Abstract:
:Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease, but many rheumatologists are not well acquainted with its management. The objective of this report is to produce evidence-based recommendations to guide rheumatologists and other health professionals in the treatment and follow-up of patients with FMF. A multidisciplinary panel, including rheumatologists, internists, paediatricians, a nurse, a methodologist and a patient representative, was assembled. Panellists came from the Eastern Mediterranean area, Europe and North America. A preliminary systematic literature search on the pharmacological treatment of FMF was performed following which the expert group convened to define aims, scope and users of the guidelines and established the need for additional reviews on controversial topics. In a second meeting, recommendations were discussed and refined in light of available evidence. Finally, agreement with the recommendations was obtained from a larger group of experts through a Delphi survey. The level of evidence (LoE) and grade of recommendation (GR) were then incorporated. The final document comprises 18 recommendations, each presented with its degree of agreement (0-10), LoE, GR and rationale. The degree of agreement was greater than 7/10 in all instances. The more controversial statements were those related to follow-up and dose change, for which supporting evidence is limited. A set of widely accepted recommendations for the treatment and monitoring of FMF is presented, supported by the best available evidence and expert opinion. It is believed that these recommendations will be useful in guiding physicians in the care of patients with FMF.
journal_name
Ann Rheum Disjournal_title
Annals of the rheumatic diseasesauthors
Ozen S,Demirkaya E,Erer B,Livneh A,Ben-Chetrit E,Giancane G,Ozdogan H,Abu I,Gattorno M,Hawkins PN,Yuce S,Kallinich T,Bilginer Y,Kastner D,Carmona Ldoi
10.1136/annrheumdis-2015-208690subject
Has Abstractpub_date
2016-04-01 00:00:00pages
644-51issue
4eissn
0003-4967issn
1468-2060pii
annrheumdis-2015-208690journal_volume
75pub_type
共识发展会议,杂志文章,实务指引abstract::Three patients are presented who sustained bilateral rupture of the Achilles tendon while on systemic steroid therapy for chest disease; a fourth patient with polymyalgia rheumatica on steroids is also presented. This is further evidence that tendon rupture can be a direct complication of steroid treatment. The Englis...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.42.6.652
更新日期:1983-12-01 00:00:00
abstract:OBJECTIVE:FoxO3a is a transcriptional factor implicated in cell cycle regulation and apoptosis. Since rheumatoid arthritis (RA) is associated with apoptosis defects, the expression level, regulation and phosphorylation status of FoxO3a was investigated in blood and synovium from patients with RA. METHODS:In microarray...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2009.109991
更新日期:2010-04-01 00:00:00
abstract::A 49 year old white woman with systemic lupus erythematosus and bronchiolitis obliterans was treated with prednisone (1 mg/kg daily), which led to a transitory improvement in pulmonary status. Cyclophosphamide was then added--4 mg/kg daily intravenously for five days, then 2 mg/kg daily orally--and this was followed b...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.50.12.956
更新日期:1991-12-01 00:00:00
abstract:BACKGROUND:Entheseal fibrocartilage (EF) derangement is hypothesised to be pivotal to the pathogenesis of spondyloarthritis. Ultrasound is useful for visualisation of the enthesis but its role in EF visualisation is uncertain. This work aimed to demonstrate face and content validity of ultrasound for EF visualisation b...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2009.127175
更新日期:2010-12-01 00:00:00
abstract:OBJECTIVE:Antiphospholipid antibody (aPL) specificity for aPL-related events was evaluated in systemic lupus erythematosus (SLE). METHODS:A study was carried out on 105 patients affected with SLE comparing the prevalence of lupus anticoagulant (LA) and IgG and IgM anticardiolipin antibodies (aCL) between patients with...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.53.2.140
更新日期:1994-02-01 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:OBJECTIVE:Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with unknown aetiology. Epstein-Barr virus (EBV) is an environmental factor associated with SLE. EBV maintains latency in B cells with frequent reactivation measured by antibodies against viral capsid antigen (VCA) and early antigen (EA). In ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2019-215361
更新日期:2019-09-01 00:00:00
abstract:BACKGROUND:In patients with systemic sclerosis (scleroderma, SSc), impaired hand function greatly contributes to disability and reduced quality of life, and is insufficiently relieved by currently available therapies. Adipose tissue-derived stromal vascular fraction (SVF) is increasingly recognised as an easily accessi...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,随机对照试验
doi:10.1136/annrheumdis-2014-205681
更新日期:2015-12-01 00:00:00
abstract:BACKGROUND:Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterised by aberrant B cell hyperactivation, whose mechanism is partially understood. METHODS:We performed whole transcriptome sequencing of B cells from three pSS patients and three matched healthy controls (HC). Differentially expressi...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2019-216428
更新日期:2020-04-01 00:00:00
abstract::Thermography before and after joint irrigation at arthroscopy showed no significant improvement of inflammation in 14 patients. Subjectively, those with chondrocalcinosis did best. ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.37.1.78
更新日期:1978-02-01 00:00:00
abstract:OBJECTIVE:Regulatory T cells (Tregs) with the plasticity of producing proinflammatory cytokine IL-17 have been demonstrated under normal and pathogenic conditions. However, it remains unclear whether IL-17-producing Tregs lose their suppressive functions because of their plasticity toward Th17 in autoimmunity. The aim ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2013-204228
更新日期:2015-06-01 00:00:00
abstract:OBJECTIVES:Infectious agents like parvovirus have been implicated as exogenous factors that could trigger onset of systemic lupus erythematosus (SLE). A number of case reports describing a SLE-like presentation of acute human parvovirus B19 infection have been published, but no systematic investigation of the actual se...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.59.1.64
更新日期:2000-01-01 00:00:00
abstract::Whole gut lavage fluid is a useful source of material for the study of intestinal immunity and inflammation in humans. Systemic and mucosal antibodies to Klebsiella pneumoniae were measured by enzyme linked immunosorbent assay (ELISA) in serum samples and whole gut lavage fluid from 14 patients with ankylosing spondyl...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.51.12.1296
更新日期:1992-12-01 00:00:00
abstract:OBJECTIVE:To assess the effects of acute stress on inflammatory, haemostatic, rheological, and haemodynamic activity in patients with rheumatoid arthritis (RA) in comparison with patients with osteoarthritis (OA). METHODS:21 patients with RA and 10 with OA underwent a brief mental stress task while standing. Inflammat...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,多中心研究
doi:10.1136/ard.2004.032151
更新日期:2005-09-01 00:00:00
abstract:OBJECTIVES:To compare the effect of a single infusion of zoledronic acid (ZA) with placebo on knee pain and bone marrow lesions (BMLs). METHODS:Adults aged 50-80 years (n=59) with clinical knee osteoarthritis and knee BMLs were randomised to receive either ZA (5 mg/100 ml) or placebo. BMLs were determined using proton...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,随机对照试验
doi:10.1136/annrheumdis-2011-200970
更新日期:2012-08-01 00:00:00
abstract:OBJECTIVE:To identify differentially expressed genes in peripheral blood mononuclear cells (PBMCs) from patients with ankylosing spondylitis (AS) compared with healthy individuals. METHODS:RNA was extracted from PBMCs collected from 18 patients with active disease and 18 gender-matched and age-matched controls. Expres...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2009.111690
更新日期:2010-09-01 00:00:00
abstract:BACKGROUND:Chondromalacia patellae is a potentially disabling disorder characterised by features of patellar cartilage degradation. OBJECTIVE:To evaluate markers of cartilage and bone turnover in patients with chondromalacia patellae. METHODS:18 patients with chondromalacia patellae were studied. Serum cartilage olig...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.61.11.981
更新日期:2002-11-01 00:00:00
abstract:OBJECTIVE:To study maternal and fetal outcome of pregnancy in patients with lupus who were exposed to hydroxychloroquine (HCQ). METHODS:The case records of women (n = 33) exposed to HCQ during their pregnancies (n = 36) and of 53 control patients from a single lupus pregnancy centre were reviewed to determine lupus ac...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.55.7.486
更新日期:1996-07-01 00:00:00
abstract::Of 60 patients with rheumatoid arthritis treated with levamisole 35% showed a persistent decrease in neutrophil counts from pre-treatment levels and 6 (10%) develped severe neutropenia of less than 1.0 X 10(9)/1. One of these neutropenic patients recoverd without stopping therapy and the other 5 patients recovered rap...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.37.4.366
更新日期:1978-08-01 00:00:00
abstract::Heart disease is a rare but important complication of polymyositis. Diagnosis of myocardial disease is usually based on non-specific clinical, electrocardiographic, and echocardiographic data. This paper reports a case of polymyositis with myocardial disease diagnosed by myocardial imaging with radiolabelled antibody ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.52.3.235
更新日期:1993-03-01 00:00:00
abstract::Psoriatic arthritis (PsA) is characterised by several unique clinical features that differentiate it from rheumatoid arthritis (RA). Attempts to identify immunopathological mechanisms, some shared with psoriasis, that underlie these differences from RA have been most challenging. Recent research studies, however, high...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,评审
doi:10.1136/ard.2004.031740
更新日期:2005-03-01 00:00:00
abstract::Serum C2 activity was measured in 135 individuals drawn from a panel of 418 tissue-typed blood donors. The study group included all donors with HLA antigens A10 and B18. Heterozygote C2 deficiency (C2Dhet) was defined by reference to the range of C2 activity in previously studied obligate heterozygotes. Five donors we...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.41.1.93
更新日期:1982-02-01 00:00:00
abstract:BACKGROUND:Tumour necrosis factor alpha (TNFalpha) blockade using infliximab, a chimeric anti-TNFalpha antibody, is an effective treatment for both psoriasis and psoriatic arthritis (PsA). OBJECTIVE:To analyse the early effects of infliximab treatment on serial skin and synovial tissue biopsy samples. METHODS:Twelve ...
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.2003.018085
更新日期:2004-07-01 00:00:00
abstract:OBJECTIVE:To assess the safety and effectiveness of imatinib mesylate in the treatment of diffuse cutaneous systemic sclerosis (dcSSc). METHODS:In this phase IIa, open-label, single-arm clinical trial, 30 patients with dcSSc were treated with imatinib 400 mg daily. Patients were monitored monthly for safety assessment...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2010.143974
更新日期:2011-06-01 00:00:00
abstract::The regression of small rheumatoid nodules was noted in four patients after starting sulphasalazine therapy. This coincided with an improvement in synovitis and also falls in erythrocyte sedimentation rate (ESR) and C reactive protein (CRP). The relation between the nodule regression and the sulphasalazine therapy is ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.46.3.244
更新日期:1987-03-01 00:00:00
abstract::The pattern of joint involvement on conventional radiographs and the four hour gammacamera image of 99Tc HMDP bone scans were studied in 33 patients with generalised nodal osteoarthritis. Both techniques showed the predominant involvement of the distal interphalangeal, scaphotrapezial, and first carpometacarpal joints...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.45.8.617
更新日期:1986-08-01 00:00:00
abstract:OBJECTIVE:To evaluate the efficacy and safety of monotherapy with adalimumab in patients with RA for whom previous DMARD treatment has failed. METHODS:In a 26 week, double blind, placebo controlled, phase III trial, 544 patients with RA were randomised to monotherapy with adalimumab 20 mg every other week, 20 mg weekl...
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.2003.013052
更新日期:2004-05-01 00:00:00
abstract:OBJECTIVES:Different prevalences of generalised osteoarthritis (GOA) in patients with knee and hip OA have been reported. The aim of this investigation was to evaluate radiographic and clinical patterns of disease in a hospital based population of patient subgroups with advanced hip and knee OA and to compare the preva...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.57.12.717
更新日期:1998-12-01 00:00:00
abstract::The serological diagnosis of juvenile rheumatoid arthritis (JRA) is difficult, with only 7-10% of patients 19S IgM rheumatoid factor positive. About 60-70% of patients are positive for hidden 19S IgM rheumatoid factor, but this test requires serum separation and is not available in most laboratories. Antiperinuclear f...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.51.3.350
更新日期:1992-03-01 00:00:00
abstract::A patient with Portuguese familial amyloid polyneuropathy who developed haemarthroses secondary to pathological fractures is described. Amyloid material was demonstrated on bone biopsy and confirmed immunohistochemically to be transthyretin (prealbumin). Although amyloid deposits in bone have been described in other t...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.50.11.820
更新日期:1991-11-01 00:00:00