Vasculitides
Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang in Roxburgh's Common Skin Diseases, 2022
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.
Inflammatory disorders of the larynx
Declan Costello, Guri Sandhu in Practical Laryngology, 2015
Granulomatosis with polyangiitis (GPA, formerly known as Wegener’s granulomatosis) is a potentially life-threatening, necrotising, autoimmune anti-neutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis. It can affect multiple systems including, typically, the kidneys and lungs. In general there is a slight male to female preponderance of 1.5:1 in adults and while GPA can present in all ages and ethnicities, the majority of cases globally involve patients between their third and fifth decade, who are of Northern European extraction.1 Many patients also have otolaryngological manifestations affecting the nose, larynx and subglottis, as well as the ear, in independent orders of frequency.2,3 No progressive pattern of organ involvement has been demonstrated and patients may present with dysphonia, dyspnoea and/or dysphagia due to perilaryngeal involvement as the first manifestations of GPA.
Vasculitis
Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley in Diagnostic Pulmonary Pathology, 2008
The treatment of ANCA-associated vasculitis depends on the severity of the disease, patient’s age, and renal function. With effective induction treatment, almost 80% patients achieve remission. Patients with WG, MPA, and CSS with one or more poor prognostic factors often respond to a combination of corticosteroids and cyclophosphamide to induce remission. Once remission is induced, less toxic immunosuppressants such as azathioprine and methotrexate are used for maintenance therapy (22). Plasma exchange is indicated as an adjuvant therapy if there is evidence of severe renal function impairment. Biologic therapies may be tried in patients who are refractory to conventional therapy. Anti-TNF-α, anti-CD20, anti-IL5, and anti-IgE monoclonal antibodies may be useful; however, their optimal regimens have not yet been defined.
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.
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.
Rationale for and clinical development of anti-fractalkine antibody in rheumatic diseases
Published in Expert Opinion on Biological Therapy, 2020
Sei Muraoka, Junko Nishio, Yoshikazu Kuboi, Toshio Imai, Toshihiro Nanki
Noninfectious vasculitis is categorized based on the predominant type of vessels involved [111]. Large vessel vasculitis includes Takayasu arteritis and giant cell arteritis; medium vessel vasculitis includes polyarteritis nodosa (PAN) and Kawasaki disease; and small vessel vasculitis is further divided into ANCA-associated vasculitis and immune complex (IC) small vessel vasculitis. ANCA-associated vasculitis includes microscopic polyangiitis (mPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis. IC small vessel vasculitis includes anti-glomerular basement membrane disease, cryoglobulinemic vasculitis, IgA vasculitis, and hypocomplementemic urticarial vasculitis. Other types of vasculitis have also been classified [111]. Systemic vasculitis affects small, medium, and large vessels, and causes various symptoms depending on the severity of inflammation and damage in vessels. In patients with ANCA-positive GN vasculitis, FKN mRNA is expressed in glomerular or tubulointerstitial lesions and strongly expressed in inflamed vascular sites. FKN is not expressed by infiltrating leukocytes [112]. The serum concentration of sFKN was found to be significantly higher in patients with mPA than in healthy subjects and correlated with disease activity [113]. CX3CR1 expression was also up-regulated on peripheral blood T cells in patients with mPA [113]. Moreover, the expression levels of FKN and CX3CR1 decreased with treatment-induced reductions in disease activity.