Treatment Updates in Antineutrophil Cytoplasmic Autoantibodies (ANCA) Vasculitis

Jain, Koyal; Jawa, Pankaj; Derebail, Vimal K.; Falk, Ronald J.

doi:10.34067/kid.0007142020

Cited by 17 publications

(25 citation statements)

References 84 publications

(92 reference statements)

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“…Table 2 below summarizes the results of the final included studies [5][6][7][8][9][10][11][12][13][14][18][19][20][21][22].…”

Section: Figure 1: Prisma Flow Chart 2020mentioning

confidence: 99%

“…However, the use of GCs, particularly in high doses, resulted in undesirable adverse effects, including osteoporosis, diabetes, increased risk of infections, glucose-induced psychosis, and progressive organ damage [7,22] that was measured by the glucocorticoid toxicity index (GTI) [21]. Alternatively, the use of CYC has successfully achieved remission in 75% to 90% of patients [9] when treated with a combined regime with medications such as GCs or rituximab [4,6]. However, CYC is also associated with several adverse effects, including urotoxicity, hematologic toxicity, infertility, cystitis, transitional-cell cancer of the bladder, and an increased risk of infections [6], hence warranting new treatment modalities.…”

Section: Traditional Treatment and Management Modalitiesmentioning

confidence: 99%

“…Alternatively, the use of CYC has successfully achieved remission in 75% to 90% of patients [9] when treated with a combined regime with medications such as GCs or rituximab [4,6]. However, CYC is also associated with several adverse effects, including urotoxicity, hematologic toxicity, infertility, cystitis, transitional-cell cancer of the bladder, and an increased risk of infections [6], hence warranting new treatment modalities.…”

Section: Traditional Treatment and Management Modalitiesmentioning

confidence: 99%

“…GCs and CYC are often still employed despite advancements, though they may be combined with new treatment modalities [2,[9][10][11][12][13][14][15]. Prior to discussing combined techniques, Table 4 will address the rationale of various advanced treatment modalities [2,[4][5][6][7][8][9]15].…”

Section: Advancements In Treatment and Management Modalitiesmentioning

confidence: 99%

“…Long-established treatment options relied on glucocorticoids (GCs), often in combination with cyclophosphamide (CYC) [4]. However, recent advancements in the treatment and management of AAV have dramatically increased patient prognosis in recent years [3], including the use of rituximab, avacopan, plasma exchange, immunological targeting, and intravenous pulse methylprednisolone, with all demonstrating success in achieving remission [2,[4][5][6][7][8][9]. Additionally, effective contemporary treatment involves combining plasma exchange with GCs, rituximab with CYC, and rituximab with GCs [9][10][11][12][13][14][15][16].…”

Section: Introductionmentioning

confidence: 99%

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Recent Advancements in the Management of Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Systematic Review

Arzoun¹,

Srinivasan²,

Thangaraj³

et al. 2022

Cureus

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare multisystem autoimmune condition that causes inflammation of small and medium-sized blood vessels and is more commonly seen in the geriatric population. ANCA-associated vasculitis (AAV) is typically characterized as necrotizing vasculitis and includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). The mortality rate remains high, with especially cardiovascular disease, infections, and malignancies being the leading causes of death. Existing treatment options depend heavily on the use of glucocorticoids (GCs), often in combination with cyclophosphamide (CYC); however, as the multitude of adverse effects associated with these agents has increased, numerous studies are being conducted to reduce not only these harmful effects but also improve remission rates. Rituximab, avacopan, corticosteroids, including intravenous pulse methylprednisolone, plasma exchange, and immunological targeting are among the emerging treatments. The purpose of this review is to emphasize the pathogenesis and traditional treatment modalities and give insights into the recent advances in managing this disorder in an attempt to spare the adverse effects of conventional therapies while achieving better remission rates with combination therapies as well. The authors explored multiple databases, employing appropriate keywords, satisfying the quality appraisal, after which a total of 14 reports were included in this review. Upon overall analysis, it can be concluded that rituximab and CYC, when used in combination, provided a safer alternative to GCs while exhibiting equal, if not superior, effectiveness and results, thus, paving the way for additional in-depth research in a larger population of interest.

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“…Table 2 below summarizes the results of the final included studies [5][6][7][8][9][10][11][12][13][14][18][19][20][21][22].…”

Section: Figure 1: Prisma Flow Chart 2020mentioning

confidence: 99%

Section: Traditional Treatment and Management Modalitiesmentioning

confidence: 99%

Section: Traditional Treatment and Management Modalitiesmentioning

confidence: 99%

Section: Advancements In Treatment and Management Modalitiesmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Recent Advancements in the Management of Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Systematic Review

Arzoun¹,

Srinivasan²,

Thangaraj³

et al. 2022

Cureus

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The frequency of Treg subsets distinguishes disease activity in ANCA vasculitis

Agosto-Burgos

Iannone

et al. 2022

Clin & Trans Imm

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Objectives. T regulatory cells (Tregs) are a heterogeneous group of immunoregulatory cells that dampen self-harming immune responses and prevent the development of autoimmune diseases. In anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis, Tregs possess diminished suppressive capacity, which has been attributed to the expression of a FOXP3 splice-variant lacking exon 2 in T cells (FOXP3D2 CD4 + T cells). However, the suppressive capacity of Tregs varies between subsets. We evaluated the frequency of Treg subsets in ANCA vasculitis as a potential explanation for diminished suppressive capacity. Methods. We developed a custom mass cytometry panel and performed deep immune profiling of Tregs in healthy controls, patients with active disease and in remission. Using these data, we performed multidimensional reduction and discriminant analysis to identify associations between Treg subsets and disease activity. Results. Total Tregs were expanded in ANCA vasculitis, which was associated with remission and the administration of rituximab and/or prednisone. The frequency of FOXP3D2 CD4 + T cells did not distinguish disease activity and this population had high expression levels of CD127 and lacked both CD25 and Helios, suggesting that they are not conventional Tregs. The frequency of CXCR3 + , CD103 + and CCR7 + Tregs distinguished disease activity, and the combination of the frequency of these three Treg subsets segregated active patients from patients in remission and healthy controls. From these three subsets, the frequency of CXCR3 + Tregs distinguished patients with active disease with renal involvement. Conclusion. Treg heterogeneity can discriminate disease activity and should be explored as a biomarker of disease activity in ANCA vasculitis.

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Optimal management of ANCA-associated vasculitis before and during pregnancy: current perspectives

Pecher¹,

Henes

Henes³

2022

Arch Gynecol Obstet

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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.

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Treatment Updates in Antineutrophil Cytoplasmic Autoantibodies (ANCA) Vasculitis

Cited by 17 publications

References 84 publications

Recent Advancements in the Management of Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Systematic Review

Recent Advancements in the Management of Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Systematic Review

The frequency of Treg subsets distinguishes disease activity in ANCA vasculitis

Optimal management of ANCA-associated vasculitis before and during pregnancy: current perspectives

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