Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies

Watts, Richard A.; Lane, Suzanne; Hanslik, Thomas; Hauser, Thomas; Hellmich, Bernhard; Koldingsnes, Wenche; Mahr, Alfred; Segelmark, Mårten; Cohen-Tervaert, Jan Willem; Scott, David G. I.

doi:10.1136/ard.2006.054593

Cited by 1,164 publications

(912 citation statements)

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“…26 A reliable classification system of vasculitis has recently been devised which can help in differentiation of PAN from small vessel vasculitis. 28 The presence of Anti neutrophilic cytoplasmic antibodies (ANCA), glomerulonephritis and/or histological involvement of arterioles, capillaries, and/or venules goes in favor of small vessel vasculitis and against the diagnosis of PAN. Both indirect immunofluorescence (IIF) and ELISA methods should be employed for doing the ANCA.…”

Section: Diagnosismentioning

confidence: 99%

Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

Sharma

2013

Journal of Clinical and Experimental Hepatology

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Two hepatotropic viruses have been shown to have causal relationship with systemic vasculitis-hepatitis B with classical polyarteritis nodosa and hepatitis C with cryoglobulinemic vasculitis. The present paper provides an updated overview on the clinical presentations and management of these vasculitides.HBV associated PAN patients have higher weight loss, peripheral neuropathy, mononeuritis multiplex, abdominal pain, gastrointestinal manifestations requiring surgery, cardiomyopathy, orchitis, hypertension, and/or elevated transaminase levels. Microaneurysms are also more common in mesenteric artery. Skin manifestations, however are less common. These patients also have a severe disease as suggested by higher five factor score and higher BVAS. Though relapses are less common, mortality is higher in patients with HBV PAN as compared to non HBV PAN. Plasmapheresis has a role in treatment in clearing off immune complexes.The common clinical manifestations of HCV associated cryoglobulinemic vasculitis are skin lesions, peripheral neuropathy, glomerulonephritis, arthritis, and sicca symptoms. Though combination therapy comprising of pegylated interferon a and ribavirin is the first line of management, immunotherapy is needed for severe or life threatening manifestations. Recent randomized trials have shown the efficacy of rituximab in such situations. ( J CLIN EXP HEPATOL 2013;3:204-212)

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Section: Diagnosismentioning

confidence: 99%

Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

Sharma

2013

Journal of Clinical and Experimental Hepatology

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“…According to the definitive criteria of the Chapel Hill Consensus Conference (CHCC) 4 and the consensus algorithm proposed by the European Medicines Agency (EMA algorithm), 14 we recruited 39 Japanese patients who were diagnosed with definitive AAV (18 men and 21 women, 61 AE 13 years [range 21-88 years]). Among them, 24 (61.5%), 12 (30.8%) and three (7.7%) patients were classified as having MPA, GPA and EGPA, respectively.…”

Section: Patients and Study Designmentioning

confidence: 99%

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

et al. 2017

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Aim: To elucidate the characteristics of patients with hypertrophic pachymeningitis (HP) in a population with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods:We retrospectively investigated the clinical records of 39 patients who were diagnosed with AAV. To determine the characteristics of HP in AAV, the epidemiological and clinical data from patients with HP were statistically compared with those from patients without HP.Results: Of 39 patients with AAV, seven (17.9%) had associated HP. All patients with HP were classified as having granulomatosis with polyangiitis (GPA), whereas only five of 32 patients without HP were diagnosed as having GPA (P < 0.0001). The frequencies of myeloperoxidase (MPO)-ANCA and proteinase 3-ANCA positivity in patients with HP were equivalent, while MPO-ANCA positivity was obviously dominant in patients without HP. HP occurred as the initial clinical episode of AAV in three patients (7.7% of all AAV). Frequent significant characteristics of patients with HP were headache, cranial neuropathy and paranasal involvement (P < 0.05), and histopathological findings from paranasal involvement were useful for the diagnosis of GPA in some patients with HP. Combination therapy of corticosteroid and an immunosuppressant, such as methotrexate, cyclophosphamide or rituximab, was effective for achieving remission and improving radiographic findings of HP.Conclusion: AAV is a common cause of HP; epidemiological features of AAV patients with HP are different from those of patients without HP. Additionally, HP impacts diagnosis because it may be an initial clinical sign of AAV.

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“…We performed a nationwide retrospective multicenter study in patients attending departments of internal medicine, pulmonology, and rheumatology. Patients who received at least 1 course of IVIG and who met the American College of Rheumatology 1990 criteria (13) and/or the European Medicines Agency (EMA) algorithm (14) and/or the definitions from the 2012 Chapel Hill Consensus Conference (15) for GPA, MPA, or eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) were included. Among all of the patients who were included in this study, 22 patients were part of a prospective open-label trial previously conducted by our group (the IVIGs for Relapses of Systemic Vasculitides Associated with ANCA [IGANCA] trial) (10), and we provide additional followup data herein.…”

Section: Methodsmentioning

confidence: 99%

Intravenous Immunoglobulin as an Immunomodulating Agent in Antineutrophil Cytoplasmic Antibody–Associated Vasculitides: A French Nationwide Study of Ninety‐Two Patients

Crickx

Machelart

Lazaro

et al. 2016

Arthritis & Rheumatology

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Objective. Intravenous immunoglobulin (IVIG) represents a therapeutic alternative in antineutrophil cytoplasmic antibody-associated vasculitides (AAV), but its efficacy has been evaluated in only 2 small prospective trials. The aim of this study was to evaluate the efficacy and safety of IVIG in patients with AAV.Methods. We conducted a nationwide retrospective study of patients who received IVIG as immunomodulatory therapy for AAV.Results. A total of 92 patients (mean age 51 years) presenting with either granulomatosis with polyangiitis (Wegener's) (68%), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (22%), or microscopic polyangiitis (10%) received at least 1 course of IVIG. Antineutrophil cytoplasmic antibodies were present in 72% during the flare that required IVIG, as determined by immunofluorescence assay. IVIG was initiated because of relapsing disease in 83% of cases. IVIG was given for a median of 6 months (range 1-156 months) and in combination with corticosteroids in 21% of the patients or with other immunosuppressive agents in 77%. Efficacy of IVIG was assessed in the entire population and in a subset of 34 patients with unmodified background therapy. Remission rates at 6 months were 56% in the entire pop-

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Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies

Abstract: 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.

Cited by 1,164 publications

References 16 publications

Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

Intravenous Immunoglobulin as an Immunomodulating Agent in Antineutrophil Cytoplasmic Antibody–Associated Vasculitides: A French Nationwide Study of Ninety‐Two Patients

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