BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis

Ntatsaki, Eleana; Carruthers, David; Chakravarty, Kuntal; D’Cruz, David; Harper, Lorraine; Jayne, David; Luqmani, Raashid; Mills, Joseph L.; Mooney, Janice; Venning, Michael; Watts, Richard

doi:10.1093/rheumatology/ket445

Cited by 270 publications

(238 citation statements)

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“…The most up-to-date guidelines on the treatment of the ANCA-associated vasculitides were published by the British Society for Rheumatology in 2014 (REF. 145) and the European League Against Rheumatism in 2016 (REF. 146) (FIG.…”

Section: Evidence From Systemic Vasculitismentioning

confidence: 99%

The nonsystemic vasculitic neuropathies

2017

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The vasculitides are a clinically diverse group of diseases with the histopathological signature of blood vessel-centred inflammation that results in vascular damage and ischaemic injury to the affected tissues 1 . Vasculitis can be caused by drugs, infections and cancers, but diagnostic workup reveals no trigger in most patients. Autoimmune mechanisms are active in all vasculitides, except those caused by direct infection of vessel walls. Most vasculitides are systemic and involve multiple organs and tissues. Those affecting small-to medium-sized vessels often manifest with a vasculitic neuropathy. For example, neuropathies occur in 60-70% of patients with polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis and cryoglobulinaemic vasculitis, and 40-50% of those with microscopic polyangiitis and rheumatoid vasculitis 2 .Vasculitic neuropathy can also occur without systemic vasculitis. This single-organ vasculitis of the PNS has commonly been referred to as nonsystemic vasculitic neuropathy (NSVN), but other forms of clinically isolated PNS vasculitis are now recognized and can be considered variants of NSVN 3 . Although NSVN is classically an acute, relapsing, multifocal neuropathy, it can present as a slowly progressive neuropathy without distinct asymmetries. As such, it should be considered as a possible cause of any progressive axonal neur opathy. NSVN can be diagnosed only with a nerve biopsy, with or without a concomitant muscle or skin biopsy; as nerve biopsies are seldom performed, the condition is under-recognized. The disorder must be distinguished from many other causes of multifocal neuropathy, the definition of which is itself not standardized and requires clarification.A Peripheral Nerve Society guideline group reviewed the literature on vasculitic neuropathy and published consensus recommendations on the classification, diagnosis and immunosuppressive treatment of NSVN in 2010 (REF. 4) Abstract | Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy. Variants include diabetic radiculoplexus neuropathy and -arguably -neuralgic amyotrophy. NSVN with proximal involvement is sometimes termed nondiabetic lumbosacral radiculoplexus neuropathy. Cutaneous polyarteritis nodosa and other skin-nerve vasculitides overlap with NSVN clinically. Three patterns of involvement in NSVN have been identified: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy (asymmetric polyneuropathy). These patterns lack standard defi...

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Section: Evidence From Systemic Vasculitismentioning

confidence: 99%

The nonsystemic vasculitic neuropathies

2017

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“…Zudem wurden bereits publizierte Empfehlungenberücksichtigt [168,175]. Basierend auf den Ergebnissen der Literatursuche, wurden in einem Konsensprozess zu vorab festgelegten Themenkomplexen die entnommenen Empfehlungen formuliert, und der Grad der Zustimmung unter den Autoren wurde ermittelt.…”

Section: S1-leitlinie Diagnostik Und Therapie Der Anca-assoziierten Vunclassified

S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

Schirmer

Moosig²

2017

Z Rheumatol

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“…Steroids, alone or combined with IVIg, or plasmapheresis are the best treatment options for CIDP (grade 1A) [37,38]. IV cyclophosphamide is the preferable treatment in vasculitic neuropathy (grade 1A) [22,39,40].…”

Section: Peripheral Neuropathiesmentioning

confidence: 99%

“…The EULAR, BSR, and BHPR guidelines highly recommend the use of immunosuppression in patients with ANCApositive vasculitides [39,40], which can also be applied in neurological manifestations of the disease. Nerve biopsy is also highly recommended.…”

Section: Antineutrophil Cytoplasmic Antibody-positive Vasculitidesmentioning

confidence: 99%

“…Induction treatment mainly consists of high-dose steroids and cyclophosphamide (grade 1A) [39,40], while in the most recent BSR and BHPR criteria, rituximab is considered to be an equal alternative for induction treatment, both at initial presentation and in refractory cases (grade 1A and 1B) [40]. Noninferiority of rituximab over cyclophosphamide was proven in the RAVE trial, a multicenter, randomized, double-blind, noninferiority study (grade 1A) [134], and it has also shown efficacy in a few patients with neurological involvement [135,136].…”

Section: Antineutrophil Cytoplasmic Antibody-positive Vasculitidesmentioning

confidence: 99%

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Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

et al. 2016

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Neurological involvement is relatively common in the majority of systemic autoimmune diseases and may lead to severe morbidity and mortality, if not promptly treated. Treatment options vary greatly, depending on the underlying systemic pathophysiology and the associated neurological symptoms. Selecting the appropriate therapeutic scheme is further complicated by the lack of definite therapeutic guidelines, the necessity to differentiate primary neurological syndromes from those related to the underlying systemic disease, and to sort out adverse neurological manifestations caused by immunosuppressants or the biological agents used to treat the primary disease. Immunotherapy is a sine qua non for treating most, if not all, neurological conditions presenting in the context of systemic autoimmunity. Specific agents include classical immune modulators such as corticosteroids, cyclophosphamide, intravenous immunoglobulin, and plasma exchange, as well as numerous biological therapies, for example antitumor necrosis factor agents and monoclonal antibodies that target various immune pathways such as B cells, cytokines, and co-stimulatory molecules. However, experience regarding the use of these agents in neurological complications of systemic diseases is mainly empirical or based on small uncontrolled studies and case series. The aim of this review is to present the state-of-the-art therapies applied in various neurological manifestations encountered in the context of systemic autoimmune diseases; evaluate all treatment options on the basis of existing guidelines; and compliment these data with our personal experience derived from a large number of patients.

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BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis

Cited by 270 publications

References 0 publications

The nonsystemic vasculitic neuropathies

The nonsystemic vasculitic neuropathies

S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

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