Antineutrophil cytoplasmic autoantibodies and clinical phenotype in patients with Churg-Strauss syndrome

Sinico, Renato Alberto; Bottero, Paolo; Guillevin, Loı̈c

doi:10.1016/j.jaci.2012.09.031

Cited by 12 publications

(4 citation statements)

References 7 publications

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“…53,54 Whether the presence or absence of ANCA defines distinct subgroups of disease pathogenesis is unclear, although constitutional symptoms, peripheral neuropathy, and glomerulonephritis are more common in patients with ANCA-positive EGPA, whereas cardiac involvement is more frequent in patients who are ANCA-negative. 55,56 Despite the fact that relapses of vasculitis seem to be more frequent in ANCA-positive EGPA, mortality might be higher in ANCA-negative EGPA, presumably related to increased cardiac involvement. 51 …”

Section: Egpamentioning

confidence: 99%

Eosinophils in vasculitis: characteristics and roles in pathogenesis

2014

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Eosinophils are multifunctional granular leukocytes that are implicated in the pathogenesis of a wide variety of disorders, including asthma, helminth infection, and rare hypereosinophilic syndromes. Although peripheral and tissue eosinophilia can be a feature of many types of small-vessel and medium-vessel vasculitis, the role of eosinophils has been best studied in eosinophilic granulomatosis with polyangiitis (EGPA), where eosinophils are a characteristic finding in all three clinical stages of the disorder. Whereas numerous studies have demonstrated an association between the presence of eosinophils and markers of eosinophil activation in the blood and tissues of patients with EGPA, the precise role of eosinophils in disease pathogenesis has been difficult to ascertain owing to the complexity of the disease process. In this regard, results of clinical trials using novel agents that specifically target eosinophils are providing the first direct evidence of a central role of eosinophils in EGPA. This Review focuses on the aspects of eosinophil biology most relevant to the pathogenesis of vasculitis and provides an update of current knowledge regarding the role of eosinophils in EGPA and other vasculitides.

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Section: Egpamentioning

confidence: 99%

Eosinophils in vasculitis: characteristics and roles in pathogenesis

2014

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“…Clinical features include hypereosinophilia, nasal polyposis, palpable purpura, peripheral neuropathy and a history of allergic asthma, usually improving just before the onset of vasculitis. Cardiac, pulmonary and gastrointestinal involvement with either vasculitic lesions or granulomatous and eosinophilic infiltration are also common [35]. Renal involvement is less frequent than in other AAV.…”

Section: Systemic Vasculitides: An Overviewmentioning

confidence: 97%

Intravascular immunity as a key to systemic vasculitis: a work in progress, gaining momentum

Ramirez

Maugeri

Sabbadini

et al. 2014

Clinical and Experimental Immunology

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SummaryVascular inflammation contributes to the defence against invading microbes and to the repair of injured tissues. In most cases it resolves before becoming apparent. Vasculitis comprises heterogeneous clinical entities that are characterized by the persistence of vascular inflammation after it has served its homeostatic function. Most underlying mechanisms have so far remained elusive. Intravascular immunity refers to the surveillance of the vasculature by leucocytes that sense microbial or sterile threats to vessel integrity and initiate protective responses that entail most events that determine the clinical manifestations of vasculitis, such as end-organ ischaemia, neutrophil extracellular traps generation and thrombosis, leucocyte extravasation and degranulation. Understanding how the resolution of vascular inflammation goes awry in patients with systemic vasculitis will facilitate the identification of novel pharmacological targets and bring us a step closer in each patient to the selection of more effective and less toxic treatments.

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“…Several additional studies confirm the usefulness of this ANCA‐centred approach at a clinical level and support the contention that some pathogenic mechanisms may vary depending on presence and type of ANCAs. For example, ANCA‐positive patients with EGPA have more prominent vasculitic manifestations . In addition, anti‐PR3‐positive patients respond better to rituximab (RTX) than to cyclophosphamide, but have a higher cardiovascular risk and are more likely to experience relapse after renal transplant when compared to MPO‐positive patients .…”

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confidence: 99%