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Ischemic Optic Neuropathies
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
While GCA is a granulomatous inflammation of the large vessels, AAV is a group of necrotizing vasculitis affecting the medium and small vessels.20 Temporal headache can misleadingly point to a diagnosis of GCA with vastly different outcomes. Pointers toward the AAV with TA involvement in this patient were peripheral neuropathy, otological and kidney involvement. The kidney biopsy findings were consistent with ANCA-associated vasculitis. Histopathological features of the left TAB in our patient cannot help in distinguishing AAV and GCA. There were no giant cells (seen in GCA) noted in the biopsy specimen but there was a break in the internal elastic lamina, which can happen in both the conditions. It is important to differentiate GCA-TA from AAV—TA as the initial choice and duration of immunosuppression is different in both the conditions as has been highlighted in the above case.12Non-arteritic-anterior ischemic optic neuropathy (NA-AION)
Vasculitis
Published in Jason Liebowitz, Philip Seo, David Hellmann, Michael Zeide, Clinical Innovation in Rheumatology, 2023
Michelle L. Robinette, Eli Miloslavsky, Zachary S. Wallace
Although the vasculature is well-suited for biomarker discovery, given direct apposition to the blood system, clinically useful markers specific to vasculitis are generally lacking. The most well-studied and frequently used disease-specific biomarker in vasculitis is the ANCA in AAV. First described in the 1980s, the expanding use of ANCA testing has led to improved recognition and earlier treatment initiation for patients with ANCA-associated vasculitis (98). In particular, the use of enzyme-linked immunosorbent assays (ELISAs) to detect ANCA specific for MPO or PR3 has largely replaced the use of immunofluorescence (e.g., perinuclear or cytoplasmic staining) in recent years (99, 100). This shift has made ANCA testing less subject to interobserver variability and improved performance overall, as detailed in recent studies.
Vasculitides
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Ivy M. Obonyo, Virginia A. Jones, Kayla A. Clark, Maria M. Tsoukas
Overview: Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) is an umbrella term used to classify three small-vessel vasculitis diagnoses as follows: GPA, EGPA, and MPA (Table 13.2). Due to significant overlap in clinical presentation, a positive ANCA can be used to further differentiate these diagnoses. ANCA autoantibodies can be differentiated by patterns found on immunofluorescence (IF). Cytoplasmic patterns found on IF are classified as cANCA, or PR3-ANCA, whereas perinuclear patterns are classified as pANCA, or MPO (myeloperoxidase)-ANCA. MPA and EGPA are likely to stain pANCA positive, whereas GPA is likely to stain cANCA positive. Although not diagnostic, the staining patterns of ANCA help guide clinicians when narrowing the differential diagnosis.
Early infectious risk in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis according to remission-induction therapy
Published in Scandinavian Journal of Rheumatology, 2023
M Gérard, H de Boysson, R Morello, N Martin-Silva, A-C Leroux, A Dumont, G Maigné, J Boutemy, K Khoy, D Mariotte, T Lobbedez, A Aouba, S Deshayes
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a heterogeneous group of autoimmune disorders characterized by small- to medium-vessel vasculitis affecting multiple organs, and is frequently associated with ANCA (1). AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). The introduction of therapy using cyclophosphamide (CYC), a cytotoxic agent, has dramatically improved the outcome of AAV patients, with 1 year survival rates increasing from 18% in the 1970s to 90% currently (2, 3). More recently, it has been shown that rituximab (RTX), an anti-CD20 monoclonal antibody, is an effective and safe alternative for CYC as a remission-induction therapy (RIT) (4, 5). Based on these data, European League Against Rheumatism (EULAR) recommendations state that, in the case of organ- or life-threatening forms of AAV, RIT relies on high-dose glucocorticoids and either CYC or RTX (6).
Vasculitis issue – introduction
Published in Postgraduate Medicine, 2023
Sophia Panaccione, David A. Cohen
Small vessel vasculitis, however, can be further categorized into two groups: antineutrophil cytoplasmic antibody (ANCA)-associated and immune-complex mediated. ANCA-associated vasculidities affect both small and medium vessels. The term ANCA-associated vasculitis is a broad term as it widely refers to auto-antibody-mediated neutrophil activation that ultimately results in small vessel inflammation and necrosis. This broad classification can further be broken down based on the auto-antibody’s preference for specific neutrophilic protein (myeloperoxidase or MPO versus proteinase 3 or PR3) which highlights that diagnosis of associated vasculidities relies not only on histological specificities but also on immunoassay and immunofluorescence findings. These vasculidities include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), all of which differ based on their pathology. GPA is commonly seen in adults between the ages of 40 and 60, and histology reveals necrotizing granulomatous inflammation. MPA statistically affects men more so than women across the fifth and sixth decades of life and histologically does not demonstrate granuloma formation and is classically associated with MPO antibodies. Lastly within this small vessel classification, is EGPA, which as the name highlights itself, is differentiated histologically by the presence of presence of eosinophilic infiltration along with findings of necrotizing vasculitis.
Endocarditis-associated rapidly progressive glomerulonephritis mimicking vasculitis: a diagnostic and treatment challenge
Published in Annals of Medicine, 2022
Sanxi Ai, Jianzhou Liu, Guotao Ma, Wenling Ye, Rongrong Hu, Shangzhu Zhang, Xiaohong Fan, Bingyan Liu, Qi Miao, Yan Qin, Xuemei Li
IE may mimic idiopathic vasculitis partly due to overlaps in clinical manifestations between the two entities. ANCA-associated vasculitis is a form of small-vessel vasculitis associated with ANCA, with predominant involvement of the upper and lower respiratory tract, kidney, skin and the nervous system. IE may closely mimic the clinical manifestations of ANCA-associated vasculitis, by presenting with similar clinical manifestations, including constitutional symptoms (fever, weight loss), involvement of skin, kidney, lung and the nervous system. Zhang et al. reviewed 27 cases of ANCA positive IE-related glomerulonephritis in literature, and involvement of skin, lung and the nervous system were present in 22.2%, 18.5% and 14.8% of patients respectively [6]. In our cohort of IE-related RPGN, involvement of skin, pulmonary and the nervous system were observed in 63%, 33% and 17% of patients respectively. IgA vasculitis is another form of small-vessel vasculitis characterised by IgA-dominant immune deposits. IE mimicking IgA vasculitis by manifesting as glomerulonephritis and purpura was rarely reported [9]. In our study, 50% (12/24) of patients with IE-related RPGN presented with purpura, overlapping with the manifestations of IgA vasculitis.