Abstract:
OBJECTIVE:To develop widely accepted international severity score for children and adult patients with familial Mediterranean fever (FMF) that can be easily applied, in research and clinical practice. METHODS:Candidate severity criteria were suggested by several FMF expert physicians. After three rounds of Delphi survey, the candidate criteria, defined by the survey, were discussed by experts in a consensus meeting. Each expert brought data of clinical manifestations, laboratory findings and physician's global assessments (PGAs) of minimum 20 patients from their centres. We used the PGAs for disease severity as a gold standard. Logistic regression analysis was used to evaluate the predicting value of each item, and receiver operating characteristic curve analysis was performed to demonstrate the success of the criteria set. RESULTS:A total of 281 patients consist of 162 children and 119 adults with FMF were enrolled and available for validity analysis: Nine domains were included in the final core set of variables for the evaluation of disease severity in FMF. The International Severity Score for FMF (ISSF) may reach a maximum of 10 if all items are maximally scored. The threshold values to determine: severe disease ≥6, intermediate disease 3-5, mild disease ≤2. Area under the curve was calculated as 0.825 for this set in the whole group. CONCLUSIONS:The initial validity of ISSF both in children and adult with FMF was demonstrated. We anticipate that it will provide a robust tool to objectively define disease severity for clinical trials, future research as well as for therapeutic decisions in managing patients with FMF.
journal_name
Ann Rheum Disjournal_title
Annals of the rheumatic diseasesauthors
Demirkaya E,Acikel C,Hashkes P,Gattorno M,Gul A,Ozdogan H,Turker T,Karadag O,Livneh A,Ben-Chetrit E,Ozen S,FMF Arthritis Vasculitis and Orphan disease Research in pediatric rheumatology (FAVOR).doi
10.1136/annrheumdis-2015-208671subject
Has Abstractpub_date
2016-06-01 00:00:00pages
1051-6issue
6eissn
0003-4967issn
1468-2060pii
annrheumdis-2015-208671journal_volume
75pub_type
杂志文章abstract::A putative retravirus was isolated from a cell culture derived by fusion of mink and dog cell lines with cultured placental cells from a patient with systemic lupus erythematosus. The virus was detected by reverse transcriptase assay of supernatant medium after prolonged subculturing. Its characterisation, including s...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.37.3.234
更新日期:1978-06-01 00:00:00
abstract:OBJECTIVES:To evaluate the rate of tuberculosis (TB) in biologic users for rheumatic diseases in South Africa, the effectiveness of our latent TB infection (LTBI) programme, risk factors and outcome. METHODS:TB cases were collected from the South African Biologics Registry (SABIO), rheumatologists and pharmaceutical c...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2019-216128
更新日期:2020-02-01 00:00:00
abstract:OBJECTIVES:The aim of this study was to assess the value of power Doppler ultrasound (PDUS) in combination with routine management in a cohort of patients with very early inflammatory arthritis (IA). METHODS:50 patients with < or =12 weeks of inflammatory symptoms with or without signs had clinical, laboratory and ima...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2008.106658
更新日期:2010-02-01 00:00:00
abstract::The effect of intranasal salmon calcitonin on pain, erosion progression, and bone loss in 40 women with rheumatoid arthritis was investigated. The study design was double blind, placebo controlled for the first four months and open for the next 36 months, allowing for cross over to active drug treatment or to the cont...
journal_title:Annals of the rheumatic diseases
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1136/ard.51.6.761
更新日期:1992-06-01 00:00:00
abstract::Feverfew, reputed by folklore to be effective in arthritis, has in vitro properties that could be beneficial in the control of inflammatory disease. Forty one female patients with symptomatic rheumatoid arthritis received either dried chopped feverfew (70-86 mg) or placebo capsules once daily for six weeks. Allocation...
journal_title:Annals of the rheumatic diseases
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1136/ard.48.7.547
更新日期:1989-07-01 00:00:00
abstract:OBJECTIVES:To develop recommendations for monitoring patients with systemic lupus erythematosus (SLE) in clinical practice and observational studies and to develop a standardised core set of variables to monitor SLE. METHODS:We followed the European League Against Rheumatism (EULAR) standardised procedures for guideli...
journal_title:Annals of the rheumatic diseases
pub_type: 共识发展会议,杂志文章,实务指引
doi:10.1136/ard.2009.117200
更新日期:2010-07-01 00:00:00
abstract::The majority of paired sera and synovial fluids from 21 patients with rheumatoid arthritis produced a rapid chemiluminescent response when incubated with human neutrophils. Synovial fluid gave considerably higher responses than the paired serum specimen. In contrast little or no response was found with paired sera and...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.42.2.158
更新日期:1983-04-01 00:00:00
abstract:OBJECTIVES:In juvenile idiopathic arthritis (JIA), the efficacy of very early disease-modifying drug therapy, synthetic or biological, is not well known. Three alternative strategies were compared for treating recent-onset polyarticular JIA. METHODS:In a 54-week multicentre open-label clinical trial, 60 disease-modify...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1136/ard.2010.143347
更新日期:2011-09-01 00:00:00
abstract::Two British secondary schools (one a specialist music school) were surveyed to assess the prevalence of upper limb pain among specialist music students compared with students in a regular school setting. Female students tended to report pain more often than male students, but for both significantly higher prevalence w...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.48.12.998
更新日期:1989-12-01 00:00:00
abstract:QUESTION:Which is the best strategy to achieve (drug-free) inactive disease in juvenile idiopathic arthritis (JIA)? METHODS:In a randomised, single-blinded, study in disease-modifying anti-rheumatic drug (DMARD)-naive patients with JIA, three treatment-strategies were compared: (1) sequential DMARD-monotherapy (sulfas...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1136/annrheumdis-2018-213902
更新日期:2019-01-01 00:00:00
abstract:OBJECTIVE:To compare the clinical, radiological, and biological profile of patients presenting late onset spondylarthropathy (LOSPA) with patients with early onset spondylarthropathy (EOSPA). METHODS:During the period April 1987 to April 1995 a retrospective chart review of inpatients and outpatients identified eight ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.56.3.176
更新日期:1997-03-01 00:00:00
abstract:OBJECTIVES:Classification criteria are biased towards classifying long-standing disease. We compared the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)-2019, Systemic Lupus International Collaborating Clinics (SLICC)-2012 and ACR-1997 criteria in an early (median 48 months) systemic l...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2019-216155
更新日期:2020-02-01 00:00:00
abstract::A patient with rheumatoid arthritis (RA), vasculitis, and aortic valve incompetence of the histologically nonspecific type is described. Valve replacement was undertaken, and an excellent haemodynamic result was achieved. Both arthritis and vasculitis subsequently remitted. Valve replacement can be successfully perfor...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.38.5.483
更新日期:1979-10-01 00:00:00
abstract:BACKGROUND:The CNS manifestations of the antiphospholipid syndrome (APS) can mimic multiple sclerosis both clinically and radiologically. OBJECTIVE:To compare evoked potential studies in APS patients and patients with multiple sclerosis with similar neurological disability. METHODS:30 APS patients with CNS manifestat...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2005.040352
更新日期:2006-04-01 00:00:00
abstract::The case is described of a 57 year old woman with polyarthritis fulfilling the 1987 revised criteria of the American Rheumatism Association for rheumatoid arthritis, accompanied by clinical carrier state infection of HTLV-I. Anti-HTLV-I IgM antibodies were detected by western blot analysis in her synovial fluid and se...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.49.9.718
更新日期:1990-09-01 00:00:00
abstract::Thirteen patients were studied after jejunoileal bypass (JIB) surgery. Seven developed arthritis and six did not. Circulating immune complexes containing IgG and IgA were detected in the sera of patients with and without arthritis. IgA complexes were shown to contain secretory component, a protein predominantly associ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.44.4.239
更新日期:1985-04-01 00:00:00
abstract:OBJECTIVE:To estimate the effects of biological drugs on the risk of demyelinating events in rheumatoid arthritis (RA). METHODS:Case-control analyses nested in an administrative database cohort. RESULTS:Initially the risk of demyelinating events appeared to be increased after exposure to anakinra and decreased after ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2009.111500
更新日期:2010-09-01 00:00:00
abstract:INTRODUCTION:The anticyclic citrullinated peptide 2 (anti-CCP2) assay is a generic test for antibodies to citrullinated proteins, among which there is a subset of about 50% with antibodies to citrullinated enolase peptide 1 (CEP-1). The anti-CEP-1 positive subset is strongly associated with the HLA-DRB1 shared epitope ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,多中心研究
doi:10.1136/ard.2010.138909
更新日期:2011-06-01 00:00:00
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journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.43.1.102
更新日期:1984-02-01 00:00:00
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journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1136/ard.2005.040196
更新日期:2006-04-01 00:00:00
abstract:BACKGROUND:A prolonged symptom or disease duration at treatment initiation is associated with unfavourable outcomes in rheumatoid arthritis (RA). It is unknown whether this relation is linear, referring to a common 'the-earlier-the-better principle', or whether a transient time frame in which the disease is more suscep...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2014-206047
更新日期:2015-05-01 00:00:00
abstract:OBJECTIVES:To compare the efficacy and gastroduodenal safety of a fixed-dose combination of diclofenac sodium 50 mg and misoprostol 200 micrograms twice daily with those of piroxicam 10 mg twice daily and naproxen 375 mg twice daily in patients with osteoarthritis. METHODS:A 4 week, randomised, double-blind, parallel-...
journal_title:Annals of the rheumatic diseases
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1136/ard.52.12.881
更新日期:1993-12-01 00:00:00
abstract:OBJECTIVE:To study the effect of meloxicam (MXC) and diclofenac (DCF) on the recruitment of leucocytes during acute experimental arthritis. METHODS:Rabbits with antigen-induced arthritis were treated with MXC, DCF, or not treated. After 48 hours, synovial fluid (SF) leucocyte influx and prostaglandin E(2) (PGE(2)) lev...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.61.11.1027
更新日期:2002-11-01 00:00:00
abstract:OBJECTIVE:To investigate the sympathetic and parasympathetic cardiovascular function in primary Sjögren's syndrome (SS) and to investigate the possible relation with ocular dryness. METHODS:41 (40 women) patients with primary SS, mean age 50 years (range 20-80) with a mean disease duration of eight years (range 1-30),...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.58.12.746
更新日期:1999-12-01 00:00:00
abstract:OBJECTIVE:To test the hypothesis that pustulosis palmaris et plantaris and psoriatic arthritis (PsA) are two distinct diseases, and that the associated dermatoses are therefore also distinct diseases. METHODS:We prospectively performed clinical, radiological, biological, and bone scan investigations in 23 outpatients ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.55.3.177
更新日期:1996-03-01 00:00:00
abstract:BACKGROUND:Increasing age at onset has been associated with worse outcome in rheumatoid arthritis, although there are few data from unselected inception cohorts. HYPOTHESIS:Increasing age is associated with a higher risk of erosions at presentation, and this increase is not explained by age-related disease confounders...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2006.055657
更新日期:2007-03-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease (AID) worldwide. The disease is caused by mutations in the MEFV gene encoding the inflammasome sensor Pyrin. Clinical diagnosis of FMF is complicated by overlap in symptoms with other diseases, and interpre...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2019-216701
更新日期:2020-07-01 00:00:00
abstract:OBJECTIVE:To define the role of ultrasound (US) for the assessment of patients with rheumatoid arthritis (RA) in clinical remission, including joint and tendon evaluation. METHODS:A multicentre longitudinal study has been promoted by the US Study Group of the Italian Society for Rheumatology. 25 Italian centres partic...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,多中心研究
doi:10.1136/annrheumdis-2018-213217
更新日期:2018-09-01 00:00:00
abstract:INTRODUCTION:Psoriasis susceptibility locus 4 (PSORS4) is a susceptibility locus for psoriasis vulgaris (PsV), a common inflammatory, hyperproliferative skin disorder. Recently, a deletion of 2 late cornified envelope (LCE) genes within epidermal differentiation complex on chromosome 1 was shown to be enriched in 1426 ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2009.108951
更新日期:2010-05-01 00:00:00
abstract::Mild iron deficiency induced by treatment with the iron chelator desferrioxamine reduced the incidence and severity of joint inflammation associated with adjuvant disease in rats but did not alter the local primary inflammatory response or the systemic sequelae. Both rats treated with desferrioxamine and control rats ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.46.4.327
更新日期:1987-04-01 00:00:00