S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

Schirmer, Jan Henrik; Moosig, Frank

doi:10.1007/s00393-017-0394-1

Cited by 67 publications

(30 citation statements)

References 233 publications

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“…Treatment is guided by severity of organ involvement and disease activity. Various cytotoxic immunosuppressants and the monoclonal anti-CD20 antibody rituximab are recommended for the induction and maintenance of remission ( 18 , 19 ). While treatment options have improved the prognosis of AAV, therapy de-escalation still carries an immanent risk of relapse ( 20 ).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Pathogenetic and Clinical Aspects of Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitides

et al. 2018

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Anti-neutrophil cytoplasmic autoantibodies (ANCA) targeting proteinase 3 (PR3) and myeloperoxidase expressed by innate immune cells (neutrophils and monocytes) are salient diagnostic and pathogenic features of small vessel vasculitis, comprising granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic GPA. Genetic studies suggest that ANCA-associated vasculitides (AAV) constitute separate diseases, which share common immunological and pathological features, but are otherwise heterogeneous. The successful therapeutic use of anti-CD20 antibodies emphasizes the prominent role of ANCA and possibly other autoantibodies in the pathogenesis of AAV. However, to elucidate causal effects in AAV, a better understanding of the complex interplay leading to the emergence of B lymphocytes that produce pathogenic ANCA remains a challenge. Different scenarios seem possible; e.g., the break of tolerance induced by a shift from non-pathogenic toward pathogenic autoantigen epitopes in inflamed tissue. This review gives a brief overview on current knowledge about genetic and epigenetic factors, barrier dysfunction and chronic non-resolving inflammation, necro-inflammatory auto-amplification of cellular death and inflammation, altered autoantigen presentation, alternative complement pathway activation, alterations within peripheral and inflamed tissue-residing T- and B-cell populations, ectopic lymphoid tissue neoformation, the characterization of PR3-specific T-cells, properties of ANCA, links between autoimmune disease and infection-triggered pathology, and animal models in AAV.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Pathogenetic and Clinical Aspects of Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitides

et al. 2018

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“…For therapy of AAV recommendations exist from the European League Against Rheumatism (EULAR), the European Renal Association – European Dialysis and TransplantAssociation (ERA-EDTA), the German Rheumatology Society and the EGPA Consensus Task Force [ 22 , 56 ].…”

Section: Anca-associated Vasculitides (Aav)mentioning

confidence: 99%

“…a recurrence with severe organ involvement, therapy with GC and CYC or RTX is recommended for remission induction (Table 4 ). In young patients, in whom preservation of fertility is of great importance, an RTX therapy should be preferred [ 56 , 64 ].…”

Section: Anca-associated Vasculitides (Aav)mentioning

confidence: 99%

Cerebral involvement in systemic vasculitides

Berlit

Krämer

2019

Neurol. Res. Pract.

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Cerebral vasculitis is a rare disorder but plays a major role in the differential diagnosis of stroke, encephalopathy and headache. This guideline was developed in order to support clinicians in the diagnosis and treatment of cerebral manifestations of systemic vasculitides and rheumatic diseases. It is based on a medline research and was developed in a modified Delphi process and approved by the involved societies. This article is an abridged and translated version of the guideline published in DGNeurologie:

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“…Kürzlich sind sowohl vom europäischen Rheumatologenverband EULAR als auch von deutschen Vaskulitisexperten Therapieempfehlungen (Deutschland: S1-Leitlinie) publiziert worden (▶ Abb. 3) (13,14).…”

Section: Granulomatose Mit Polyangiitis (Wegenersche Granulomatose)unclassified

Entzündlich-rheumatische Erkrankungen und neurologische Manifestationen

Röther¹,

Bickel²,

Kötter³

2018

Arthritis und Rheuma

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ZusammenfassungEntzündliche Erkrankungen des peripheren und zentralen Nervensystems sind oft eine Herausforderung, und regelmäßig stellt sich die Frage nach einer entzündlich-rheumatischen Grunderkrankung. Die Erstdiagnose einer Vaskulitis oder einer Kollagenose erfolgt sowohl in der Neurologie als auch in der Rheumatologie und daher benötigen Rheumatologen neurologisches Symptomwissen und Neurologen ein Verständnis der entzündlich-rheumatischen Krankheitszeichen. Die diagnostische Zuordnung kann eindeutig sein, wenn typische Symptomkonstellationen vorhanden und diagnostische Kriterien seitens der zerebralen Bildgebung und der Labordiagnostik passend sind. Häufig ist aber der Nachweis einer entzündlich-rheumatologischen Erkrankung erschwert, und eine enge Kooperation zwischen dem neurologischen und rheumatologischen Fachgebiet ist sinnvoll. Wir geben nachfolgend eine Übersicht über die häufigsten neurologischen Manifestationen entzündlich-rheumatologischer Erkrankungen und deren moderne Therapieoptionen.

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S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

Cited by 67 publications

References 233 publications

Pathogenetic and Clinical Aspects of Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitides

Pathogenetic and Clinical Aspects of Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitides

Cerebral involvement in systemic vasculitides

Entzündlich-rheumatische Erkrankungen und neurologische Manifestationen

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