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Modern Rheumatology Mar 2024To revise the 2017 clinical practice guidelines (CPG) for the management of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) to reflect...
Clinical practice guidelines of the Japan Research Committee of the Ministry of Health, Labour, and Welfare for Intractable Vasculitis for the management of microscopic polyangiitis and granulomatosis with polyangiitis: The 2023 update - secondary publication.
OBJECTIVE
To revise the 2017 clinical practice guidelines (CPG) for the management of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) to reflect advancements in the field.
METHODS
Similar to the 2017 CPG, the Grading of Recommendations, Assessment, Development, and Evaluation system was adopted for this revision. The intended users of this CPG include patients diagnosed with MPA or GPA in Japan and their families and healthcare professionals, including specialists and non-specialists. Based on a scoping review, four clinical questions (CQs) of the 2017 guidelines were modified, and six new CQs were added.
RESULTS
We suggest a combination of glucocorticoid and cyclophosphamide or rituximab for remission induction therapy. In cases where cyclophosphamide or rituximab is used, we suggest the use of avacopan over high-dose glucocorticoid. Furthermore, we suggest against the use of plasma exchange in addition to the standard treatment in severe cases of MPA/GPA. Finally, we suggest the use of glucocorticoid and rituximab over glucocorticoid and azathioprine for remission maintenance therapy.
CONCLUSIONS
The recommendations have been updated based on patient preference, certainty of evidence, benefit and risk balance, and cost.
Topics: Humans; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antibodies, Antineutrophil Cytoplasmic; Cyclophosphamide; Glucocorticoids; Granulomatosis with Polyangiitis; Immunosuppressive Agents; Japan; Microscopic Polyangiitis; Rituximab
PubMed: 37599461
DOI: 10.1093/mr/road081 -
Innere Medizin (Heidelberg, Germany) Feb 2024Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune-mediated inflammation of small and medium-sized vessels that can affect... (Review)
Review
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune-mediated inflammation of small and medium-sized vessels that can affect virtually any organ system and bears the risk of irreversible organ damage. Without treatment the mortality rates are high, which necessitates rapid diagnosis and initiation of treatment. Histological confirmation, which is not feasible in all cases, should be strived for, especially to delineate differential diagnoses and vasculitis mimics. The new American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria are primarily designed for study purposes and show limitations in the routine application. Globally, the recently updated EULAR recommendations represent the most up to date management guidelines. Therapeutically, rituximab and cyclophosphamide in combination with glucocorticoids remain the pillars of treatment in remission induction for severe organ-threatening and life-threatening diseases. For the first time, mepolizumab and avacopan represent approved treatment options for specific entities that make a significant contribution to steroid reduction. New attention has been paid to patient-reported outcomes, for which a disease-specific outcome questionnaire is now available.
Topics: Humans; Immunosuppressive Agents; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Rituximab; Cyclophosphamide; Antibodies, Antineutrophil Cytoplasmic
PubMed: 38253699
DOI: 10.1007/s00108-023-01655-2 -
Rheumatology (Oxford, England) Jul 2023ANCA-associated vasculitis (AAV) is a group of multisystem diseases that can have several ocular manifestations. There are published data on ocular manifestations of...
OBJECTIVES
ANCA-associated vasculitis (AAV) is a group of multisystem diseases that can have several ocular manifestations. There are published data on ocular manifestations of granulomatosis with polyangiitis (GPA), but few for eosinophilic granulomatosis with polyangiitis (EGPA) or microscopic polyangiitis (MPA). There is little information concerning chronicity, complications, and association with other cranial manifestations of AAV.
METHODS
This study retrospectively analysed longitudinal multicentre cohorts of individuals with AAV followed between 2006 and 2022. Data included diagnosis, demographics, cranial manifestations of disease, presence of manifestations at onset of disease and/or follow-up, and ocular complications of disease. Univariate and multivariable logistic regression analysis assessed associations across disease manifestations.
RESULTS
Data from 1441 patients were analysed, including 395 with EGPA, 876 with GPA, and 170 with MPA. Ocular manifestations were seen within 23.1% of patients: 39 (9.9%) with EGPA, 287 (32.7%) with GPA, and 12 (7.1%) with MPA at any time in the disease course. There were more ocular manifestations at onset (n = 224) than during follow-up (n = 120). The most common disease-related manifestations were conjunctivitis/episcleritis and scleritis. In multivariable analysis, dacryocystitis, lacrimal duct obstruction, and retro-orbital disease were associated with sinonasal manifestations of GPA; ocular manifestations were associated with hearing loss in MPA. The most common ocular complications and/or damage seen were cataracts (n = 168) and visual impairment (n = 195).
CONCLUSION
Ocular manifestations occur in all forms of AAV, especially in GPA. Clinicians should be mindful of the wide spectrum of ocular disease in AAV, caused by active vasculitis, disease-associated damage, and toxicities of therapy.
Topics: Humans; Granulomatosis with Polyangiitis; Churg-Strauss Syndrome; Retrospective Studies; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Microscopic Polyangiitis; Scleritis; Antibodies, Antineutrophil Cytoplasmic
PubMed: 36440847
DOI: 10.1093/rheumatology/keac663 -
International Urology and Nephrology Nov 2023Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a group of diseases characterised by necrotizing inflammation of small vessels such as... (Review)
Review
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.
Topics: Humans; Antibodies, Antineutrophil Cytoplasmic; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Granulomatosis with Polyangiitis; Microscopic Polyangiitis; Immunosuppressive Agents; Kidney Diseases; Churg-Strauss Syndrome
PubMed: 37010734
DOI: 10.1007/s11255-023-03565-6 -
Porto Biomedical Journal 2023Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of rare diseases characterized by necrotizing inflammation... (Review)
Review
Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of rare diseases characterized by necrotizing inflammation predominantly of small vessels and the presence of these circulating antibodies. AAV comprises three important diseases, namely granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis, which affect multiple organ systems, significantly affecting patients' quality of life and survival. The diagnosis is established according to the clinical manifestations, detectable ANCA, and histopathology findings. Primary treatment strategies are adapted to the severity of the disease and based on immunosuppression with corticosteroids and cyclophosphamide, with increasing adoption of new, less toxic agents aimed at sustained remission of the disease, such as rituximab, methotrexate, and mycophenolate mofetil. Several international medical organizations have proposed recommendations for diagnosing and managing these diseases to standardize the procedures. In this study, we provide an up-to-date European perspective on AAV management, compiling current and relevant information regarding its epidemiology, symptoms, diagnosis, treatment strategies, and prognosis.
PubMed: 38093794
DOI: 10.1097/j.pbj.0000000000000237 -
Kidney International Reports Oct 2023Diagnosis and management of microscopic polyangiitis (MPA) have evolved considerably over the past decades, but it is unknown whether clinical and histological...
INTRODUCTION
Diagnosis and management of microscopic polyangiitis (MPA) have evolved considerably over the past decades, but it is unknown whether clinical and histological presentation and patient and renal outcomes have changed accordingly.
METHODS
We compared clinical and histopathological characteristic at diagnosis, risk of death, end-stage kidney disease (ESKD), and relapse rate in patients diagnosed with MPA between 1980 and 2022, after grouping them in 2 periods (p): p1980-2001 and p2002-2022. We compared the mortality rate between the 2 periods using Kaplan-Meier estimator and Cox-regression, and competing risks of ESKD and death using the Aalen-Johansen estimator, Fine-Gray multiple regression, and multistate models.
RESULTS
Out of 187 patients, 77 were in p1980-2001 and 110 in p2002 to 2022. Patients in p2002 to 2022 were older (66.2 ± 14.0 SD vs. 57.7 ± 15.8; < 0.001), had a better kidney function (estimated glomerular filtration rate [eGFR] 25.9 ± 24.8 vs. 21.5 ± 28.2 ml/min per 1.73 m; = 0.011) and a lower prevalence of the Berden sclerotic class (5.9 vs. 20.9%; = 0.011). Despite a similar crude and adjusted patient survival, the risk of ESKD decreased during p2002 to 2022 (subdistribution hazard ratio [HR] 0.30, 95% confidence interval [CI]: 0.16-0.57; < 0.001). The results remained significant after accounting for death after ESKD and after adjusting for potential confounders (HR 0.33 [95% CI: 0.18-0.63; < 0.001]). The risk of relapse was numerically higher during p2002 to 2022 (subdistribution-HR 1.64 [95% CI: 0.95-2.83; = 0.075]).
CONCLUSION
MPA kidney involvement has become less severe over the past decades, leading to a reduced risk of ESKD and a higher relapse rate, despite a comparable risk of death.
PubMed: 37850011
DOI: 10.1016/j.ekir.2023.07.008 -
Arthritis Research & Therapy Dec 2023To classify the different clinical phenotypes and compare the distinct prognoses of microscopic polyangiitis (MPA).
BACKGROUND
To classify the different clinical phenotypes and compare the distinct prognoses of microscopic polyangiitis (MPA).
METHODS
A retrospective analysis of 436 patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) from 2015 to 2022 was conducted in our center, of which 90 patients were diagnosed with MPA and underwent renal biopsy.
RESULTS
Among the 90 MPA patients, 63% were female, and the median age at onset was 63 years (25th-75th percentile: 58-68). The median follow-up time was 26 months (25th-75th percentile: 10-53). We identified four subtypes: renal impairment type (cluster 1, 39%), pure type (cluster 2, 22%), systemic inflammation type (cluster 3, 26%), and rapid progress type (cluster 4, 13%). Cluster 1, characterized by renal dysfunction at onset (80%), demonstrated poor prognoses with only 26% achieved complete remission (CR), 11% dying, and 19% developing renal failure. In contrast, patients in cluster 2, exclusively female, most had only kidney involvement showed the best prognoses with 55% achieving CR and none experiencing death or renal failure within 10 years. Cluster 3 mostly consisted of males; high fever and C-reactive protein levels were the primary characteristics. These cases exhibited moderate prognoses with 53% achieving CR, 9% dying, and 4% developing renal failure. Finally, patients in cluster 4, which was characterized by rapidly progressive glomerulonephritis, had the worst prognoses, with none achieving CR, 8% dying, and 75% developing renal failure despite aggressive treatment.
CONCLUSIONS
MPA is classified into four subtypes with distinct clinical manifestations and prognoses.
Topics: Male; Humans; Female; Middle Aged; Microscopic Polyangiitis; Retrospective Studies; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Prognosis; Antibodies, Antineutrophil Cytoplasmic; Kidney; Phenotype; Renal Insufficiency; Biopsy; Granulomatosis with Polyangiitis
PubMed: 38062524
DOI: 10.1186/s13075-023-03218-0 -
Indian Journal of Nephrology 2024ANCA associated vasculitides are multi-system autoimmune diseases which are increasing in prevalence. In this review we will discuss the clinical manifestations and... (Review)
Review
ANCA associated vasculitides are multi-system autoimmune diseases which are increasing in prevalence. In this review we will discuss the clinical manifestations and review the management options. We highlight the various trials of induction and maintenance therapy and discuss the areas of unmet need. These include understanding which patients are at highest risk of relapse, clinical adaptation of improved biomarkers of disease activity and tools to discuss long term prognosis.
PubMed: 38645911
DOI: 10.4103/ijn.ijn_346_23