The vasculitides encompass a group of inflammatory conditions affecting the blood vessels with severe consequences including tissue ischemia, structural abnormalities, such as aneurysms/dissections, and end organ damage. The different forms are commonly classified based on the size of the blood vessel involved as large-vessel, medium-vessel, and small-vessel vasculitis. The American College of Rheumatology/Vasculitis Foundation recently published guidelines on the management of several forms of primary systemic vasculitides. In this review, the recommendations for giant cell arteritis, Takayasu arteritis, polyarteritis nodosa, granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis are discussed. We highlight the key recommendations, aspects where they diverge from other published guidelines, controversies, and areas of uncertainty.

To provide evidence-based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN).
Twenty-one clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for systemic, non-hepatitis B-related PAN. Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel.
We present 16 recommendations and 1 ungraded position statement for PAN. Most recommendations were graded as conditional due to the paucity of evidence. These recommendations support early treatment of severe PAN with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, and the use of imaging and tissue biopsy for disease diagnosis. These recommendations endorse minimizing risk to the patient by using established therapy at disease onset and identify new areas where adjunctive therapy may be warranted.
These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and imaging for patients with PAN.

To provide evidence-based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN).
Twenty-one clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for systemic, non-hepatitis B-related PAN. Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel.
We present 16 recommendations and 1 ungraded position statement for PAN. Most recommendations were graded as conditional due to the paucity of evidence. These recommendations support early treatment of severe PAN with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, and the use of imaging and tissue biopsy for disease diagnosis. These recommendations endorse minimizing risk to the patient by using established therapy at disease onset and identify new areas where adjunctive therapy may be warranted.
These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and imaging for patients with PAN.

The European initiative Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) aimed to optimize care for children with rheumatic diseases. Systemic vasculitides are very rare in children. Consequently, despite recent advances, paediatric-specific information is sparse. The lack of evidence-based recommendations is an important, unmet need. This study aimed to provide recommendations for diagnosing and treating children with rare forms of childhood systemic vasculitis.
Recommendations were developed by a consensus process in accordance with the European League Against Rheumatism standard operating procedures. A systematic literature review informed the recommendations, which were devised and evaluated by a panel of experts via an online survey, and two consensus meetings using nominal group technique. Recommendations were accepted when ⩾ 80% of experts agreed.
Ninety-three relevant articles were found, and 78 recommendations were accepted in the two consensus meetings. General, cross-cutting recommendations and disease-specific statements regarding the diagnosis and treatment of childhood-onset PAN, granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and Takayasu arteritis are provided.
These Single Hub and Access point for paediatric Rheumatology in Europe recommendations were formulated through an evidence-based consensus process to support uniform, high-quality standard of care for children with rare forms of paediatric systemic vasculitis.

Cutaneous polyarteritis nodosa (CPAN) is a rare cutaneous small- to medium-vessel vasculitis of unknown etiology. Clinically it ranges in manifestation from livedo reticularis to large cutaneous ulcers and necrosis. Prognosis is favorable and progression to systemic polyarteritis nodosa is rare. There are multiple treatment options, none of which have proven to be definitively effective. Cutaneous polyarteritis nodosa has been associated with abnormal antibody testing with elevations of antiphospholipid cofactor antibody, lupus anticoagulant, anticardiolipin antibody, and anti-β2-glycoprotein I-dependent cardiolipin antibodies, as well as elevated anti-phosphatidylserine-prothrombin complex antibody. These antibodies suggest increased risk for thrombosis and systemic diseases such as lupus or other autoimmune connective tissue disease. We present a case of asymptomatic CPAN and evaluate if treatment should be instituted for asymptomatic disease that presents with abnormal antibody findings.

Vasculitis, characterized by inflammation of vessel walls, is comprised of heterogeneous clinicopathological entities, and thus poses a diagnostic challenge. The most widely used approach for classifying vasculitides is based on the International Chapel Hill Consensus Conference (CHCC) nomenclature system. Based on the recently revised CHCC 2012, we propose computed tomography (CT) features of vasculitides and a differential diagnosis based on location and morphological characteristics. Finally, vasculitis mimics should be differentiated, because erroneous application of immunosuppressive drugs on vasculitis mimics may be ineffective, even deteriorating. This article presents the utility of CT in the diagnosis and differential diagnosis of vasculitides.

OBJECTIVE: Many patients with systemic necrotizing vasculitis (SNV) satisfy classification criteria of different disease entities when different classification systems are used. A new classification algorithm has been proposed recently by using the American College of Rheumatology criteria, Chapel Hill Consensus Criteria (CHCC) and Sorensen surrogate markers for a more uniform classification of patients suffering from these rare disorders.
METHODS: We applied this algorithm to patients diagnosed as having systemic vasculitis between 2007 and 2011. We also analyzed the data using this algorithm by incorporating the recently proposed revised CHCC nomenclature of vasculitis in place of the older criteria.
RESULTS: Seventy-nine patients with SNV were studied. One patient diagnosed as microscopic polyangiitis (MPA) had to be excluded from analysis as she had previously been diagnosed as having Behcet\'s disease. All patients of eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA) and MPA were reclassified to the same diagnostic subcategory after application of the algorithm. Three (16.7%) of 18 polyarteritis nodosa patients were unclassifiable after application of the consensus algorithm while two (11.1%) were reclassified as MPA. All previously unclassifiable patients could be classified either as MPA or GPA after application of the new algorithm. There was no difference in the results when the CHCC 2012 nomenclature was used instead of the older CHCC in the consensus algorithm.
CONCLUSIONS: The new classification algorithm is a reliable method for classification of SNV for epidemiological purposes in our population.

Vasculitis is a heterogeneous group of rare disorders in which inflammation of blood vessels is the common feature. Due to the increasing number of diseases as well as overlaps and gaps in the definition and nomenclature, the classification criteria were constantly changing in the past decades. The classifications were based essentially on the size of affected blood vessels and pathologic characteristics of inflamed vessel walls. The standard procedures and validated diagnostic criteria are missing from the diagnostics of vasculitis, thus in clinical practice the classification criteria are applicable. The 2012 Chapel Hill Consensus Conference brought a change in the definition, nomenclature and classification of previously uncategorized diseases. The definitions of subgroups accurately determine the diagnosis of the specific disease, and they are suitable for establishing homogeneous disease groups. By better understanding of the etiopathogenetic factors, further diseases and subgroups may be defined in the near future.
A vasculitisek a ritka betegségek olyan heterogén csoportja, amelyben közös jellemző az erek falának gyulladása. A szaporodó kórképek, a definícióban és a nevezéktanban mutatkozó átfedések és hiányosságok miatt a klasszifikációs kritériumok folyamatosan változtak. A beosztásoknál alapvető szempont volt az erek mérete és a gyulladásos érfal patológiai sajátossága. A vasculitis diagnosztikájában hiányoznak a standard eljárások és validált diagnosztikai kritériumok, így a klinikai gyakorlatban a klasszifikációs kritériumok az irányadók. A 2012-es Chapel Hill-i Konszenzuskonferencia változást hozott a definícióban, nevezéktanban és a hiányzó kórképek besorolásában. Az alcsoportok definíciója pontosan kijelöli az adott betegség diagnózisát és alkalmas homogén betegségcsoportok kialakítására. Az etiopatogenetikai tényezők jobb megismerésével a közeljövőben újabb betegségek, alcsoportok definiálása várható. Orv. Hetil., 2013, 154, 1571–1578.

暂无摘要。

BACKGROUND: The classification of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and polyarteritis nodosa (PAN) for epidemiology studies is confusing. The existing schemes such as American College of Rheumatology (ACR) criteria, Chapel Hill Consensus Conference (CHCC) definitions and Lanham criteria produce overlapping and conflicting classifications, making it difficult to compare incidence figures.
OBJECTIVE: To develop a consensus method of using these criteria and definitions for epidemiological studies to permit comparison without confounding by classification.
METHODS: A stepwise algorithm was developed by consensus between a group of doctors interested in the epidemiology of vasculitis. The aim was to categorise patients with Wegener\'s granulomatosis, microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and PAN into single clinically relevant categories. The ACR and Lanham criteria for CSS, and ACR criteria for Wegener\'s granulomatosis were applied first, as these were considered to be the most specific. Surrogate markers for Wegener\'s granulomatosis were included to distinguish Wegener\'s granulomatosis from MPA. MPA was classified using the CHCC definition and surrogate markers for renal vasculitis. Finally, PAN was classified using the CHCC definition. The algorithm was validated by application to 20 cases from each centre and 99 from a single centre, followed by a paper case exercise.
RESULTS: A four-step algorithm was devised. It successfully categorises patients into a single classification. There was good correlation between observers in the paper case exercise (91.5%; unweighted kappa = 0.886).
CONCLUSIONS: The algorithm achieves its aim of reliably classifying patients into a single category. The use of the algorithm in epidemiology studies should permit comparison between geographical areas.