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Clinical Trials in Vasculitis.

ABSTRACT: The systemic vasculitides include a heterogenous group of diseases characterised by inflammation of blood vessels. Evidence for treatment in this group of patients is limited due to rarity of the diseases, incomplete understanding of the pathogenesis and lack of appropriate biomarkers. In the last 20 years, international collaboration and networking led to clinical trials in a select subgroup of patients with systemic vasculitis. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is the most studied subgroup. This article discusses the treatment options of AAV in light of evidence from clinical trials. Treatment of AAV, which includes an induction and a maintenance phase, is dependent on the severity of the disease. Oral or intravenous cyclophosphamide and high-dose glucocorticoids are considered to be standard of care for induction of remission in AAV patients with generalised disease. Latest evidence supports rituximab as an alternative to cyclophosphamide especially in relapsing patients and is increasingly being used in patients who cannot have cyclophosphamide. Plasma exchange and intravenous immunoglobulins (IVIGs) are used as adjunctive therapies for induction. Azathioprine or methotrexate (in non-renal patients) is considered to be the choice for remission maintenance, whilst mycophenolate mofetil is reserved for patients who cannot tolerate either of them. Rituximab is also being increasingly used for remission maintenance in relapsing patients. Even though an enormous progress has been made in the outlook of patients with AAV, a number of questions remain unanswered with regard to the optimal treatment strategy.

SUBMITTER: Gopaluni S

PROVIDER: S-EPMC5566121 | biostudies-other | 2016

REPOSITORIES: biostudies-other

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Clinical Trials in Vasculitis.

Gopaluni Seerapani S Jayne David D Jayne David D

Current treatment options in rheumatology 20160412 2

The systemic vasculitides include a heterogenous group of diseases characterised by inflammation of blood vessels. Evidence for treatment in this group of patients is limited due to rarity of the diseases, incomplete understanding of the pathogenesis and lack of appropriate biomarkers. In the last 20 years, international collaboration and networking led to clinical trials in a select subgroup of patients with systemic vasculitis. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis ...[more]

PMID: 28936387

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Project description:BackgroundThe vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development.ResultsA total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet's disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small- and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment (p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported.ConclusionsReuma.pt/vasculitis is a bespoke web-based registry adapted for routine care of patients with this form of rare and complex diseases, allowing an efficient data-repository at a national level with the potential to link with other international databases. It facilitates research, trials recruitment, service planning and benchmarking.

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Clinical Trials in Vasculitis.

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Clinical Trials in Vasculitis.

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