Chapel Hill Consensus Conference Research Articles

Classification des vascularites

Following the first case report of Polyarteritis Nodosa (PAN), by A. Kussmaul and K. Maier in 1866, PAN became the archetype of systemic vasculitis and was the only generic name used to designate diseases characterized by systemic vascular inflammation for almost 85 years. The first attempt to classify systemic vasculitis was published in 1952, by P.M. Zeek. As both basic and clinical knowledge improved, several iterations of classification systems emerged. In 1994, the “Chapel Hill Consensus Conference (CHCC) for the nomenclature of systemic vasculitis” (lately to be revised in 2013) was published and established as the current standard for the classification of vasculitis. The CHCC classification provides detailed information on classification concepts and disease definitions, thereby providing a clear and practical nomenclature. Yet, it is not intended to replace the otherwise available classification criteria nor to be used for diagnostic purposes. Classification criteria (namely the 1990 American College of Rheumatology criteria for vasculitis) highlight the most characteristic symptoms and clinical and laboratory features of diseases to allow for creating homogeneous and mutually exclusive patient populations in clinical, epidemiological and fundamental research studies. Here, we review the currently available nomenclature systems and classification criteria in the field of vasculitis and discuss their strengths and weaknesses.

Mortality in ANCA-associated vasculitis: ameta-analysis of observational studies

ObjectiveTo determine the magnitude of all-cause mortality risk in patients with antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) compared with the general population through a meta-analysis of observational studies.MethodsWe searched Medline and...

Hypocomplementemic urticarial vasculitis

Hypocomplementemic urticarial vasculitis (HUV), called anti-C1q vasculitis in the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides, is a rare systemic vasculitis of unknown etiology, affecting small vessels. HUV is characterized by urticarial lesions, hypocomplementemia and systemic manifestations, mostly musculoskeletal and ocular, but also gastrointestinal, pulmonary and kidney involvement. Anti-C1q antibodies are detected in only half of the patients, and low C1q seems to represent a more sensitive marker. Published data on the therapeutic management are scarce in the literature. Hydroxychloroquine and colchicine seem to represent effective first-line therapies. In patient with relapsing or refractory disease, response rates appeared slightly higher for corticosteroids together with conventional immunosuppressive agents, in particular azathioprine, mycophenolate mofetil, or cyclophosphamide, while a rituximab-based regimen tended to have higher efficacy. The best strategy for treating HUV has yet to be defined.

CT Features of Vasculitides Based on the 2012 International Chapel Hill Consensus Conference Revised Classification.

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.

AA Amyloidosis in a Cohort of 128 Patients with Takayasu's Arteritis

Systemic AA amyloidosis is a relatively rare complication of chronic inflammatory disorders, e.g. rheumatoid arthritis, ankylosing spondylitis, familial Mediterranean fever and other autoinflammatory syndromes, Crohn’s disease, malignancies and recurrent infections. Isolated case of AA amyloidosis have been reported in many other chronic diseases, including systemic vasculitides, diffuse connective tissue diseases, sarcoidosis, chronic hepatitis, etc. In the past ten years the incidence of AA amyloidosis decreased significantly due to earlier and more aggressive treatment of underlying conditions such as rheumatoid arthritis or infections. In the recent series of 474 patients with renal amyloidosis from Mayo clinic there were only 7% cases of AA amyloidosis while 86% patients presented with AL form of disease. Nevertheless, AA amyloidosis remains a life-threatening complication of immune-mediated and inflammatory conditions and deserves effective prevention and treatment. Takayasu’s arteritis (TAK) is a rare largevessel vasculitis of unknown etiology that more frequently affects women of childbearing age and is defined as granulomatous inflammation of the aorta and its major branches by the revised Chapel Hill Consensus Conference. The search in Pubmed database revealed only a dozen reports of AA amyloidosis in patients with TAK. The objective of our study was to evaluate the incidence of AA amyloidosis in a cohort of consecutive TAK patients.

Pachymeningitis in granulomatosis with polyangiitis: case series with earlier onset in younger patients and literature review.

The objective of this study is to describe the characteristics of patients with pachymeningitis (PM) in granulomatosis with polyangiitis (GPA) from Latin America, including three young patients. This is a retrospective case series. Patients were classified according to the ACR criteria, the 2012 Chapel Hill Consensus Conference Nomenclature and the EMA algorithm. Demographic, clinical, serological, and neuroimaging characteristics are described. Thirteen patients (nine females, four males) were identified. Mean age±SD of PM diagnosis was 35.5±20.4years (median 48, range 8-71years). Mean time±SD between GPA first symptom and PM diagnosis was 59.8±70.1months (median 48, range 2-252months). An important difference between children and adults was the median time elapsed between first GPA symptoms and PM diagnosis (range 2-4months vs 5-252months, respectively). Chronic headache was present in all, followed by intracranial hypertension (n=5), single cranial nerve palsy and orbital mass (n=4), seizures (n=3), cavernous sinus syndrome and multiple cranial nerve palsies (n=2), and meningism and cerebellar syndrome (n=1 each). At time of PM diagnosis, mean BVAS/WG (Birmingham Vasculitis Activity Score for Wegener's granulomatosis) was 4±2.4 and mean VDI of 2±1.6, mostly due to ENT damage. Gadolinium-enhanced brain MRI showed dural thickening in 12 patients and leptomeningeal enhancement in one. All received a combination of glucocorticoids plus immunosuppressants, rituximab being used favourably in one refractory case. Improvement was observed in 12 patients. Chronic headache should lead to suspect PM. PM predominates in localised GPA. Children may present it earlier in the disease course than adults. Treatment is non-standardised and remains difficult.

Scientific people named in the classification of vasculitis

The first International Chapel Hill Consensus Conference was held in 1994. There have been suggestions about the nomenclature of systemic vasculitis. Important categories were added to the classification of vasculitis, and many changes were made for disease names in the second Chapel Hill Consensus Conference 2012, which were not included in the Chapel Hill Consensus Conference 1994. The new nomenclature was introduced instead of being referred to by many names such as Churg-Strauss and Wegener”s. New categories such as Behçet”s and Cogan etc. were also added. These people are honored by the classification. They contribute to science through their case studies, scientific articles, and observations. This article reviews only eponyms present in the current classification of vasculitis. The aim of this paper is to give information about scientists mentioned in the classification of vasculitis.

Etiologies and prognostic factors of leukocytoclastic vasculitis with skin involvement: A retrospective study in 112 patients.

In this study, outcomes of patients with leukocytoclastic vasculitis (LCV) were analyzed focusing on clinical, histopathology and laboratory findings, relapses, and survival.Data from patients with cutaneous vasculitis diagnosed between January 1, 2000, and December 31, 2010, at Dijon University Hospital (France) were retrospectively reviewed. LCV was defined as perivascular neutrophilic infiltrate, endothelial cell nuclear swelling, extravasation of red blood cells, and/or fibrin deposition in vessels. Patients were classified according to the 2012 Chapel Hill Consensus Conference. Relapses were defined as the recurrence of vasculitis symptoms after a period of remission >1 month. Time to relapse and/or death was calculated from the date of diagnosis. Univariate and multivariate (Cox model) analyses were performed.A total of 112 patients (57 males and 55 females), with a mean age of 60 ± 19 (18–98) years, were analyzed. Overall follow-up was 61 ± 38 months. At diagnosis, all patients had skin lesions, purpura being the most common (n = 83). Lesions were associated with systemic involvement in 55 (51%) patients. Only 41 (36.6%) patients received specific treatment: glucocorticoids in 29 of 41 (70.7%) and immunosuppressants in 9 of 41 (22%). Sixty-two patients (55%) had LCV due to underlying causes, 29 (25.9%) had single-organ cutaneous small vessel vasculitis (SoCSVV), and 21 (18.8%) had unclassifiable LCV. Twenty patients of the cohort (18%) experienced relapse, 14 ± 13 (1–40) months after the diagnosis of LCV. None of the 29 patients with SoCSVV relapsed. Independent risk factors for relapse were vascular thrombosis in the biopsy [hazard ratio (HR) = 4.9; P = 0.017], peripheral neuropathy (HR = 9.8; P = 0.001), hepatitis (HR = 3.1; P = 0.004), and positive antineutrophil cytoplasm antibodies (ANCA, HR = 5.9 P = 0.005). In contrast, SoCSVV was a protective factor for relapse (HR = 0.12; P = 0.043).The 1-, 3-, and 6-year overall survival rates were 99%, 83%, and 71%, respectively, with no difference between relapsers and nonrelapsers (P = 0.960) or between SoCSVV and unclassifiable LCV (P = 0.588).This study demonstrates that global survival for LCV patients is good but relapses remain frequent, especially when the cutaneous biopsy shows vascular thrombosis, or in patients with peripheral neuropathy or hepatitis. Conversely, SoCSVV is a protective factor for relapse.

Nomes de cientistas usados na classificação das vasculites

The first International Chapel Hill Consensus Conference (CHHC) was held in 1994. There have been suggestions about the nomenclature of systemic vasculitis. Important categories were added to the classification of vasculitis, and many changes were made for disease names in the second CHHC 2012, which were not included in the CHCC 1994. The new nomenclature was introduced instead of being referred to by many names such as Churg‐Strauss and Wegener”s. New categories such as Behçet”s and Cogan etc. were also added. These people are honored by the classification. They contribute to science through their case studies, scientific articles, and observations. This article reviews only eponyms present in the current classification of vasculitis. The aim of this paper is to give information about scientists mentioned in the classification of vasculitis.

Connective Tissue Disorder-Associated Vasculitis.

Vasculitides secondary to connective tissue diseases are classified under the category of 'vasculitis associated with systemic disease' in the revised International Chapel Hill Consensus Conference (CHCC) nomenclature. These secondary vasculitides may affect any of the small, medium or large vessels and usually portend a poor prognosis. Any organ system can be involved and the presentation would vary depending upon that involvement. Treatment depends upon the type and severity of presentation. In this review, we describe secondary vasculitis associated with rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis, relapsing polychondritis, systemic sclerosis, Sjogren's syndrome and idiopathic inflammatory myositis, focusing mainly on recent advances in the past 3years.

Eosinophilic granulomatosis with polyangiitis in childhood: retrospective experience from a tertiary referral centre in the UK.

To describe the presenting clinical features, treatment and outcome in children with eosinophilic granulomatosis with polyangiitis (EGPA) and to define factors that predicted mortality. A retrospective case notes review of patients fulfilling the Chapel Hill Consensus Conference definition and/or ACR criteria for EGPA seen at Great Ormond Street Hospital, London. Demographics, clinical features, histopathology, treatment and outcomes were recorded. Descriptive statistics were expressed as median and range. Fisher's exact test was used for group comparisons. The Paediatric Vasculitis Activity Score and Paediatric Vasculitis Damage Index (PVDI) were calculated. Thirteen children (38% female) aged at diagnosis 14.1 (4-15.6) years were identified. The median time to diagnosis was 2 (0-7.3) years. History of asthma was documented in 76%. The most common presenting features were pulmonary (69%), skin (61%), gastrointestinal (46%), cardiac involvement (46%), paranasal sinus abnormality (38%), arthritis/arthralgia (38%) and neurological involvement (15%). Paediatric Vasculitis Activity Score at presentation was 8/63 (2-25/63); ANCA was negative in all 10/13 patients tested. Treatment included corticosteroids in all, combined with CYC in 38% or AZA in 23%. PVDI at 12 (3-48) months follow-up was 3/72 (0-13/72). Relapses were recorded in 46%. Mortality was 15%; cardiomyopathy and PVDI scores ⩾5 significantly associated with mortality risk (P = 0.012). EGPA in the paediatric population is a rare and potentially life-threatening vasculitis. Increased awareness is essential to secure a timely diagnosis and to promptly initiate treatment since our data emphasize a high mortality, particularly in those with cardiac involvement and significant accrued damage.

IgA vasculitis in adults: the performance of the EULAR/PRINTO/PRES classification criteria in adults.

BackgroundIn 2010, EULAR/PRINTO/PRES proposed new classification criteria for paediatric IgA vasculitis (IgAV) that have a higher diagnostic sensitivity than the 1990 ACR criteria. These criteria have so far not been evaluated in adults, in whom IgAV is considered as a rare disease. Our main objective was to compare the diagnostic performance of EULAR/PRINTO/PRES and ACR classification criteria in adult IgAV.MethodsAdult IgAV cases fulfilling the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (ICHCCNV) definition of IgAV at a secondary/tertiary rheumatology referral centre were critically reviewed in a partially retrospective and partially prospective manner. First, we compared the diagnostic sensitivity of ACR and EULAR/PRINTO/PRES criteria in this group of patients. Second, the diagnostic specificity of ACR and EULAR/PRINTO/PRES was determined by applying these criteria to a control group of patients with other systemic vasculitides.ResultsBetween 1 January 2010 and 31 December 2014 350 new cases of systemic vasculitis were identified. IgAV was diagnosed in 129, and other systemic vasculitides in 221 (123 had large, six medium and 92 small vessel vasculitis) cases according to ICHCCNV. The diagnostic sensitivity and specificity of the IgAV EULAR/PRINTO/PRES criteria were 99.2 % (95 % CI 95.4–99.9 %) and 86.0 % (95 % CI 80.7–90.3 %), and of the ACR criteria 86.8 % (95 % CI 79.7–92.1 %) and 81.0 % (95 % CI 75.2–85.9 %), respectively with an inter-criteria agreement of 77.5 % (95 % CI: 70.8–84.1 %).ConclusionsIn the adult population the EULAR/PRINTO/PRES IgAV classification criteria had a higher sensitivity and specificity than the ACR criteria.

Non-systemic Vasculitic Neuropathy

Non-systemic vasculitic neuropathy (NSVN) is a vasculitis confined to the peripheral nervous system. It is one of the most common causes of vasculitic neuropathy, along with microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). It remains unclear whether this disease is truly an isolated nosological entity, or part of the spectrum of systemic vasculitides. Anti-neutrophil cytoplasmic antibodies (ANCA) are negative in NSVN even though the size of affected vessels in this disease is similar to that in ANCA-associated vasculitis. Although the concept of single organ vasculitis was added to the 2012 revised International Chapel Hill Consensus Conference (CHCC) nomenclature of vasculitides, a description of NSVN has not yet been incorporated. Further studies are needed to elucidate the pathogenesis of NSVN, particularly a search for causative antibodies or participation of complement pathways. These studies may help clarify the position of NSVN along the spectrum of vasculitides based on CHCC nomenclature.

Comparison between classical polyarteritis nodosa and single organ vasculitis of medium-sized vessels: a retrospective study of 25 patients and review of the literature

Background:: Polyarteritis nodosa (PAN) is a rare form of systemic vasculitis, characterized by necrotizing inflammation of medium-sized vessels. In clinical practice, the distinction is made between a limited, mostly cutaneous, form and a generalized form. The Chapel Hill Consensus Conference of 2012 on the classification of the vasculitides classifies PAN as a medium vessel vasculitis, whereas the limited forms fall under the heading ‘single-organ vasculitis’ (SOV), with subdivions such as ‘cutaneous arteritis’ (formerly called cutaneous PAN) and ‘others’. In this last category, forms of PAN limited to a single organ (e.g. testicle, gall bladder or appendix) should be categorized. The relation between classical and limited forms of PAN remains enigmatic.Objective:: To compare demographics, clinical characteristics and prognosis between SOV and generalized PAN.Methods:: Clinical files of all patients with a diagnosis of classical or limited PAN made in the departments of general internal medicine and dermatology between 1983 and 2013 in a tertiary care university hospital were reviewed.Results:: The patients of the SOV group tend to be younger, with a female predominance, while we observed a male predominance in the generalized PAN group. Relapses were more common in SOV than in classical PAN. None of the patients initially diagnosed with cPAN/SOV progressed to generalized disease.Discussion:: Though SOV and classical PAN share a lot of similarities, they are probably different disease entities, based on their different demographical, clinical and prognostic characteristics. The 1990 ACR-criteria for classical PAN are too broad since they allow patients with limited disease to be classified as classic PAN.

Clinical Characteristics of Antineutrophil Cytoplasmic Antibodies Associated Vasculitis in the Elderly

Antineutrophil cytoplasmic antibodies (ANCAs) associated vasculitis is a necrotizing vascular inflammation, usually involving multiple organs. It is one of the major diseases that cause deterioration of renal function rapidly in the elderly. The latest Chapel Hill Consensus Conference 2012 has updated the name of ANCA-associated vasculitis. Some high quality randomized clinical trials provide the evident basis for the treatment of vasculitis. However, elderly patients often suffer complex diseases, and are associated with underlying diseases, so it is difficult to be diagnosed and treated. It is very important to make optimized individual strategies of treatment for elderly patients with vasculitis.

Diagnostic Difficulties of Cogan Syndrome

Cogan’s syndrome (CS) is a very rare disorder of unknown origin characterised by inflammatory eye disease and vestibuloauditory symptoms. According to the International Chapel Hill Consensus Conference Nomenclature of Vasculitides, CS is defined as “variable vessel vasculitis”. We present the case of a 44-year old male, primarily diagnosed and treated for large/medium vessel vasculitis probably due to indeterminate colitis, who finally developed CS. Difficulties in the diagnosis of this rare disease are also discussed.

Takayasu Arteritis and Pregnancy.

To assess the relationship between Takayasu arteritis (TAK) and pregnancy outcome. This study included 240 pregnancies in 96 patients fulfilling the American College of Rheumatology 1990 criteria for the classification of TAK and/or the 1994 Chapel Hill Consensus Conference nomenclature/criteria for vasculitis. We analyzed obstetric and maternal outcomes in women who were pregnant before and/or at the same time as or after TAK diagnosis. We assessed factors associated with complicated pregnancy. One hundred forty-two pregnancies occurred in 52 patients before TAK diagnosis (median age at pregnancy 26 years [interquartile range 23-30 years]), and 98 pregnancies occurred in 52 patients concomitant with or after TAK diagnosis (median age at pregnancy 28 years [interquartile range 26-31 years]). Pregnancies concomitant with or after TAK diagnosis had a 13-fold higher rate of obstetric complications compared to pregnancies before TAK diagnosis (odds ratio 13 [95% confidence interval 5-33], P < 0.0001). TAK was associated with a 40% frequency of obstetric complications, including preeclampsia/eclampsia (24 pregnancies [24%]), premature delivery (8 pregnancies [8%]), and intrauterine fetal growth restriction or death (5 pregnancies [5%]). Maternal complications of TAK occurred during 39% of pregnancies and included mainly new-onset or worsening hypertension (26 pregnancies [27%]). In multivariate analysis, smoking (odds ratio 6.15 [95% confidence interval 1.31-28.8]) and disease activity of TAK (a National Institutes of Health score of >1) (odds ratio 28.7 [95% confidence interval 7.89-104.7]) were independently associated with obstetric and maternal complications. TAK negatively affects pregnancy outcomes. Disease activity increases the risk of obstetric and maternal complications, mainly due to arterial hypertension.

Outcome assessment in Takayasu arteritis.

Takayasu arteritis (TAK) is a systemic granulomatous large-vessel vasculitis with a phenotype that overlaps with GCA and defined by the 1993 and 2012 Chapel Hill Consensus Conference statements. However, the diagnosis of TAK is often delayed since TAK patients may be asymptomatic or have non-specific symptoms. Once a diagnosis is made, it is difficult to judge remission or recurrence since there are no reliable assessment tools. With the availability of newer agents, such as cytokine blockade, which are being evaluated in GCA, there is the potential for real advances in TAK patient management. Without reliable assessment tools it will be difficult to introduce newer agents in an organized way or to optimally benefit patients in the future. In this article we review the use and performance of disease indicators in TAK clinical trials as a basis for the further development of assessment tools for this disease.

Inflammatory ulcera caused of vasculitides and vasculopathy

SummaryIn addition to traditional vascular causes ulceration of the lower leg as peripheral arterial disease and chronic venous insufficiency, it is primarily the vasculitis and vasculopathies, often are both diagnostically and therapeutically to a challenge.The disorder group ranked in the frequency distribution of the causes of a leg ulcer with &lt;10 % rarely in the area, but is much more complex of the pathogenesis, the clinical picture, the course of therapy and often much more complex.The revised International in 2012 Chapel Hill Consensus Conference (CHCC) Nomenclature of vasculitis is a first impression of the diversity of this group of diseases and makes it clear how important the medical history for diagnosis is.In the CHCC- categorizing the etiology, pathogenesis, the caliber of the vessel, the inflammatory type, the organs mainly affected, the clinical manifestation, genetic predisposition and demographic parameters were included.With this revision of the first CHCC nomenclature of 1994 took place an important adaptation to the latest findings. The group of vasculitis is divided specific, this facilitates the handling of the disease in clinical practice.The vasculopathies represent another rare fringe of the causes of an ulcer curis. These disorders are often as complex as a vasculitis and must be always used in difficult to treat ulcers in the differential diagnosis.

Update of Systemic Vasculitides Nomenclature. International Chapel Hill Consensus Conference, 2012

Update of Systemic Vasculitides Nomenclature. International Chapel Hill Consensus Conference, 2012