Antineutrophil cytoplasmic antibody-associated vasculitis, update on molecular pathogenesis, diagnosis, and treatment

Arman, Farid; Barsoum, Marina; Selamet, Umut; Shakeri, Hania; Wassef, Olivia; Mikhail, Mira; Rastogi, Anjay; Hanna, Ramy M

doi:10.2147/ijnrd.s162071

Cited by 11 publications

(11 citation statements)

References 43 publications

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“…ANCA-associated vasculitides (AAV) are a group of systemic vasculitides characterized by inflammation of smallsized blood vessels leading to multi-organ involvement [1][2][3][4][5]. AAV include three major clinic-pathologic entities: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) [6,7].…”

Section: Introductionmentioning

confidence: 99%

Healthcare and economic burden of ANCA-associated vasculitis in Italy: an integrated analysis from clinical and administrative databases

Quartuccio

Treppo

Valent³

et al. 2020

Intern Emerg Med

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ANCA-associated vasculitides (AAV) comprise a group of systemic vasculitides characterized by inflammation of small-sized blood vessels leading to multi-organ involvement. The worldwide annual incidence of AAV ranges from 1.2 to 3.3 cases per 100 000 individuals with a prevalence of 4.6-42.1 cases per 100 000 individuals. The prevalence of AAV is geographically heterogeneous; therefore, regional epidemiological studies can be more informative to improve health care systems. Even though clinicians are aware that the healthcare burden and the risk of hospitalization of AAV appear high, data on hospitalization and cost of illness due to AAV are still scarce or even lacking. This study aims to characterize the economic burden of AAV in Friuli Venezia Giulia (FVG), Italy. Thus, a retrospective study was conducted through the integration of many administrative health databases of the FVG as the source of information. From data integration, we estimated that more than two-thirds of AAV patients showed at least one hospitalization in their medical history, most frequently caused by the disease itself or superimposed infections. Around 10% of patients developed end-stage renal disease. In an 8-year follow-up, the overall healthcare cost was € 1,215,078, corresponding to € 6,168 patient-year. ANCA-positive patients showed much higher costs than ANCA-negative patients did. Overall, AAV are rare diseases, but imply very high healthcare costs. Early diagnosis and optimal treatment probably still remain unmet needs for AAV.

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Section: Introductionmentioning

confidence: 99%

Healthcare and economic burden of ANCA-associated vasculitis in Italy: an integrated analysis from clinical and administrative databases

Quartuccio

Treppo

Valent³

et al. 2020

Intern Emerg Med

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“…9 c-ANCAs specifically correspond to anti-PR3 antibodies and contribute to the clinical criteria used to diagnose GPA. 10 A majority of tested patients in this study were c-ANCA positive, thereby helping clinicians reach a diagnosis and initiate appropriate therapy. Furthermore, 48% of patients were PR3 positive, and 41% were positive for c-ANCA and PR3.…”

Section: Discussionmentioning

confidence: 80%

Cranial Base Manifestations of Granulomatosis with Polyangiitis

Kiessling

Marinelli

Peters

et al. 2020

Otolaryngol.--head neck surg.

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Objective Although granulomatosis with polyangiitis (GPA; Wegener’s granulomatosis) is classically characterized by systemic disease involving the kidneys and airway, approximately 10% of patients who have it present with isolated central nervous system disease. When involving the skull base, GPA frequently mimics more common pathology, resulting in diagnostic challenges and delay. The primary objective of this study is to characterize the cranial base manifestations of GPA, highlighting aspects most relevant to the skull base surgeon. Study Design Retrospective review. Setting Tertiary academic referral center. Subjects and Methods Retrospective analysis of all patients with skull base GPA treated at a tertiary referral center from January 1, 1996, to May 1, 2018. Results Twenty-nine patients met inclusion criteria. Twenty-one (72%) initially presented with skull base symptomatology as their cardinal manifestation of GPA. Twenty-four (82%) presented with cranial neuropathy at some point in their disease course. The trigeminal nerve was most commonly involved (12 of 24, 50%), followed by the facial (11 of 24, 46%) and optic (8 of 24, 33%) nerves. Eighteen patients reported hearing loss attributed to the GPA disease process, presenting as conductive, sensorineural, or mixed. The most common locations for GPA-derived inflammatory skull base disease on imaging included the cavernous sinus (12 of 29, 41%) and the orbit (7 of 29, 24%). Conclusion Establishing the diagnosis of skull base GPA remains challenging. Cranial neuropathy is diverse in presentation and often mimics more common conditions. Imaging findings are also unpredictable and frequently nonspecific. Careful review of patient history, clinical presentation, serology and biopsy results, and imaging can reveal important clues toward the diagnosis.

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“…Proteinase 3, myeloperoxidase, elastase, cathepsin G, lactoferrin, and lysozyme are intracellular proteins that ANCAs target in granulocytes. With a high degree of sensitivity and specificity, the P-ANCA and C-ANCA patterns match anti-MPO (97%) and anti-PR3 (80%) [19].…”

Section: Disease Pathologymentioning

confidence: 99%

“…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%

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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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Antineutrophil cytoplasmic antibody-associated vasculitis, update on molecular pathogenesis, diagnosis, and treatment

Cited by 11 publications

References 43 publications

Healthcare and economic burden of ANCA-associated vasculitis in Italy: an integrated analysis from clinical and administrative databases

Healthcare and economic burden of ANCA-associated vasculitis in Italy: an integrated analysis from clinical and administrative databases

Cranial Base Manifestations of Granulomatosis with Polyangiitis

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

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