtypes, revealing a notable prevalence of ocular complications. • A machine learning model is built to predict eye/retinal involvement, enhancing early diagnostic capabilities. • The findings could guide more systematic ocular screenings in ANCA-associated vasculitis patients and influence treatment protocols. [ABSTRACT FROM AUTHOR]

Purpose of Review: This review aims to summarize the evolution and recent developments in the classification of ANCA associated vasculitis (AAV) and to summarize evaluations of the 2022 ACR/EULAR classification criteria of AAV in several cohorts. Recent Findings: The classification of AAV has been a field of controversy for some time. The parallel existence of classification criteria and disease definitions produced some overlap in classification, leading to challenges when comparing different cohorts. The 2022 ACR/EULAR classification criteria derived from the largest study ever conducted in vasculitis account for significant changes in vasculitis classification with the integration of ANCA and modern imaging. These criteria show good performance compared to previous ones but also raise questions as ANCA serotypes have substantial impact on classification. In addition, there are some discrepancies with earlier agreed histopathological features of AAV disease phenotypes. Summary: During the last 35 years, several sets of classification criteria have evolved to facilitate epidemiologic studies and clinical trials in AAV. While some of these criteria have been in use for many years, they were criticized due to either not using ANCA or not integrating surrogate markers for vasculitis but also due to overlapping when used in parallel. The long-awaited new ACR/EULAR criteria for AAV were published in 2022 and are the result of a large international study, introducing for the first time ANCA and modern imaging in the classification of AAV. Though the criteria show good performance, they bring several other challenges with practical application. [ABSTRACT FROM AUTHOR]

Background: Antineutrophil cytoplasmic antibody-associated vasculitides (AAV) is a group of systemic necrotizing small vessel autoimmune diseases, with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) being the two most common. The co-existence of AAV with different immune-mediated diseases (autoimmune disesases - AID) might affect the clinical presentation of the primary disease. The purpose of the study was to assess the co-existence of AAV with AID and to investigate whether it affects the characteristics and the course of AAV. Methods: A retrospective single-center study was performed to identify patients with a diagnosis of MPA or GPA and concomitant AID, and to investigate their clinical features and characteristics. The group consisted of consecutive unselected AAV patients treated at a large university-based hospital, since 1988 with follow-up until 2022. Results: Among 284 patients diagnosed either with GPA (232) or MPA (52), 40 (14,1%) had co-existing AIDs. The most frequent were: Hashimoto thyroiditis (16 cases), rheumatoid arthritis (8 cases), followed by psoriasis (6 cases), pernicious anemia (3 cases), and alopecia (3 cases). Patients with autoimmune comorbidities had a significantly longer time between the onset of symptoms and the diagnosis (26 vs. 11 months, p < 0.001). Laryngeal involvement (20.0% vs. 9.0%, p = 0,05), peripheral nervous system disorders (35.0% vs. 13.9%, p < 0.001), and neoplasms (20.0% vs. 8.6%, p = 0,044) were more common in patients with AID comorbidities, compared to subjects without AID. In contrast, renal involvement (45.0% vs. 70.9%, p = 0.001) and nodular lung lesions (27.5% vs. 47.5%, p = 0.044) were significantly less frequent in patients with co-morbidities. Following EUVAS criteria, patients with autoimmune co-morbidities had a generalized form of the disease without organ involvement (52.5% vs. 27.2%, p = 0.007), while the others had a higher percentage of generalized form with organ involvement (38.3% vs. 20.0%, p = 0.007). Conclusions: The coexistence of AAV with different autoimmune diseases is not common, but it might affect the clinical course of the disease. Polyautoimmunity prolonged the time to diagnosis, but the AAV course seemed to be milder. Particular attention should be paid to the increased risk of cancer in these patients. It also seems reasonable that AAV patients should receive a serological screening to exclude the development of overlapping diseases. [ABSTRACT FROM AUTHOR]

Background: Kidney and life outcomes remain unsatisfactory in patients with microscopic polyangiitis (MPA). Appropriate treatment intensity must be provided to the appropriate patients. To identify severe cases early, we investigated the factors related to kidney and life outcomes. Methods: We included patients diagnosed with MPA based on myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity and kidney histopathology results after kidney biopsies between January 1, 2021, and May 11, 2023, at 10 affiliated centers, including our hospital. Death, maintenance dialysis, and estimated glomerular filtration rate (eGFR) < 15 after 6 months of treatment were defined as poor prognosis groups, and factors associated with these conditions were investigated. Results: We included 84 (36 men and 48 women) patients in this study. Median age was 73.8 (interquartile range: 71–81) years. After 6 months of treatment, the proportion of patients in the poor prognosis group was 16.7 %, with a mortality of 7.1 % and a poor kidney prognosis rate of 9.5 %. Area under the receiver operating characteristic curve showed that eGFR at 2 weeks had a comparable prognostic performance equal as eGFR at 4 weeks (area under the curve: 0.875 and 0.896, respectively). After adjustment by various factors, eGFR at 2 weeks was related with prognosis significantly (p = 0.031). Conclusion: Kidney function 2 weeks after the start of treatment for MPA can predict prognosis. [ABSTRACT FROM AUTHOR]

Objectives: The cerebral vessels may be affected in primary systemic vasculitis (PSV), but little is known about cerebrovascular events (CVEs) in this population. This study aimed to determine the frequency of CVEs at the time of diagnosis of PSV, to identify factors associated with CVEs in PSV, and to explore features and outcomes of stroke in patients with PSV. Methods: Data from adults newly diagnosed with PSV within the Diagnostic and Classification Criteria in VASculitis (DCVAS) study were analysed. Demographics, risk factors for vascular disease, and clinical features were compared between patients with PSV with and without CVE. Stroke subtypes and cumulative incidence of recurrent CVE during a prospective 6-month follow-up were also assessed. Results: The analysis included 4828 PSV patients, and a CVE was reported in 169 (3.50%, 95% CI 3.00–4.06): 102 (2.13% 95% CI 1.73–2.56) with stroke and 81 (1.68% 95% CI 1.33–2.08) with transient ischemic attack (TIA). The frequency of CVE was highest in Behçet's disease (9.5%, 95% CI 5.79–14.37), polyarteritis nodosa (6.2%, 95% CI 3.25–10.61), and Takayasu's arteritis (6.0%, 95% CI 4.30–8.19), and lowest in microscopic polyangiitis (2.2%, 95% CI 1.09–3.86), granulomatosis with polyangiitis (2.0%, 95% CI 1.20–3.01), cryoglobulinaemic vasculitis (1.9%, 95% CI 0.05–9.89), and IgA-vasculitis (Henoch-Schönlein) (0.4%, 95% CI 0.01–2.05). PSV patients had a 11.9% cumulative incidence of recurrent CVE during a 6-month follow-up period. Conclusion: CVEs affect a significant proportion of patients at time of PSV diagnosis, and the frequency varies widely among different vasculitis, being higher in Behçet's. Overall, CVE in PSV is not explained by traditional vascular risk factors and has a high risk of CVE recurrence. [ABSTRACT FROM AUTHOR]

The aim of this study is to present the current care situation of patients with giant cell arteritis (GCA), Takayasu arteritis (TAK), ANCA-associated vasculitis (AAV) and Behçet's disease (BD). Trends over the last 15 years will reflect improvements and remaining deficits in the management of vasculitides. Consecutive cross-sectional data from patients with vasculitides from the German National Database (NDB) of the Collaborative Arthritis Centres between 2007 and 2021 were included. Medication, physician- and patient-reported outcomes on disease activity and disease burden, inpatient stays and occupational participation are compared for different vasculitis entities and over time. Employment rates were compared to German population rates. Between 502 and 854 vasculitis patients were annually documented. GCA and AAV were the most common vasculitides. Median disease duration ranged from 2 to 16 years. Over the years, glucocorticoids decreased in proportion and dose, most markedly in GCA and TAK, while biologic therapies increased up to 27%. Physicians rated disease activity as low for the vast majority of patients, while patients-reported moderate outcomes in many dimensions. PROs remained largely unchanged. The proportion of employed patients (< 65 years) increased from 47 to 57%. In recent years, biologics are increasingly used in patients with vasculitides, while glucocorticoids decreased significantly. PRO's have not improved. Work participation increased but remains lower than that in the German population. [ABSTRACT FROM AUTHOR]

IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that is characterized by storiform fibrosis, infiltration of IgG4-positive lymphocytes, obliterative phlebitis, and high IgG4 levels. Since IgG4-RD affects a wide variety of organs, a differential diagnosis must include multiple conditions. IgG4-RD is also believed to coexist with certain diseases. In recent years, case reports and case series describing the co-occurrence of IgG4-RD and ANCA-associated vasculitis (AAV) have been published. We intended to evaluate patients with IgG4-RD and AAV overlap in the literature using a case similar to one that was diagnosed and monitored in our department. We searched the databases of Web of Science, Scopus, and Google Scholar as well as PubMed with the keywords ANCA, IgG4, IgG4-RD, granulomatosis with polyangiitis, Wegener's granulomatosis, microscopic polyangiitis, Eosinophilic granulomatosis with polyangiitis, and Churg–Strauss syndrome. Cases and Case series addressing the coexistence of IgG4-RD and AAV have been selected. Comprehensive diagnostic criteria are used to diagnose IgG4-RD. The Chapel Hill Consensus Conference nomenclature criteria were used for the inclusion of AAV. Out of a total of 910 publications, 20 articles, including 65 cases, were found to be eligible. Forty-seven cases with IgG4-RD were evaluated as definitive (71.2%), 10 cases as probable (15.1%), and 9 cases as possible IgG4-RD (13.6%). 26 patients were diagnosed with GPA, 1 patient with localized GPA, 23 patients with MPA, and 4 patients with EGPA. The aorta, lacrimal tissue, pancreas, and retroperitoneum are the sites of IgG4-RD rather than AAV. AAV and IgG4-RD might coexist in the same patient. IgG4-RD is mainly associated with GPA. [ABSTRACT FROM AUTHOR]

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ANCA-associated vasculitis (AAV) comprises a group of necrotizing vasculitis that mainly affects small- and medium-sized vessels. Serum anti-neutrophil cytoplasmic antibodies (ANCA), mainly anti-myeloperoxidase (anti-MPO) and anti-proteinase 3 (anti-PR3), levels may correlate to severity, prognosis, and recurrence of the disease. A retrospective analysis of 101 patients with MPO-positive and 54 PR3-positive vasculitis was performed, using laboratory established cut-off value, measured by chemiluminescence. Furthermore, data of renal disease and pulmonary involvement were collected at vasculitis diagnosis, as well as the progress, requiring dialysis, transplant, or mortality. For anti-MPO antibodies with a diagnosis of vasculitis (n = 77), an area under the curve (AUC) was calculated (AUC = 0.8084), and a cut-off point of 41.5 IU/ml was determined. There were significant differences in anti-MPO levels between patients with renal or pulmonary dysfunction (n = 65) versus those without them (n = 36) (p = 0.0003), and a cut-off threshold of 60 IU/ml was established. For anti-PR3 antibodies with a diagnosis of vasculitis (n = 44), an area under the curve (AUC) was calculated (AUC = 0.7318), and a cut-off point of 20.5 IU/ml was determined. Significant differences in anti-PR3 levels were observed between those patients with renal or pulmonary dysfunction (n = 30) and those without them (n = 24) (p = 0.0048), and a cut-off threshold of 41.5 IU/ml was established. No significant differences between those patients who had a worse disease progression and those who did not were found for anti-MPO and anti-PR3. Anti-MPO and anti-PR3 levels at the moment of vasculitis diagnosis are related with disease severity but not with disease outcome or vasculitis recurrence. [ABSTRACT FROM AUTHOR]

Objective: 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. Methods: 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. Results: 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). Conclusion: 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. [ABSTRACT FROM AUTHOR]

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a group of diseases characterised by necrotizing inflammation of small vessels such as arterioles, venules, and capillaries. ANCA-associated vasculitides (AAV) are referred to as small vessel vasculitides. Three AAV subgroups, namely granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA), are defined according to clinical features. The most common disease with renal involvement in AAV is MPA Approximately 90% of patients with MPA have renal involvement. While this rate is 70–80% in GPA, less than half of EGPA patients have renal involvement. Untreated survival in AAVs is less than one year. With appropriate immunosuppressive therapy, the 5-year renal survival rate is 70–75%. Without therapy, the prognosis is poor but treatments, typically immunosuppressants, have improved survival, albeit with considerable morbidity from glucocorticoids and other immunosuppressive medications. Current challenges include improving the measures of disease activity and risk of relapse, uncertainty about optimal therapy duration and a need for targeted therapies with fewer adverse effects. In this review, we described the treatment of renal involvement in AAV in line with current studies. [ABSTRACT FROM AUTHOR]

The immunological basis of the clinical heterogeneity in autoimmune vasculitis remains poorly understood. In this study, we conduct single-cell transcriptome analyses on peripheral blood mononuclear cells (PBMCs) from newly-onset patients with microscopic polyangiitis (MPA). Increased proportions of activated CD14+ monocytes and CD14+ monocytes expressing interferon signature genes (ISGs) are distinctive features of MPA. Patient-specific analysis further classifies MPA into two groups. The MPA-MONO group is characterized by a high proportion of activated CD14+ monocytes, which persist before and after immunosuppressive therapy. These patients are clinically defined by increased monocyte ratio in the total PBMC count and have a high relapse rate. The MPA-IFN group is characterized by a high proportion of ISG+ CD14+ monocytes. These patients are clinically defined by high serum interferon-alpha concentrations and show good response to immunosuppressive therapy. Our findings identify the immunological phenotypes of MPA and provide clinical insights for personalized treatment and accurate prognostic prediction. Autoimmune vasculitis can be heterogeneous in terms of immune cell involvement. Here the authors use a single cell transcriptomics approach to characterise a group of microscopic polyangiitis patients that could be split into two groups typified by monocyte or Interferon associated gene expression. [ABSTRACT FROM AUTHOR]

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of systemic vasculitis characterized by autoantibodies against neutrophil cytoplasmic antigens (proteinase 3 PR3-ANCA and myeloperoxidase MPO-ANCA) and inflammation of small vessels. AAV include the diagnosis Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), which share many clinical and pathological features. Immunomodulatory therapies have significantly improved prognosis during the last decade. Nevertheless, especially in undiagnosed and thus uncontrolled AAV mortality due to renal impairment or pulmonary haemorrhages is still high. AAV are rare in fertile women, as the typical age of manifestation is above 50 years but there are women with AAV who are or want to become pregnant. This review focusses on how to manage patients with AAV planning to become pregnant and during their pregnancy. [ABSTRACT FROM AUTHOR]

Purpose: Intermittent claudication (IC) refers to leg pain that is induced by walking and relieved by rest. Neurogenic IC is usually associated with lumbar canal stenosis (LCS). We present rare findings from an autopsied patient who had neurogenic IC caused by vasculitis in the cauda equina. Methods: We performed antemortem neurological and electrophysiological assessments, sural nerve biopsy, and post-mortem examination of the spinal cord and brain. Results: A 61-year-old man noted sudden-onset leg pain that was not associated with any traumatic trigger. His leg pain consistently appeared when the patient walked and quickly faded on stopping. Spine surgery and cardiovascular departments both made a diagnosis of IC. However, magnetic resonance imaging (MRI) did not show LCS, and all ankle-brachial pressure indices were normal. He subsequently developed diffuse muscle weakness of the legs a month after disease onset. Myeloperoxidase antineutrophil cytoplasmic autoantibody was seropositive (140 IU/mL), and a sural nerve biopsy revealed axonal injury and angiitis. MRI showed multiple cerebral infarctions. He was diagnosed with microscopic polyangiitis (MPA) and underwent corticosteroid therapy. He died from complications two months after the onset. A post-mortem study revealed vasculitis in the subarachnoid space of the cauda equina, spinal cord, and brain parenchyma. The cauda equina showed a combined loss of small and large axonal fibres. The lumbar cord displayed central chromatolysis of the lower motor neurons. Conclusion: MPA is a rare cause of neurogenic IC when the symptom is acute and multimodal. Small-vessel vasculitis affecting the cauda equina may underlie MPA-associated IC. [ABSTRACT FROM AUTHOR]

Because of some similarities in organ involvement, clinical manifestations, and histopathological features, IgG4-related disease (IgG4-RD) may occur concurrently with some clinicopathologic variants of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). An overlap syndrome of IgG4-RD and AAV has recently been proposed in clinical and/or histopathological studies, indicating that there may be some potential pathophysiological associations between the two disease entities; however, the mechanisms underlying these are incompletely understood. Here, we describe a rare case of a 63-year-old man with IgG4-related tubulointerstitial nephritis (IgG4-TIN) and microscopic polyangiitis-associated glomerulonephritis (MPA-GN) overlap syndrome. The clinical diagnosis of MPA was based on the 2022 American College of Rheumatology (ACR)/European League Against Rheumatology (EULAR) classification criteria. Remission induction therapy with intravenous methylprednisolone was initiated, followed by oral prednisone maintenance therapy with gradual tapering. The patient remained asymptomatic and his renal function was essentially normalized within 3.5 months of follow-up. The serum IgG4 levels decreased to 5 g/L. We also conducted a literature review to identify clinical findings, treatment options, and outcomes of patients with concurrent IgG4-RD and MPA and briefly discussed the potential pathophysiological association between IgG4-RD and MPA. Our findings enrich the database of this rare overlap syndrome and provide a basis for the diagnosis and early intervention in both diseases. These results provide some insights for clinicians to recognize and treat this overlap syndrome. Key Points • We report a unique case that enriches the database of this rare overlap syndrome and provides a basis for the outcome and early intervention in both diseases. •Several studies have suggested that IgG4-RD and MPA have a potential association, and we describe the pathophysiological mechanism of the overlap syndrome for the first time. • There have been no standardized diagnostic criteria for MPA for over 30 years; the 2022 ACR/EULAR classification criteria are expected to solve this problem. • We provide some clues for the diagnosis and treatment of this overlap syndrome based on the literature review. [ABSTRACT FROM AUTHOR]

Microscopic polyangiitis (MPA) is an autoimmune disease, characterized by ANCA in blood and necrotizing inflammation of small and medium-sized vessels, one of the three clinical phenotypes of ANCA-associated vasculitis (AAV). Autophagy has been confirmed to be involved in the pathogenesis of AAV. AKT1 is one of the autophagy-regulated proteins. Its single nucleotide polymorphisms (SNPs) are associated with multiple immune-related diseases, but there are rarely studies in AAV. The incidence rate of AAV has a notable geographic difference, and MPA is predominant in China. The aim of this study was to investigate the association between AKT1 SNP and MPA risk. Genotypes of 8 loci in AKT1 were evaluated by multiplex polymerase chain reaction (PCR) and high-throughput sequencing in 416 people, including 208 MPA patients and 208 healthy volunteers from Guangxi in China. Additionally, data of 387 healthy volunteers from China were obtained from the 1000Genomes Project on public database. Differences were observed between the loci (rs2498786, rs2494752, and rs5811155) genotypes in AKT1 and MPA risk (P = 7.0 × 10–4, P = 3.0 × 10–4, and P = 5.9 × 10–5, respectively). A negative association was detected in the Dominant model (P = 1.2 × 10–3, P = 2.0 × 10–4 and P = 3.6 × 10–5, respectively). A haplotype (G-G-T) was associated with MPA risk negatively (P = 7.0 × 10–4). This study suggests that alleles (rs2498786 G, rs2494752 G and rs5811155 insT) are protective factors for MPA and alleles (rs2494752 G and rs5811155 insT) for MPO-ANCA in patients with MPA. There is a haplotype (G-G-T), which is a protective factor for MPA. It suggests that the role of AKT1 in MPA/AAV needs further study to provide more intervention targets for MPA/AAV. [ABSTRACT FROM AUTHOR]

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 53.5 years (43–61), and a disease duration of 82 months (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 < 50 years, and in patients with disease duration < 5 years. The domain of concerns about the future showed a higher score in patients with disease duration < 5 years. 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. [ABSTRACT FROM AUTHOR]

Objective: Antineutrophil cytoplasm antibody-associated vasculitis (AAV) with interstitial lung disease (AAV-ILD) is the main manifestation of AAV involving the lung, further increasing the risk of poor prognosis in patients with AAV. This study aimed to investigate the risk factors associated with mortality in patients with AAV-ILD.In Web of Science, PubMed, Embase and Scopus databases, a comprehensive search was performed for English studies on AAV and ILD published from inception date until May 17, 2024. Hazard ratios (HR) and 95% confidence intervals (CI) of mortality-related risk factors in AAV-ILD were collected, and subgroup analyses were carried out based on different candidate risk factors. Cochran's Q statistic and inconsistency value were utilized to assess the heterogeneity of included studies. Sensitivity analysis was executed using one-by-one elimination method, and publication bias was evaluated with Egger's test and the trim-and-fill method.A total of 654 patients with AAV-ILD in eight studies were included for the pooled analysis of mortality risk factors. The results showed that age (HR = 1.06, 95%CI: 1.04, 1.08), ever smoker (HR = 1.61, 95%CI: 1.13, 2.29), usual interstitial pneumonia pattern (HR = 2.07, 95%CI: 1.43, 3.00), acute exacerbation (HR = 2.73, 95%CI: 1.70, 4.40) and microscopic polyangiitis (HR = 4.03, 95%CI: 1.70, 9.55) were associated with an increased risk of AAV-ILD mortality. Conversely, percent predicted forced vital capacity (HR = 0.97, 95%CI: 0.96, 0.99) and immunosuppressant for induction (HR = 0.40, 95%CI: 0.28, 0.58) were associated with a reduced risk of AAV-ILD mortality. Male (HR = 1.27, 95%CI: 0.90, 1.80), nervous system involvement (HR = 0.99, 95%CI: 0.65, 1.52), renal involvement (HR = 1.24, 95%CI: 0.97, 1.95) and five factor score ≥ 1 (HR = 1.00, 95%CI: 0.67, 1.48) showed no significant correlation with mortality risk in patients with AAV-ILD. Heterogeneity test indicated no significant heterogeneity among the pooled studies. The results of sensitivity analysis, Egger's test and the trim-and-fill method revealed that the pooled findings were stable and reliable.The pooled analyses demonstrated that age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for mortality in patients with AAV-ILD, while percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against mortality. A systematic understanding of the risk factors for AAV-ILD may provide clues for developing effective interventions and managements to improve poor prognosis in patients.Key Points• Increase of age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for poor prognosis in patients with AAV-ILD.• High level of percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against poor prognosis.• Male, nervous system involvement, renal involvement and five factor score ≥ 1 showed no significant correlation with poor prognosis in patients with AAV-ILD.Key Points• Increase of age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for poor prognosis in patients with AAV-ILD.• High level of percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against poor prognosis.• Male, nervous system involvement, renal involvement and five factor score ≥ 1 showed no significant correlation with poor prognosis in patients with AAV-ILD.Methods: Antineutrophil cytoplasm antibody-associated vasculitis (AAV) with interstitial lung disease (AAV-ILD) is the main manifestation of AAV involving the lung, further increasing the risk of poor prognosis in patients with AAV. This study aimed to investigate the risk factors associated with mortality in patients with AAV-ILD.In Web of Science, PubMed, Embase and Scopus databases, a comprehensive search was performed for English studies on AAV and ILD published from inception date until May 17, 2024. Hazard ratios (HR) and 95% confidence intervals (CI) of mortality-related risk factors in AAV-ILD were collected, and subgroup analyses were carried out based on different candidate risk factors. Cochran's Q statistic and inconsistency value were utilized to assess the heterogeneity of included studies. Sensitivity analysis was executed using one-by-one elimination method, and publication bias was evaluated with Egger's test and the trim-and-fill method.A total of 654 patients with AAV-ILD in eight studies were included for the pooled analysis of mortality risk factors. The results showed that age (HR = 1.06, 95%CI: 1.04, 1.08), ever smoker (HR = 1.61, 95%CI: 1.13, 2.29), usual interstitial pneumonia pattern (HR = 2.07, 95%CI: 1.43, 3.00), acute exacerbation (HR = 2.73, 95%CI: 1.70, 4.40) and microscopic polyangiitis (HR = 4.03, 95%CI: 1.70, 9.55) were associated with an increased risk of AAV-ILD mortality. Conversely, percent predicted forced vital capacity (HR = 0.97, 95%CI: 0.96, 0.99) and immunosuppressant for induction (HR = 0.40, 95%CI: 0.28, 0.58) were associated with a reduced risk of AAV-ILD mortality. Male (HR = 1.27, 95%CI: 0.90, 1.80), nervous system involvement (HR = 0.99, 95%CI: 0.65, 1.52), renal involvement (HR = 1.24, 95%CI: 0.97, 1.95) and five factor score ≥ 1 (HR = 1.00, 95%CI: 0.67, 1.48) showed no significant correlation with mortality risk in patients with AAV-ILD. Heterogeneity test indicated no significant heterogeneity among the pooled studies. The results of sensitivity analysis, Egger's test and the trim-and-fill method revealed that the pooled findings were stable and reliable.The pooled analyses demonstrated that age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for mortality in patients with AAV-ILD, while percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against mortality. A systematic understanding of the risk factors for AAV-ILD may provide clues for developing effective interventions and managements to improve poor prognosis in patients.Key Points• Increase of age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for poor prognosis in patients with AAV-ILD.• High level of percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against poor prognosis.• Male, nervous system involvement, renal involvement and five factor score ≥ 1 showed no significant correlation with poor prognosis in patients with AAV-ILD.Key Points• Increase of age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for poor prognosis in patients with AAV-ILD.• High level of percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against poor prognosis.• Male, nervous system involvement, renal involvement and five factor score ≥ 1 showed no significant correlation with poor prognosis in patients with AAV-ILD.Results: Antineutrophil cytoplasm antibody-associated vasculitis (AAV) with interstitial lung disease (AAV-ILD) is the main manifestation of AAV involving the lung, further increasing the risk of poor prognosis in patients with AAV. This study aimed to investigate the risk factors associated with mortality in patients with AAV-ILD.In Web of Science, PubMed, Embase and Scopus databases, a comprehensive search was performed for English studies on AAV and ILD published from inception date until May 17, 2024. Hazard ratios (HR) and 95% confidence intervals (CI) of mortality-related risk factors in AAV-ILD were collected, and subgroup analyses were carried out based on different candidate risk factors. Cochran's Q statistic and inconsistency value were utilized to assess the heterogeneity of included studies. Sensitivity analysis was executed using one-by-one elimination method, and publication bias was evaluated with Egger's test and the trim-and-fill method.A total of 654 patients with AAV-ILD in eight studies were included for the pooled analysis of mortality risk factors. The results showed that age (HR = 1.06, 95%CI: 1.04, 1.08), ever smoker (HR = 1.61, 95%CI: 1.13, 2.29), usual interstitial pneumonia pattern (HR = 2.07, 95%CI: 1.43, 3.00), acute exacerbation (HR = 2.73, 95%CI: 1.70, 4.40) and microscopic polyangiitis (HR = 4.03, 95%CI: 1.70, 9.55) were associated with an increased risk of AAV-ILD mortality. Conversely, percent predicted forced vital capacity (HR = 0.97, 95%CI: 0.96, 0.99) and immunosuppressant for induction (HR = 0.40, 95%CI: 0.28, 0.58) were associated with a reduced risk of AAV-ILD mortality. Male (HR = 1.27, 95%CI: 0.90, 1.80), nervous system involvement (HR = 0.99, 95%CI: 0.65, 1.52), renal involvement (HR = 1.24, 95%CI: 0.97, 1.95) and five factor score ≥ 1 (HR = 1.00, 95%CI: 0.67, 1.48) showed no significant correlation with mortality risk in patients with AAV-ILD. Heterogeneity test indicated no significant heterogeneity among the pooled studies. The results of sensitivity analysis, Egger's test and the trim-and-fill method revealed that the pooled findings were stable and reliable.The pooled analyses demonstrated that age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for mortality in patients with AAV-ILD, while percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against mortality. A systematic understanding of the risk factors for AAV-ILD may provide clues for developing effective interventions and managements to improve poor prognosis in patients.Key Points• Increase of age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for poor prognosis in patients with AAV-ILD.• High level of percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against poor prognosis.• Male, nervous system involvement, renal involvement and five factor score ≥ 1 showed no significant correlation with poor prognosis in patients with AAV-ILD.Key Points• Increase of age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for poor prognosis in patients with AAV-ILD.• High level of percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against poor prognosis.• Male, nervous system involvement, renal involvement and five factor score ≥ 1 showed no significant correlation with poor prognosis in patients with AAV-ILD.Conclusion: Antineutrophil cytoplasm antibody-associated vasculitis (AAV) with interstitial lung disease (AAV-ILD) is the main manifestation of AAV involving the lung, further increasing the risk of poor prognosis in patients with AAV. This study aimed to investigate the risk factors associated with mortality in patients with AAV-ILD.In Web of Science, PubMed, Embase and Scopus databases, a comprehensive search was performed for English studies on AAV and ILD published from inception date until May 17, 2024. Hazard ratios (HR) and 95% confidence intervals (CI) of mortality-related risk factors in AAV-ILD were collected, and subgroup analyses were carried out based on different candidate risk factors. Cochran's Q statistic and inconsistency value were utilized to assess the heterogeneity of included studies. Sensitivity analysis was executed using one-by-one elimination method, and publication bias was evaluated with Egger's test and the trim-and-fill method.A total of 654 patients with AAV-ILD in eight studies were included for the pooled analysis of mortality risk factors. The results showed that age (HR = 1.06, 95%CI: 1.04, 1.08), ever smoker (HR = 1.61, 95%CI: 1.13, 2.29), usual interstitial pneumonia pattern (HR = 2.07, 95%CI: 1.43, 3.00), acute exacerbation (HR = 2.73, 95%CI: 1.70, 4.40) and microscopic polyangiitis (HR = 4.03, 95%CI: 1.70, 9.55) were associated with an increased risk of AAV-ILD mortality. Conversely, percent predicted forced vital capacity (HR = 0.97, 95%CI: 0.96, 0.99) and immunosuppressant for induction (HR = 0.40, 95%CI: 0.28, 0.58) were associated with a reduced risk of AAV-ILD mortality. Male (HR = 1.27, 95%CI: 0.90, 1.80), nervous system involvement (HR = 0.99, 95%CI: 0.65, 1.52), renal involvement (HR = 1.24, 95%CI: 0.97, 1.95) and five factor score ≥ 1 (HR = 1.00, 95%CI: 0.67, 1.48) showed no significant correlation with mortality risk in patients with AAV-ILD. Heterogeneity test indicated no significant heterogeneity among the pooled studies. The results of sensitivity analysis, Egger's test and the trim-and-fill method revealed that the pooled findings were stable and reliable.The pooled analyses demonstrated that age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for mortality in patients with AAV-ILD, while percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against mortality. A systematic understanding of the risk factors for AAV-ILD may provide clues for developing effective interventions and managements to improve poor prognosis in patients.Key Points• Increase of age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for poor prognosis in patients with AAV-ILD.• High level of percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against poor prognosis.• Male, nervous system involvement, renal involvement and five factor score ≥ 1 showed no significant correlation with poor prognosis in patients with AAV-ILD.Key Points• Increase of age, ever smoker, usual interstitial pneumonia pattern, acute exacerbation and microscopic polyangiitis were risk factors for poor prognosis in patients with AAV-ILD.• High level of percent predicted forced vital capacity and immunosuppressant therapy for induction serve as protective factors against poor prognosis.• Male, nervous system involvement, renal involvement and five factor score ≥ 1 showed no significant correlation with poor prognosis in patients with AAV-ILD. [ABSTRACT FROM AUTHOR]

Introduction: Spontaneous renal hemorrhage (SRH) in ANCA-associated vasculitis (AAV) is rare but fatal. We aimed to characterize clinical manifestations and managements of AAV patients with SRH. Method: Hospitalized AAV patients were screened from January 2000 to April 2021, at Peking Union Medical College Hospital (PUMCH). Also, a systematic review was based on retrieving all the relevant literature from PubMed, MedlinePlus, and Web of Science until April 2021. Clinical features, management, and prognosis of the patients were collected and concluded. Results: In PUMCH, four out of 1640 AAV patients with SRH were included in our study; three had granulomatosis with polyangiitis (GPA) and one had microscopic polyangiitis (MPA). The ratio of men to women was 3 to 1, and the average age of onset was 55 years. The Birmingham Vasculitis Activity Score (BVAS) ranged from 21 to 23. Combining with documented reports, 13 patients were diagnosed as AAV complicated with SRH (including four from PUMCH), 7 with GPA, and 6 with MPA. Mean BVAS was 25.2 ± 6.6. The symptoms of SRH presented as severe back or abdominal pain. Patients with SRH to age- and gender-matched patients without SRH were compared, and we found that in the SRH group, the duration of disease was shorter, and BVAS, renal function, and inflammatory markers (WBC and ESR) were significantly greater, whereas Hb, Alb, and renal function greatly reduced. Conclusion: This is the first summary of clinical features and treatments of SRH in AAV. Patients with AAV in early stage and with high disease activity appeared to be more likely to develop SRH. Key Points • This is the first summary of clinical features and treatments of SRH in AAV. • SRH more likely occurs in AAV patients in the early stage (≤ 3 months) and with high disease activity. • Clinicians should be aware of the possibility of SRH when AAV patients complain of back or abdominal pain. [ABSTRACT FROM AUTHOR]

Introduction: Patients with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis and idiopathic interstitial lung diseases (IIPs) are positive for myeloperoxidase (MPO)–ANCA. MPO–ANCA-positive vasculitis mainly comprises microscopic polyangiitis (MPA) and unclassifiable vasculitis. These diseases are frequently complicated by interstitial lung disease (ILD). Few studies have reported the clinical differences between the subtypes of MPO–ANCA-positive ILD. Therefore, this study aimed to examine the clinical findings and courses of MPO–ANCA-positive ILD. Method: This retrospective study enrolled 100 patients with MPO–ANCA-positive ILD who were categorized into three groups: MPA (n = 44), unclassifiable vasculitis (n = 29), and IIP (n = 27). Our study compared the clinical findings and prognosis of these patients and analyzed the poor prognostic factors. Furthermore, we assessed the association between the patients with and without acute exacerbation of ILD (AE-ILD). Results: Our study found clinical differences in serum markers, clinical symptoms, and treatment regimens among the three groups. ILD complications, as the main cause of death, differed among the three groups (P = 0.04). Patients with unclassifiable vasculitis showed higher survival rates than those with IIP (P = 0.046). Patients with AE-ILD showed fewer general symptoms (P = 0.02) and lower survival rates (P < 0.01) than those without AE-ILD. In multivariate analysis, AE-ILD development was a strong poor prognostic factor for MPO–ANCA-positive ILD. Conclusions: The subtypes of MPO–ANCA-positive ILD have different clinical features and prognoses. Patients who develop AE-ILD require careful evaluation of clinical courses. Key Points • In myeloperoxidase (MPO)–antineutrophil cytoplasmic antibody (ANCA)–positive interstitial lung disease (ILD), patients with unclassifiable vasculitis showed a better prognosis than those with idiopathic ILD.. • Development of acute exacerbation in ILD was a strong poor prognostic factor in patients with MPO–ANCA-positive ILD.. [ABSTRACT FROM AUTHOR]

Avacopan (TAVNEOS®), a first-in-class, small molecule, selective antagonist of the complement fragment 5a receptor (C5aR), is an effective and glucocorticoid-sparing treatment option with a manageable tolerability profile for granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). It is administered orally in combination with standard therapy (e.g. rituximab, cyclophosphamide, glucocorticoids). In a 52-week, multinational phase 3 study in patients (most of whom were adults) with GPA and MPA, the avacopan regimen was non-inferior, but not superior, to the tapered prednisone regimen in achieving remission at week 26, and superior to the prednisone regimen in sustaining remission at week 52. It may also have beneficial effects on relapse rates, glucocorticoid-induced toxicity, kidney function and health-related quality of life. In this study, overall infections (a major driver of early mortality in this patient population), serious infections and serious opportunistic infections were less frequent with avacopan than tapered prednisone. Plain Language Summary: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are conditions that result in inflamed small- to medium-sized blood vessels in various organs, including the lungs, kidneys, eyes, ears, nose, throat, skin and nervous system. Rapid diagnosis and treatment are critical, with the organ damage (which is often cumulative and irreversible) resulting from obstructed or broken vessels leading to several poor outcomes. Current treatment options (e.g. glucocorticoids) are, however, associated with a range of toxicities. Avacopan (TAVNEOS®) is an orally administered drug that targets a specific receptor involved in the disease process. Compared with a gradually reduced dose of the glucocorticoid prednisone, avacopan was as effective in achieving remission at week 26 and superior in sustaining remission at week 52 in individuals with GPA or MPA. Avacopan may permit the accompanying glucocorticoid dose to be reduced, thereby reducing glucocorticoid-induced toxicity, and may improve relapse rates, kidney function and quality of life. Infections are a major driver of early mortality in individuals with GPA and MPA but were less frequently seen with avacopan than prednisone. Thus, oral avacopan is an effective and glucocorticoid-sparing treatment option with a manageable tolerability profile for adults with GPA and MPA. [ABSTRACT FROM AUTHOR]

Introduction/objectives : Birmingham vasculitis activity score (BVAS) version 3 (BVAS 3.0) and BVAS/granulomatosis with polyangiitis (BVAS/GPA) are used as indicators of disease activity in anti-neutrophil cytoplasmic antibody-associated vasculitis. We evaluated the association between these indices and the significance in patients with GPA and microscopic polyangiitis (GPA/MPA). Methods: We retrospectively reviewed the records of 203 patients with GPA/MPA in our hospital. The correlation between BVAS 3.0 and BVAS/GPA with the five-factor score (FFS) and laboratory data was investigated. The episodes of all-cause mortality, end-stage renal disease, and disease relapse were counted as adverse clinical outcomes. Multivariate Cox hazard analyses were performed to assess the relationships between both indices and patient outcomes. Results: Sixty-five (32.0%) and 138 (68.0%) patients with GPA and MPA were included. The median BVAS 3.0 was significantly higher in patients with MPA than in those with GPA (13.0 vs. 11.0, p = 0.015), whereas BVAS/GPA was higher in patients with GPA (4.0 vs. 3.0, p = 0.001). BVAS 3.0 and BVAS/GPA correlated significantly (r = 0.670, p < 0.001); both BVAS 3.0 and BVAS/GPA were shown to be associated with the outcomes investigated in separate Cox models. However, the correlation between BVAS 3.0 and BVAS/GPA was especially higher in a subgroup of patients with MPA than in those with GPA (MPA: r = 0.817, p < 0.001 vs. GPA: r = 0.570, p < 0.001) and with renal involvement (r = 0.676, p < 0.001). Conclusions: Although both BVAS 3.0 and BVAS/GPA significantly correlated and predicted outcomes well in those with GPA/MPA, a discord was observed based on disease subtypes and organ involvement. Key Points • BVAS 3.0 and BVAS/GPA significantly correlated and predicted outcomes in those with GPA/MPA. • A discordance was also observed based on disease subtypes and organ involvement. [ABSTRACT FROM AUTHOR]

Microscopic polyangiitis (MPA) is a systemic small-vessel vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA) and predominantly causes kidney and pulmonary injuries. Subarachnoid hemorrhage, a life-threatening manifestation of the central nervous system (CNS), rarely occurs in patients with ANCA-associated vasculitis (AAV). We report the case of a young man with spontaneous SAH recurrence and active nephritis. The patient was treated with a glucocorticoid pulse and intravenous cyclophosphamide (CTX) in combination with decreasing cerebral perfusion pressure and analgesic therapy. All the patients' symptoms except the proteinuria resolved. We reviewed the clinical characteristics of 34 previously reported cases of SAH with AAV, comprising six cases of MPA, eight cases of granulomatosis with polyangiitis (GPA), and 19 cases of eosinophilic granulomatosis with polyangiitis (EGPA), and one case of unclassified AAV. All the cases showed features of active vasculitis. Concomitant nephritis and peripheral neuropathy were found in the MPA and EGPA cases with SAH, respectively. Renal and pulmonary manifestations were predominant in the patients with GPA and SAH. Ten patients had aneurysmal abnormalities, and six patients had cardiac abnormalities. Thirty-one patients were treated with glucocorticoids, and 18 patients received concurrent immunosuppressants. Patients with SAH had a mortality rate of 38.2%. The presence of cerebrovascular events or cardiac involvement in patients with AAV and SAH is associated with increased mortality of 64.3%. Our study indicates that SAH should be cautioned as a disease occurring in patients with AAV. Early diagnosis with aggressive immunosuppressive therapy can help improve the prognosis of patients with SAH. [ABSTRACT FROM AUTHOR]

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), that share features of pauci-immune small-vessel vasculitis and the positivity of ANCA targeting proteinase-3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). AAV syndromes are rare, complex diseases and their aetio-pathogenesis is mainly driven by the interaction between environmental and genetic factors. In patients with GPA and MPA, the genetic associations are stronger with ANCA specificity (PR3- versus MPO-ANCA) than with the clinical diagnosis, which, in keeping with the known clinical and prognostic differences between PR3-ANCA-positive and MPO-ANCA-positive patients, supports an ANCA-based re-classification of these disorders. EGPA is also made up of genetically distinct subsets, which can be stratified on ANCA-status (MPO ANCA-positive versus ANCA-negative); these subsets differ in clinical phenotype and possibly in their response to treatment. Interestingly, MPO-ANCA-positive patients with either MPA or EGPA have overlapping genetic determinants, thus strengthening the concept that this EGPA subset is closely related to the other AAV syndromes. The genetics of AAV provides us with essential information to understand its varied phenotype. This Review discusses the main findings of genetic association studies in AAV, their pathogenic implications and their potential effect on classification, management and prognosis. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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Data on anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) are limited in children. This study is to determine the clinical features and outcomes of childhood-onset AAV. A retrospective study was performed on patients who were diagnosed with AAV before 18 years old in Xiangya Hospital. Their medical records were analyzed by retrospective review. Sixteen patients were diagnosed with AAV before 18 years old in the past 9 years, with an average age of 13.3 ± 3.3 years and 13 of them were female. There were 15 patients with microscopic polyangiitis (MPA) and 1 with Wegener's granulomatosis. The interval between onset of disease and diagnosis of AAV was 2 (1.5–3) months. Most patients (15/16, 93.8%) had multi-organ involvement, and all patients had renal involvement with 7 (43.8%) patients requiring dialysis at presentation. Eleven patients underwent a renal biopsy, of which mixed class and sclerotic class were the most two common histological types. All patients received immunosuppressive therapy for induction therapy including intravenous administrations of methylprednisolone (MP) pulse therapy for 8 patients. 8 patients (50%) achieved remission after induction therapy. After a median follow-up of 46.3 ± 36.1 months, nine (56.3%) patients progressed to end-stage renal disease (ESRD) and 5 (31.3%) patients died. Childhood-onset AAV showed similar clinical and pathological features compared to those of adults, except that it usually occurs in girls. The most commonly involved organ was the kidney, and it had a high risk of progression to ESRD. Early diagnosis and initiation of appropriate immunomodulatory therapy would be important to improve outcomes. [ABSTRACT FROM AUTHOR]

Introduction: The aim of this work was to assess the impact of prolonged low immunoglobulin (IgG or IgM) serum concentrations on the potential cumulative serious infection (SI) risk in pediatric patients following rituximab treatment for granulomatosis with polyangiitis or microscopic polyangiitis (GPA/MPA) in PePRS. Methods: Patients aged ≥ 2 to < 18 years received four weekly intravenous rituximab infusions of 375 mg/m2 and concomitant glucocorticoid taper. After 6 months, patients could receive further rituximab and/or other immunosuppressants per investigator discretion. Immunoglobulin levels and SIs were assessed throughout the 4.5-year observation period. Prolonged low IgG or IgM was defined as below the lower limit of normal age-specific reference range for ≥ 4 months. Results: A total of 25 patients were included, of whom 19 (76%) had GPA and six (24%) had MPA; 18 (72%) had newly diagnosed disease and seven (28%) had relapsing disease. All 25 patients completed the rituximab induction regimen; 24 completed ≥ 18 months of follow-up. At month 18, eighteen patients (72%) had prolonged low IgG; 19 (76%), prolonged low IgM; and 15 (60%), both. Seven patients (28%) had nine SIs; one occurred during or after prolonged low IgG only, two during or after prolonged low IgM only, and six during or after concurrent prolonged low IgG and IgM. No patients died or discontinued the study due to SI. All patients had complete and sustained peripheral B-cell depletion for ≥ 6 months. Conclusions: The majority of pediatric patients who received rituximab for GPA/MPA with prolonged low immunoglobulin levels did not experience SIs. In patients with SIs, these events were manageable, and the number of SIs did not increase over time or with multiple rituximab treatments. These observations are consistent with the rituximab safety profile in adults with GPA/MPA. Trial registration: ClinicalTrials.gov identifier, NCT01750697. [ABSTRACT FROM AUTHOR]

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic disorder that frequently affects the peripheral nervous system and consists of three distinct conditions: microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, previously Wegener's granulomatosis), and eosinophilic granulomatosis with polyangiitis (EGPA, previously Churg-Strauss syndrome). The neuropathic features associated with this condition usually include mononeuritis multiplex, which reflects the locality of lesions. Findings suggestive of vasculitis are usually found in the epineurium and occur diffusely throughout the nerve trunk. Nerve fiber degeneration resulting from ischemia is sometimes focal or asymmetric and tends to become conspicuous at the middle portion of the nerve trunk. The attachment of neutrophils to endothelial cells in the epineurial vessels is frequently observed in patients with ANCA-associated vasculitis; neutrophils play an important role in vascular inflammation by binding of ANCA. The positivity rate of ANCA in EGPA is lower than that in MPA and GPA, and intravascular and tissue eosinophils appear to participate in neuropathy. Immunotherapy for ANCA-associated vasculitis involves the induction and maintenance of remission to prevent the relapse of the disease. A combination of glucocorticoids along with cyclophosphamide, rituximab, methotrexate, or mycophenolate mofetil is considered depending on the severity of the condition of the organ to induce remission. A combination of low-dose glucocorticoids and azathioprine, rituximab, methotrexate, or mycophenolate mofetil is recommended to maintain remission. The efficacy of anti-interleukin-5 therapy (i.e., mepolizumab) was demonstrated in the case of refractory or relapsing EGPA. Several other new agents, including avacopan, vilobelimab, and abatacept, are under development for the treatment of ANCA-associated vasculitis. Multidisciplinary approaches are required for the diagnosis and management of the disorder because of its systemic nature. Furthermore, active participation of neurologists is required because the associated neuropathic symptoms can significantly disrupt the day-to-day functioning and quality of life of patients with ANCA-associated vasculitis. [ABSTRACT FROM AUTHOR]

Little is known about the epidemiology of systemic vasculitis in South American countries. The aim of this study is to compare the prevalence of systemic vasculitides in two vasculitis referral centers from Brazil and Peru. A cross-sectional study was performed and all patients above 18 years of age, with at least 6 months of follow-up and who met classification or diagnosis criteria for the most common forms of vasculitis, were included. A total of 562 patients with systemic vasculitis were analyzed, 345 (61.4%) from Brazil and 217 (38.6%) from Peru. The frequency of Behçet's disease (37.9% vs. 1.8%; p < 0.0001), Takayasu arteritis (TAK) (25.2% vs. 6.9%; p < 0.0001), and giant cell arteritis (9.8% vs. 0.9%; p < 0.0001) was higher in the Brazilian center than the Peruvian one. On the other hand, the frequency of microscopic polyangiitis (MPA) (67.3% vs. 2.8%; p < 0.0001) and renal-limited vasculitis (2.8% vs. 0.0%; p = 0.009) was higher in the Peruvian center. No differences were found concerning other forms of vasculitis. At diagnosis, Brazilian patients with TAK, granulomatosis with polyangiitis, and MPA were younger than Peruvian patients. Epidemiologic differences in the frequency of systemic vasculitis are observed between a vasculitis referral center from Brazil and another from Peru. Key Points • Significant differences are observed regarding the epidemiologic profile of systemic vasculitis between Brazil and Peru. • MPA is the predominant form of vasculitis in Peru while BD and TAK are the most frequent forms of vasculitis in Brazil. • The age at diagnosis of TAK, MPA, and GPA was lower in Brazilian patients than in Peruvian patients. [ABSTRACT FROM AUTHOR]

Diffuse alveolar hemorrhage (DAH) is a rare but potentially life-threatening emergency that has both immune and non-immune etiologies. The objective of this investigation was to compare the risk factors and outcomes of immune and non-immune causes of DAH at a tertiary-care academic center. This was a retrospective observational study conducted at a University center. We reviewed all chest radiographs spanning 12 years (2007–2019) at our institute with the words "diffuse alveolar hemorrhage" in the body of their report, and ascertained cases of DAH through a detailed chart review. We used Chi-squared test to determine the differences in risk factors and outcomes between immune versus non-immune causes of DAH. We performed logistic regressions to assess whether baseline demographics and clinical features influence four critical outcomes: death, shock, renal failure, and severe anemia requiring transfusions. Over the 12-year period, there were 88 patients with DAH, 55 with non-immune and 33 with immune etiologies. Among immune causes of DAH, granulomatosis with polyangiitis (GPA) (10.2%), microscopic polyangiitis (MPA) (9%) and systemic lupus erythematosus (SLE) (9%) were most common. Among non-immune causes of DAH, coagulopathy (6.8%), decompensated heart failure (4.5%) and infection (3.4%) were most common. Patients with non-immune causes of DAH were 45.8% more likely to die and 20.7% less likely to experience sustained remission (p = 0.001). Patient with immune causes of DAH were 21% more likely to have extra-pulmonary findings and 23.7% more likely to have received hemodialysis (HD). The presence of extra-pulmonary findings was statistically significantly correlated with the number of blood products received, the need for HD and non-statistically significantly correlated with likelihood of death. Patients with immune causes of DAH were 71.5% more likely to receive multimodal therapy including corticosteroids. Immune-mediated DAH is associated with a better prognosis than non-immune DAH, despite its greater association with extra-pulmonary findings and requirement for hemodialysis. [ABSTRACT FROM AUTHOR]

Copyright of Der Internist is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a heterogeneous group of conditions resulting in frequent hospitalizations and high in-hospital mortality (IHM). Our study aimed to use the National Inpatient Sample (NIS) to determine and categorize the main reasons for hospital admission and IHM in patients with AAV. Methods: We performed a retrospective study of adult AAV hospitalizations in 2016, 2017, and 2018 in acute care hospitals across the USA conducted using the NIS database. We classified the main reasons for hospital admission and IHM into 19 different categories using the principal International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10) diagnosis. Results: A total of 41,155 hospitalizations had either a principal or secondary ICD-10 code for AAV (GPA or MPA). Rheumatologic and respiratory diagnoses were the most common reasons for hospitalization, while infectious and respiratory diagnoses were the most common reasons for IHM. Sepsis, unspecified organism A41.9, was the most common specific principal diagnosis for hospitalized and deceased AAV patients. Conclusions: Our results show that the leading reasons for hospitalization and mortality for AAV patients were rheumatologic, respiratory, and infectious diagnoses. This data suggests that careful monitoring and management of infectious and pulmonary complications in AAV may improve hospital outcomes. Key points • AAV is a heterogeneous group of conditions resulting in frequent hospitalizations and high IHM. In our study, AAV hospitalizations ended in IHM 4.5% of the time, substantially greater than non-ANCA patients. • The leading reasons for hospital admission for AAV patients were rheumatologic and respiratory diagnoses, but the main reason for IHM were infectious and respiratory diagnoses. • Sepsis was the most common principal diagnosis for hospitalized and deceased AAV patients. • Our results highlight the importance of close monitoring and timely management of infectious and respiratory complications to improve hospitalization outcomes. [ABSTRACT FROM AUTHOR]

ANCA-associated vasculitis (AAV) in general involves small blood vessels and includes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Although reported in a few studies, the prevalence of large vessel vasculitis (LVV) in patients with AAV remains to be further explored. The goal of the present study was to assess the prevalence of LVV in a cohort of patients with AAV and to characterize this population. We conducted a ten-year retrospective study of a single-center cohort of AAV, including 101 patients with GPA (n = 58), EGPA (n = 28), MPA (n = 15), and compared the groups with or without associated LVV. LVV was diagnosed in five patients, two with aortitis and three with temporal arteritis, corresponding to a total prevalence of 5.0% [95% CI 1.6–11.2%]. This value was significantly higher than the estimated prevalence of LVV in the normal Swiss population (OR 234.9 95% CI 91.18–605.2, p < 0.001). All five patients had GPA, whereas no cases with EGPA or MPA were identified. Anti-PR3 antibodies were detected in four out of five patients, anti-MPO in one patient. Since LVV can occur in a significant proportion of patients with GPA, evaluation for LVV may be considered systematically in the diagnostic workup of AAV. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study was to evaluate the importance of MR imaging findings of musculoskeletal involvement of the lower limbs in diagnosing microscopic polyangiitis (MPA) vs polymyositis (PM) or dermatomyositis (DM). Materials and methods: This study included 13 patients diagnosed with MPA clinically and through histologically, and 38 diagnosed with PM/DM, who underwent MR imaging of the lower limbs prior to treatment. Axial and coronal short tau inversion recovery (STIR) images were reviewed retrospectively. Results: The sites affected by MPA were the lower legs in six (46%) patients and the thighs in seven (54%). Intramuscular hyperintensity and fascial hyperintensity were observed in all cases of MPA (100%). Fascial hyperintensity was more frequently encountered in MPA than in PM/DM (100% vs. 45%, p < 0.01). As the predominantly involved sites, the fascial regions were more frequently affected by MPA than by PM/DM (77% vs. 18%, p < 0.01). Diffuse subcutaneous fat hyperintensity was more frequently observed in MPA than in PM/DM (100% vs. 16%, p < 0.01). However, no significant differences in intramuscular hyperintensity (100% vs. 97%, p = 0.745) and subcutaneous fat hyperintensity (54% vs. 50%, p = 0.533) were found between MPA and PM/DM. Conclusion: Intramuscular hyperintensity and fascial hyperintensity have always been observed in MPA, and the predominantly affected sites were usually the fascial regions. Compared with PM/DM, fascial hyperintensity and diffuse subcutaneous fat hyperintensity were more frequent in MPA. [ABSTRACT FROM AUTHOR]