Recommendations for early referral of individuals with suspected polymyalgia rheumatica: An initiative from the international giant cell arteritis and polymyalgia rheumatica study group

Objective To develop international consensus-based recommendations for early referral of individuals with suspected polymyalgia rheumatica (PMR). Methods A task force including 29 rheumatologists/ internists, 4 general practitioners, 4 patients and a healthcare professional emerged from the international giant cell arteritis and PMR study group. The task force supplied clinical questions, subsequently transformed into Population, Intervention, Comparator, Outcome format. A systematic literature review was conducted followed by online meetings to formulate and vote on final recommendations. Levels of evidence (LOE) (1–5 scale) and agreement (LOA) (0–10 scale) were evaluated. Results Two overarching principles and five recommendations were developed. LOE was 4–5 and LOA ranged between 8.5 and 9.7. The recommendations suggest that (1) each individual with suspected or recently diagnosed PMR should be considered for specialist evaluation, (2) before referring an individual with suspected PMR to specialist care, a thorough history and clinical examination should be performed and preferably complemented with urgent basic laboratory investigations, (3) individuals with suspected PMR with severe symptoms should be referred for specialist evaluation using rapid access strategies, (4) in individuals with suspected PMR who are referred via rapid access, the commencement of glucocorticoid therapy should be deferred until after specialist evaluation and (5) individuals diagnosed with PMR in specialist care with a good initial response to glucocorticoids and a low risk of glucocorticoid related adverse events can be managed in primary care. Conclusions These are the first international recommendations for referral of individuals with suspected PMR, which complement the European Alliance of Associations for Rheumatology/American College of Rheumatology management guidelines for established PMR

Intérêt de la détection de JAK2V617F dans l'exploration d'une polyglobulie

BORDEAUX2-BU Santé (330632101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Place de la recherche de la mutation JAK2 V617F dans le cadre d'un évènement vasculaire (étude rétrospective des pratiques au CHRU de Brest entre 2005 et 2012)

Les événements thrombotiques sont les principales complications des syndromes myéloprolifératifs (SMP). La recherche d'un SMP à cette occasion permet de mettre en place un traitement adapté et de diminuer le risque de récidive. Dans ce travail, nous avons analysé les motifs de prescription de la recherche de mutation JAK2V617F (présente dans 95% des cas de maladie de Vaquez, 50% des cas de thrombocytémie essentielle et myélofibrose primitive), au CHRU de Brest, lors d'un événement vasculaire thrombotique. Un motif de prescription a été retrouvé dans 93% des cas. Il s'agissait: d'une thrombose splanchnique (19%), de la présence d'une anomalie à la NFS (20%), de la survenue de thromboses dans des localisations atypiques ou devant des récidives thrombotiques inexpliquées (environ 50%). Cette recherche s'est révélée positive chez 12 patients (6%): un diagnostic de SMP a été posé chez 9 d'entre eux, celui-ci était connu chez une patiente et les 2 autres présentaient un SMP latent.Vascular events are main complications of myéloproliferative disorders (MPD). In this work, we retrospectively analyzed all JAK2V617F screening performed in the hematology laboratory of the Brest University Hospital, in a context of vascular events. JAK2V617F mutation has been found, which is present in about 95% of PV, 50% TE and 50% of MFP. JAK2V617F search purposes have been found in 93% of situations. It was: splanchnics thrombosis (19%), presence of an NFS abnormality (20%), thrombosis occurred in unusual sites or unexplained recurrent thrombosis (in 50%). Searches were positive for 12 patients (6%) and allowed MPD diagnostics for 9 patients (6 PV, 2 ET and 1 PM), MPD was known for one patient and 2 patients have a latent MPD.LYON1-BU Santé (693882101) / SudocRENNES1-BU Santé (352382103) / SudocSudocFranceF

Etude de la physiopathologie de la polyglobulie de Vaquez (recherche d' anomalies dans la transduction du signal)

PARIS7-Bibliothèque centrale (751132105) / SudocSudocFranceF

Prevalence of the JAK2 V617F mutation is low among unselected patients with a first episode of unprovoked venous thromboembolism.

International audienc

JAK2V617F and calreticulin mutations in recurrent venous thromboembolism: results from the EDITH prospective cohort.

International audienceCancer incidence in patients with recurrent unprovoked venous thromboembolism (VTE) is much higher than after a first event, but the incidence of myeloproliferative neoplasms (MPN) in this situation is still unknown. We tested for JAK2V617F and calreticulin mutants, 372 DNA samples of patients treated for (VTR). Among these patients, 10 (2.7%) were carrying JAK2V617F mutation and none of them any of the calreticulin (CALR) mutations. Among the 19 patients who had VTE recurrence under vitamin K antagonists, 4 patients (21.0%) were positive for JAK2V617F. Despite the identification of JAK2V617F mutation, only three patients were diagnosed for MPN despite a median follow-up of 4 years. We showed that the screening for JAK2V617F not CALR mutations should be helpful in this indication especially if recurrence happened under VKA therapy

Expansion of a BCR-ABL clone in a JAK2 V617F myeloproliferative neoplasm treated by ruxolitinib.

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Multiple Electrode Aggregometry is an adequate method for aspirin response testing in myeloproliferative neoplasms and differentiates the mechanisms of aspirin resistance

International audienc