In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. ...The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate. The evidence presented was discussed and summarised by the experts in the course of a consensus-finding and voting process. Levels of evidence and grades of recommendations were derived and levels of agreement (strengths of recommendations) determined. In addition to the voting by the task force members, the relevance of the recommendations was assessed by an online voting survey among members of EUVAS. Fifteen recommendations were developed, covering general aspects, such as attaining remission and the need for shared decision making between clinicians and patients. More specific items relate to starting immunosuppressive therapy in combination with glucocorticoids to induce remission, followed by a period of remission maintenance; for remission induction in life-threatening or organ-threatening AAV, cyclophosphamide and rituximab are considered to have similar efficacy; plasma exchange which is recommended, where licensed, in the setting of rapidly progressive renal failure or severe diffuse pulmonary haemorrhage. These recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries and drug regulatory organisations.
Despite advances in the diagnosis and treatment, the mortality rate of Takayasu arteritis (TAK) is still elevated even today. The diagnosis is often made after a long time delay and the course of the ...disease is characterized by progressive structural vascular lesions. Recently, new recommendations for the management of large vessel vasculitis were published by the European League Against Rheumatism (EULAR). For induction of remission oral glucocorticoids (GC) are administered in an initial daily dose of 40-60 mg. As experience has shown that the cumulative GC demand in TAK is high, GC-sparing treatment with moderately potent immunosuppressants, such as methotrexate, azathioprine and mycophenolate mofetil is recommended from the time of initial diagnosis. In cases of a relapsing course, tocilizumab or tumor necrosis factor (TNF)-alpha inhibitors can be used as an additive off-label treatment. If vascular stenoses persist despite supposedly sufficient inflammation control and if these stenoses are symptomatic, vascular surgery or interventional treatment procedures can be indicated. Such revascularization or even surgical procedures for the treatment of aneurysms should be performed during phases of sufficient drug control of the vasculitis. In quite a few patients progressive vascular lesions continue to develop despite clinical and laboratory analytical remission. Due to the poor correlation of clinical symptoms and acute phase markers with the progression of vascular lesions, the distinction between active and inactive diseases is often a challenge in the clinical practice. Imaging studies can then support therapeutic decisions but are not yet formally and comprehensively validated in the long-term course of TAK.
Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) and microscopic polyangiitis (MPA) are associated with the detection of antibodies against neutrophilic cytoplasmic antigens ...(ANCA) and are referred to as ANCA-associated vasculitides (AAV). In the event of the clinical suspicion of AAV the ANCA should first be determined by means of an antigen-specific immunoassay for proteinase 3‑ANCA and myeloperoxidase-ANCA, according to current consensus recommendations. The diagnosis of AAV should also be confirmed by biopsy if possible. The classification criteria for AAV are currently being revised. Diagnostic criteria do not exist. The standard induction therapy consists of rituximab or cyclophosphamide, each in combination with glucocorticoids (GC). In the absence of severe organ involvement, methotrexate can alternatively be used. Recent study data suggest that additive plasmapheresis does not improve the long-term outcome. After remission, remission-preserving treatment with azathioprine, methotrexate or rituximab should be given for at least 48 months. The risk of severe infections is markedly increased, especially during the remission induction phase but can also be reduced during treatment with rituximab by the prophylactic administration of trimethoprim-sulfamethoxazole. In view of the increased risk of infection, GC-reduced or GC-free treatment regimens are currently the focus of clinical development.
To develop European League Against Rheumatism (EULAR) recommendations for the management of small and medium vessel vasculitis.
An expert group (consisting of 10 rheumatologists, 3 nephrologists, 2 ...immunologists, 2 internists representing 8 European countries and the USA, a clinical epidemiologist and a representative from a drug regulatory agency) identified 10 topics for a systematic literature search using a modified Delphi technique. In accordance with standardised EULAR operating procedures, recommendations were derived for the management of small and medium vessel vasculitis. In the absence of evidence, recommendations were formulated on the basis of a consensus opinion.
In all, 15 recommendations were made for the management of small and medium vessel vasculitis. The strength of recommendations was restricted by low quality of evidence and by EULAR standardised operating procedures.
On the basis of evidence and expert consensus, recommendations have been made for the evaluation, investigation, treatment and monitoring of patients with small and medium vessel vasculitis for use in everyday clinical practice.
To develop European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis.
An expert group (10 rheumatologists, 3 nephrologists, 2 immunolgists, 2 internists ...representing 8 European countries and the USA, a clinical epidemiologist and a representative from a drug regulatory agency) identified 10 topics for a systematic literature search through a modified Delphi technique. In accordance with standardised EULAR operating procedures, recommendations were derived for the management of large vessel vasculitis. In the absence of evidence, recommendations were formulated on the basis of a consensus opinion.
Seven recommendations were made relating to the assessment, investigation and treatment of patients with large vessel vasculitis. The strength of recommendations was restricted by the low level of evidence and EULAR standardised operating procedures.
On the basis of evidence and expert consensus, management recommendations for large vessel vasculitis have been formulated and are commended for use in everyday clinical practice.
Diabetic cheiroarthropathy (DCA) is one of the musculoskeletal manifestations of diabetes mellitus. It is clinically diagnosed using the prayer and tabletop signs. The clinical appearance, however, ...mimics autoimmune-mediated polyarthritis of the hands and fingers. It is therefore crucial to positively identify DCA patients.OBJECTIVEDiabetic cheiroarthropathy (DCA) is one of the musculoskeletal manifestations of diabetes mellitus. It is clinically diagnosed using the prayer and tabletop signs. The clinical appearance, however, mimics autoimmune-mediated polyarthritis of the hands and fingers. It is therefore crucial to positively identify DCA patients.We used high-frequency B-mode ultrasound to investigate 14 patients with DCA and seven non-DCA diabetics with anti-cyclic citrullinated peptide antibody-positive rheumatoid arthritis (RA). We recorded the frequency of synovitis in radiocarpal, metacarpophalangeal, and proximal interphalangeal joints, the presence of tenosynovitis of the finger flexor tendons, echogenicity of the synovia and the flexor tendon sheaths, and soft tissue alterations in the digits. We compared our findings between groups to determine sonographic characteristics of DCA.METHODWe used high-frequency B-mode ultrasound to investigate 14 patients with DCA and seven non-DCA diabetics with anti-cyclic citrullinated peptide antibody-positive rheumatoid arthritis (RA). We recorded the frequency of synovitis in radiocarpal, metacarpophalangeal, and proximal interphalangeal joints, the presence of tenosynovitis of the finger flexor tendons, echogenicity of the synovia and the flexor tendon sheaths, and soft tissue alterations in the digits. We compared our findings between groups to determine sonographic characteristics of DCA.A low rate of small finger joint involvement in the presence of a high rate of finger flexor tendinopathy showed a high association with DCA in correlation (p = 0.002) and regression analysis (p < 0.001). Tendon sheaths were significantly more often hyperechoic and proliferative in DCA compared to RA (p = 0.008), and hypoechoic soft tissue alterations were almost exclusively seen in DCA patients (p = 0.003). Radiocarpal joint involvement and its echogenicity did not differ between groups.RESULTSA low rate of small finger joint involvement in the presence of a high rate of finger flexor tendinopathy showed a high association with DCA in correlation (p = 0.002) and regression analysis (p < 0.001). Tendon sheaths were significantly more often hyperechoic and proliferative in DCA compared to RA (p = 0.008), and hypoechoic soft tissue alterations were almost exclusively seen in DCA patients (p = 0.003). Radiocarpal joint involvement and its echogenicity did not differ between groups.Ultrasonography shows typical features in DCA, and is capable of discriminating DCA from non-DCA patients with RA and diabetes.CONCLUSIONUltrasonography shows typical features in DCA, and is capable of discriminating DCA from non-DCA patients with RA and diabetes.
Recent data from randomized controlled clinical trials have allowed the development of recommendations for treatment of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV).
A ...selective literature search was carried out for studies and recommendations for treatment of AAV.
In active severe AAV a combination of prednisolone and cyclophosphamide or rituximab leads to a therapeutic response in approximately 90 % of cases. Once remission is attained the administration of azathioprine or methotrexate for 2-4 years is required for maintenance of remission. Relapse occurs in more than 30 % of patients despite maintenance treatment. In cases of persistence or progression of disease activity during standard therapy, referral to an expert center should be considered. Despite improvement in the prognosis in recent years early mortality is increased, particularly due to infections.
Stage and activity adapted treatment strategies have improved the outcome of AAV in the past three decades. The elevated early mortality and the risk of relapse show the need for further improvement of current treatment protocols with respect to substance selection, dosage of glucocorticoids and immunosuppressants and the duration of therapy.
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. Aim: 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 κ = 0.886). Conclusion: 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.
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