Tofacitinib is an oral synthetic DMARD displaying linear pharmacokinetics. Metabolism, primarily mediated by CYP3A4, accounts for 70% of the total clearance of the drug; the remaining 30% are renally excreted. Tofacitinib monotherapy, or in combination with traditional DMARDs, has demonstrated its efficacy while having an acceptable safety profile in RA patients who have responded inadequately to current DMARDs, including TNF antagonists. In view of its undetermined benefit to risk ratio, in the real-world population, tofacitinib should, for now, only be prescribed to selected patients.

This systematic review demonstrates a correlation between oropharyngeal manifestations of systemic sclerosis and impaired quality of life, despite the low level of evidence of included studies. Large-scaled studies are needed to provide stronger evidence of this association.

Orofacial involvement is common and often understated in the treatment clinical guidelines of systemic sclerosis. It impairs daily life by having repercussions on comfort, nutrition, aesthetics and self-confidence. This review aimed at describing exhaustively the different orofacial consequences of systemic sclerosis. A systematic search was conducted using four databases (PubMed, Cochrane Library, Dentistry & Oral Sciences Source and SCOPUS) up to December 2016 according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses. Grey literature and hand search were also included. To be eligible for the inclusion, studies needed to meet the following criteria: randomised controlled trials, cross-sectional studies, case-control studies, pilot studies or cohort studies and full text available in English or French, with abstract. The studies had to concern at least 30 patients suffering from systemic sclerosis and having clinical and radiological oropharyngeal examination. The diagnosis of systemic sclerosis had to be determined according to precise recommendations; the retrieved oropharyngeal manifestations had to affect hard or soft tissues of the mouth and/or pharynx and needed to be evaluated with clinical measures. Study selection, risk bias assessment (Newcastle-Ottawa scale) and data extraction were performed by two independent reviewers. The retrieved features were microstomia and xerostomia associated with real hyposialia, temporomandibular joint symptoms, high caries experience, periodontal diseases as well as an increased risk of oral cavity and pharynx cancer. Early diagnosis enabling early management, prevention and oral hygiene is the key to avoid complicated and invasive procedures. Studies with higher level of evidence remain necessary to create standardised protocols.

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BackgroundRetention rate, efficacy, and safety of abatacept (ABA) was compared between patients with rheumatoid arthritis receiving ABA as monotherapy to those in combination ABA + conventional synthetic DMARD (csDMARD).MethodsThe patients were obtained from the ORA registry. The retention rate was analysed in two ways: (1) therapeutic strategy retention, in which the addition of a csDMARD was considered to indicate failure of the monotherapy strategy; and (2) ABA retention, which was assessed by the discontinuation of ABA regardless of other treatment modifications. Efficacy and safety were compared between ABA initiated alone and ABA used in combination with a csDMARD.ResultsThe retention rate at month 6 (M6) was evaluated in 569 patients. A significant difference was identified in the retention rate between the ABA monotherapy strategy and the ABA + csDMARD strategy (58.5 % [110/188] vs. 68 % [258/381], respectively, p = 0.031). No significant difference was identified in the ABA retention rate initiated either as a monotherapy or in combination with csDMARDs (75 % [142/188] vs. 76 % [291/381], respectively, p = 0.824). Data regarding ABA efficacy were available for 444 patients. There was no significant difference in the responder proportion after 6 months of treatment between ABA monotherapy and ABA + csDMARD treatment (60.2 % [88/146] vs. 60 % [179/298], respectively, p = 0.967).ConclusionsThis “real-life” analysis, which is relevant for bedside practice, emphasised the satisfactory efficacy and safety of ABA used in monotherapy, which provides an acceptable alternative when csDMARDs are undesirable.

BackgroundSevere weakness of axial muscle leads to dropped head syndrome or camptocormia. The signification of these symptoms has not been studied in inflammatory myopathies (IM).ObjectivesTo assess the signification of dropped head syndrome and/or camptocormia in patients with IM.MethodsAll practitioners of the French Myositis Network and the Club Rhumatisme et Inflammation (>1000 physicians) were invited to report their patients suffering from IM (myopathy with myositis specific autoantibody and/or typical muscle biopsy according to the ENMC criteria) with camptocormia and/or dropped head syndrome. These axial IM cases were included only if no other explanation of axial weakness was found. IM patients without axial involvement (non-axial IM group), were randomly selected from the participative centers, and included as control patients (ratio 1:2). Clinical, serological, muscle pathological features, management and outcomes were studied using a standardized form.ResultsTwenty patients (sex ratio 2.6) with axial involvement (camptocormia: 60%, dropped head syndrome: 40%) were included. Compared with the control group, these axial IM-patients were older (64.05±11.64 y. vs. 48.22±18.28. p<0.005) and diagnosis of IM was delayed (16.4±4.5 months vs. 8.5±2.7, p<0.05).All patient except one had also proximal weakness of the limbs. CK blood level was 2794±870 UI/L, which was similar to the controls (2864±559 UI/L). According to the ENMC classification, non-specific myositis was the most frequent finding on muscle biopsy (n=5/15, 30% vs. n=1/24, 4% in the non-axial group, p<0.05) and dermatomyositis (DM) pattern tended to be less frequent in axial-IM patients (3/12 20% vs. 11/24, 46%, p=0.10).Most of the patients (75%) had also extra muscular involvement including acrosyndrome (45%), interstitial lung disease (35%), sclerodactyly (30%), telangiectasia (25%), digital tip ulcer (10%), sclerodermy (5%). By contrast, no patient had polyarthrithis (vs. 20% in the controls, p<0.05). DM rash was hardly threefold less frequent in axial IM patients (15% vs. 42%, p<0.05).Auto-antibodies associated with scleromyositis were the most frequent in axial-IM patients (30% vs. 10.3%, p=007, manly anti-PM/Scl). One patient (5%) had cancer within the 3 years before or after IM diagnosis (NS vs. controls).Thus, most frequent diagnosis in axial IM-patients were scleromyositis (35% vs. 5% in the controls, p<0.05) and inclusion body myositis (20% vs. 2.6% in the controls, p<0.05). DM was twofold less frequent than in control (10% vs 41%, p<0.05). Other IM subtypes were not statistically different from the control groups.Except patients with diagnosis of sIBM, all axial IM-patients received corticosteroids with another immunomodulatory drugs (median number 2, range 1–5). Half of the axial-IM patients received intravenous immunoglobulin. After a mean follow up of 68.4±3.76 months all patients had improvement, including in axial weakness, except in patients with sIBM. One patient died from ischemic cardiomyopathy.ConclusionsIn IM, camptocormia and ...

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Introduction: There is a lack of joint recommendations by healthcare professionals (HCP) and patient organizations when a partnership between high and low-income countries in the field of haemophilia is planned. Aim: To draft recommendations to clarify the methodology when a partnership between low-and high-income countries is planned with the objective of a long-term implication. This methodology is to be implemented for fulfilling both medical and associative aims. Methods: Based on the available literature, a first document was written, then diffused to AFATH (Alliance Franco-Africaine pour le Traitement de l'Hémophilie) members, and after a one-day meeting and further amendments, a second draft was approved by all members before submission for publication. Results: Based on 6 years experience, several recommendations regarding the joint and separate roles of patient association and HCP for a first mission in French-speaking sub-Saharan African countries have been established. The proposed methodology for establishing preliminary contacts, the first visit and the key points for diagnostic action, medical follow-up, patient education and advocacy strategy outlines a model of partnership between patients and HCP. Conclusion: This paper written jointly by patients and physicians underlines the importance of reciprocal expert guidance and a partnership based on complementary inputs.