Lymphopénie T CD4 idiopathique : caractéristiques cliniques et immunologiques, suivi à long terme de 36 patients

REIMS-BU Santé (514542104) / SudocSudocFranceF

Rituximab for Induction and Maintenance Therapy of Granulomatosis with Polyangiitis: A Single-Center Cohort Study on 114 Patients

Univ Paris 05, Hop Cochin, AP HP, Natl Referral Ctr Rare System Autoimmune Dis, Paris, FranceUniv Fed Sao Paulo, Hosp Sao Paulo, Sao Paulo, BrazilHop Hotel Dieu, Epidemiol, Paris, FranceHop Cochin, AP HP, DHU Authors,Serv Med Interne, Ctr Reference Natl Malad Syst Autoimmunes Rares I, Paris, FranceUniv Paris Descartes Sorbonne Paris, Paris, FranceParis Hotel Dieu, Paris, FranceHop Cochin, Serv Med Interne, Ctr Reference Malad Autoimmunes & Autoinflammatoi, Paris, FranceUniv Fed Sao Paulo, Hosp Sao Paulo, Sao Paulo, BrazilWeb of Scienc

Situations de départ et attendus d’apprentissage en deuxième cycle des études médicales : comment utiliser ces nouveaux référentiels en médecine générale ?

International audienc

Clinical Presentation, Treatment and Outcome of IgG4-related Pachymeningitis: From a National Case Registry and Literature Review

International audienc

Treatment of mixed connective tissue disease: A multicenter retrospective study

International audienceIntroductionMixed connective tissue disease (MCTD) is a rare systemic disorder that belongs to connective tissue diseases (CTD). Few studies are available on MCTD treatment.MethodsWe conducted an observational study within the French MCTD cohort. Data were collected at diagnosis, during follow-up, and at the last follow-up (LFU). We studied three treatment groups i) no treatment, ii) hydroxychloroquine (HCQ) and/or glucocorticoids (GC) and iii) disease-modifying antirheumatic drugs (DMARDs)/immunosuppressant (IS).ResultsThree hundred and fifteen patients were included and followed for 96 [40–156] months. At MCTD diagnosis, 52 (16.5 %) patients were treatment-free, while 224 (71.1 %) received GC and/or HCQ and 39 (12.4 %) received DMARDs and/or IS. During follow-up, 10 (3.2 %) patients remained treatment-free, and 77 (24.4 %) were GC-free. Most patients (n = 271; 85.8 %) received HCQ, and 161 (51.1 %) were treated with DMARDs and/or IS. DMARDs and/or IS, including anti-B cell therapeutics, were more frequently prescribed in patients with musculoskeletal involvement (p < 0.0001), interstitial lung disease (ILD, p < 0.0001) and/or pulmonary arterial hypertension (PAH, p < 0.01). Patients in clinical remission and those who did not evolve to a differentiated CTD (MCTD-dCTD) received significantly less frequently DMARDs and/or IS (including anti-B cell therapeutics; p < 0.0001 for both). Patients who received HCQ at MCTD diagnosis appeared to develop less frequently ILD or PAH (p < 0.05).ConclusionHCQ and GC were the cornerstones of MCTD treatment and were sufficient to control disease manifestations in nearly half of the patients, reflecting the good prognosis of this disease. DMARDs and IS were used for musculoskeletal involvement, PAH/ILD, and in MCTD-dCTD patients