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Ozen S.,Hacettepe University | Demirkaya E.,Gulhane Military Medical Academy | Erer B.,Istanbul University | Livneh A.,Tel Aviv University | And 11 more authors.
Annals of the Rheumatic Diseases | Year: 2016

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.

Magni-Manzoni S.,Pediatria Ospedaliera | Malattia C.,Pediatria II Reumatologia | Lanni S.,Pediatria Ospedaliera | Ravelli A.,Pediatria II Reumatologia
Nature Reviews Rheumatology | Year: 2012

Imaging assessments of the joints of children with juvenile idiopathic arthritis (JIA) are challenging, owing to the unique features of the growing skeleton. Traditionally, imaging studies in childhood arthritis have been based on conventional radiography. However, in the past few years, interest in the use of MRI and ultrasonography has increased. As a result, imaging has become a main area of clinical and research investigation in paediatric rheumatology. The chief advance in the field of conventional radiography has been the development and validation of paediatric scoring systems for the assessment of radiographic progression. Several studies have shown that MRI provides a precise quantification of synovitis in children with JIA. Furthermore, a high frequency of bone marrow oedema and bone erosions has been found early in the disease course. Ultrasonography has been proven to be superior to clinical examination in detecting synovitis, tenosynovitis and enthesitis. A high frequency of subclinical synovitis has been demonstrated in patients with JIA who have clinically inactive disease using both MRI and ultrasonography. However, more information from healthy children is needed to enable differentiation of the bone and cartilage abnormalities that reflect damage from those that are part of normal development using MRI or ultrasonography. This Review provides a summary of the current information on conventional radiography, ultrasonography and MRI in JIA and highlights the advantages and limitations of each imaging modality. © 2012 Macmillan Publishers Limited.

Prinold J.A.I.,University of Sheffield | Mazza C.,University of Sheffield | Di Marco R.,University of Sheffield | Di Marco R.,University of Rome La Sapienza | And 10 more authors.
Annals of Biomedical Engineering | Year: 2015

Juvenile idiopathic arthritis (JIA) is the leading cause of childhood disability from a musculoskeletal disorder. It generally affects large joints such as the knee and the ankle, often causing structural damage. Different factors contribute to the damage onset, including altered joint loading and other mechanical factors, associated with pain and inflammation. The prediction of patients’ joint loading can hence be a valuable tool in understanding the disease mechanisms involved in structural damage progression. A number of lower-limb musculoskeletal models have been proposed to analyse the hip and knee joints, but juvenile models of the foot are still lacking. This paper presents a modelling pipeline that allows the creation of juvenile patient-specific models starting from lower limb kinematics and foot and ankle MRI data. This pipeline has been applied to data from three children with JIA and the importance of patient-specific parameters and modelling assumptions has been tested in a sensitivity analysis focused on the variation of the joint reaction forces. This analysis highlighted the criticality of patient-specific definition of the ankle joint axes and location of the Achilles tendon insertions. Patient-specific detection of the Tibialis Anterior, Tibialis Posterior, and Peroneus Longus origins and insertions were also shown to be important. © 2015 The Author(s)

Miettunen P.M.,University of Calgary | Pistorio A.,Servizio di Epidemiologia e Biostatistica | Palmisani E.,Pediatria II Reumatologia | Ravelli A.,Pediatria II Reumatologia | And 17 more authors.
Annals of the Rheumatic Diseases | Year: 2013

Objectives: To evaluate therapeutic approaches and response to therapy in juvenile systemic lupus erythematosus (SLE) with renal involvement in a large prospective international cohort from four geographic areas. Methods New onset and flared patients with active renal disease (proteinuria ≥0.5 g/24 h) were enrolled in 2001.2004. Therapeutic approaches and disease activity parameters were analysed at baseline, 6, 12 and 24 months. Response was assessed by the PRINTO/ACR criteria. Results: 218/557 (79.8% female subjects, 117 new onset and 101 flared) patients with active renal disease were identified; 66 patients were lost to follow-up and 11 died. Mean age at disease onset for new onset group was higher than for flared group (13.1 vs 10.2 years, p<0.0001). At baseline, both groups had similar renal activity with similar median doses of corticosteroids ( mg/kg/day). Cyclophosphamide (43.1%) and azathioprine (22%) were the most common immunosuppressive drugs. At baseline, South American patients received higher doses of corticosteroids than in other areas in new onset (median 1.16 vs 0.8.1 mg/kg/day) while cyclophosphamide use was similar in all four regions in the new onset group. There were no differences regarding the use of azathioprine or mycophenolate mofetil worldwide. PRINTO 70 response was reached in a greater percentage of new onset versus flared patients (74.8% vs 53.3%; p=0.005) at 6 months while at 24 months ACR 90 was reached by 69.9% and 56.1%, respectively. Conclusions: New onset and flared juvenile SLE improved similarly over 24 months with minimal differences in therapeutic approaches worldwide.

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