Ophthalmic manifestations in IgG4-related disease

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Interstitial Lung Disease during ANCA-Associated Vasculitis: A Poor-Prognosis Factor

IF 7.873; International audience

Reducing the initial number of rituximab maintenance-therapy infusions for ANCA-associated vasculitides: randomized-trial post-hoc analysis

AbstractObjectiveThe randomized, controlled MAINRITSAN2 trial was designed to compare the capacity of an individually tailored therapy [randomization day 0 (D0)], with reinfusion only when CD19+ lymphocytes or ANCA had reappeared, or if the latter’s titre rose markedly, with that of five fixed-schedule 500-mg rituximab infusions [D0 + D14, then months (M) 6, 12 and 18] to maintain ANCA-associated vasculitis (AAV) remissions. Relapse rates did not differ at M28. This ancillary study was undertaken to evaluate the effect of omitting the D14 rituximab infusion on AAV relapse rates at M12.MethodsMAINRITSAN2 trial data were subjected to post-hoc analyses of M3, M6, M9 and M12 relapse-free surviv…

ANCA-associated vasculitides: Recommendations of the French Vasculitis Study Group on the use of immunosuppressants and biotherapies for remission induction and maintenance

Treatment of vasculitides associated with anti-neutrophil cytoplasm antibodies (ANCA) (AAVs) has evolved dramatically in recent years, particularly since the demonstration of rituximab efficacy as remission induction and maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis. In 2013, the French Vasculitis Study Group (FVSG) published recommendations for its use by clinicians. Since then, new data have made it possible to better specify and codify prescription of rituximab to treat AAVs. Herein, the FVSG Recommendations Committee, an expert panel comprised of physicians with extensive experience in the treatment and management of vasculitides, presents its con…

Risk of scleroderma according to the type of immune checkpoint inhibitors

Abstract Introduction Immune checkpoint inhibitors (ICIs) are associated with immune-related adverse events (irAEs). Among them, ICIs-induced systemic sclerosis (SSc) is poorly known. Methods To better characterize this irAE, our comprehensive approach combined the description of ICIs-induced scleroderma cases, the systematic review of the literature and the analysis of VigiBase, the WHO pharmacovigilance database. Results We identified two cases with underlying limited cutaneous SSc who presented a dramatic increase in the skin thickening following pembrolizumab, associated with scleroderma renal crisis in one case. In the literature, four cases of scleroderma and four cases of morphea hav…

Tocilizumab en association à la prednisone au cours des 3 premiers mois de traitement de l’artérite à cellules géantes : résultats d’une étude prospective, multicentrique de phase II (PHRC National HORTOCI)

Introduction L’arterite a cellules geantes (ACG) est une vascularite des gros vaisseaux habituellement traitee par corticoides. Ce traitement est remarquablement efficace mais doit etre donne a forte dose pendant 12 a 24 mois, ce qui est responsable d’une importante morbi-mortalite dans cette population âgee. Le tocilizumab (TCZ) est un anticorps monoclonal humanise dirige contre les recepteurs (soluble et membranaire) de l’interleukine-6 (IL-6). Son efficacite a recemment ete montree au cours du traitement de l’ACG [1] . Cependant, les donnees concernant l’evolution apres l’arret du TCZ manquaient dans cet essai therapeutique. De plus, la duree optimale de ce traitement couteux n’est pas c…

Long-term efficacy of remission-maintenance regimens for ANCA-associated vasculitides.

International audience; Objective - To compare long-term efficacy of remission-maintenance regimens in patients with newly diagnosed or relapsing antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. Methods - The 28-month Maintenance of Remission using Rituximab in Systemic ANCA-associated Vasculitis trial compared rituximab with azathioprine to maintain remission in patients with newly diagnosed or relapsing granulomatosis with polyangiitis, microscopic polyangiitis or renal-limited ANCA-associated vasculitis. Thereafter, prospective patient follow-up lasted until month 60. The primary endpoint was the major-relapse rate at month 60. Relapse and serious adverse event-free …

Systematic retrospective study of 64 patients with anti-Mi2 dermatomyositis: A classic skin rash with a necrotizing myositis and high risk of malignancy

T cell Polarization toward T(H)2/T(FH)2 and T(H)17/T(FH)17 in Patients with IgG4-related Disease

International audience; IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder involving virtually every organ with a risk of organ dysfunction. Despite recent studies regarding B cell and T cell compartments, the disease's pathophysiology remains poorly understood. We examined and characterized subsets of circulating lymphocytes in untreated patients with active IgG4-RD. Twenty-eight consecutive patients with biopsy-proven IgG4-RD were included in a prospective, multicentric study. Lymphocyte's subsets were analyzed by flow cytometry, with analysis of T(H)1/T(H)2/T(H)17, T-FH cells, and cytokine release by peripheral blood mononuclear cells. Results were compared to healthy contro…

Low 25-OH vitamin D serum levels correlate with severe fibrosis in HIV-HCV co-infected patients with chronic hepatitis.

Background & Aims Recent findings in hepatitis C virus (HCV)-monoinfected patients have shown a correlation between low serum levels of 25-OH vitamin D3 [25(OH)D3] and severe liver fibrosis and low sustained virologic response to therapy. Data are lacking in HIV-HCV coinfected patients. Methods One hundred and eighty nine HIV-HCV coinfected patients, who received ⩾80% of interferon (IFN) plus ribavirin therapy, were analyzed for baseline serum 25(OH)D3 levels. Correlations between serum 25(OH)D3 levels, chronic hepatitis C features, HCV virologic response to antiviral therapy, and HIV infection characteristics were analyzed. Results Mean serum 25(OH)D3 level was 18.5±9.8ng/ml, including 162…

Simulation en santé et médecine interne : quel avenir ?

Simulation-based learning (SBL) is developing rapidly in France and the question of its use in the teaching of internal medicine (IM) is essential. While HAS encourages its integration into medical education, French Young Internists (AJI) set up a working group to reflect on the added-value of this tool in our specialty. Different sorts of SBL exist: human, synthetic and electronic. It enables student to acquire and evaluate technical skills (strengths, invasive procedures, etc.) and non-technical skills (relational, reasoning…). The debriefing that follows the simulation session is an essential time in pedagogical terms. It enables the acquisition of knowledge by encouraging the students' …