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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 consensus guidelines based on literature analysis, the results of prospective therapeutic trials and personal experience., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

IgG4 related disease (IgG4 RD) was first reported as autoimmune pancreatitis then it was established as a systemic disorder characterised by high blood level of IgG4 and fibrosis with rich plasmocytes IgG4+ in almost all organs. IgG4 RD is very sensitive to corticosteroid therapy. IgG4 RD has a high prevalence in eastern countries. Numerous articles on this topic are published and new diagnostic criteria are regularly established. The autoimmune or allergic mechanism of IgG4 RD is still a matter of debate. Interestingly, IgG4 subclass of antibody has anti-inflammatory features. IgG4 RD is not yet very well characterised in western countries. Whether IgG4 is involved in IgG4 RD, pathophysiology is to be defined. IgG4 RD spontaneously regresses in some cases so indications of treatment are not already well clear., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Objective: To assess the etiologic diagnoses and outcome of acute pericardial effusion associated to acute renal renal failure., Patients and Methods: A retrospective study from 1978 to 1996 in a 10 bed medical/surgical intensive care unit included 11 patients who have an acute pericardial effusion associated with an acute renal failure., Results: Etiological diagnoses were systemic lupus with extracapillary glomerulonephritis (n = 2), systemic fibrosis with obstructive renal failure (n = 2), anticoagulation accident with hemodynamic renal failure (n = 2), lung adenocarcinoma (n = 2), adenocarcinoma of undetermined origin (n = 1), systemic polyarteritis nodosa (n = 1), and Wegener granulomatosis (n = 1). Intensive care unit deaths was 4, including 2 cases of neoplasic origin., Discussion: Despite infection or malignancy being claimed as the leading causes of acute pericardial effusion, when associated to acute renal failure other etiologic diagnoses, such connectivite tissue disease or vasculitis, must be evoked that prompt specific treatment and could prevent unfavorable evolution. Neoplasic causes were characterised by receiving pericardial effusion and a high mortality rate.

The naevoid basal cell syndrome, also called Gorlin's syndrome, is a dysembryoplasia akin to the phakomatoses and which may affect the skin, the bones, the nervous system, the eyes, the endocrine glands and the genitalia. A case with numerous tumours and malformations is presented, together with a review of the literature.