Search results for "granulomatosis with polyangiitis"
showing 7 items of 27 documents
Human endothelial cells express proteinase 3, the target antigen of anticytoplasmic antibodies in Wegener's granulomatosis
1993
Abstract Autoantibodies directed against cytoplasmic antigens of neutrophils (ANCA), especially proteinase 3 (PR-3), have proved to be a useful clinical tool confirming the diagnosis or monitoring disease activity of Wegener's granulomatosis (WG). Although several concepts concerning the pathophysiologic potentials of ANCA have been discussed, only sparse data about ANCA-endothelium interactions have been available. In this study, we have investigated the expression of PR-3 in cytokine- treated human endothelial cells using purified anti-PR-3 antibodies of patients with WG, murine and human monoclonal anti-PR-3 antibodies as probes. We were able to show that tumor necrosis factor-alpha, int…
Wegener's granulomatosis : description of a case with oral manifestation
2009
Wegener?s granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolution. The diagnosis is made by clinical symptoms and signs, the presence of c-ANCA and a positive biopsy. The anatomic pathology is characterized by vasculitis, granulomatous inflammation with multinuclear gia…
Inflammatory pituitary lesions
2021
Abstract Inflammatory pituitary lesions are rare but important differential diagnoses of nonfunctioning pituitary masses. Primary hypophysitis as an autoimmune disorder has received increasing attention over the past decades. In the last decade, immune checkpoint inhibitor–induced hypophysitis has emerged as a new type of secondary hypophysitis. Various pituitary tumors may also cause secondary hypophysitis. Systemic, noninfectious granulomatous diseases that can involve the pituitary region include sarcoidosis, granulomatosis with polyangiitis, Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease. Furthermore, infectious diseases can affect the pituitary gland.…
Anti‐laminin auto antibodies in ANCA‐associated vasculitis
2000
Background. Endothelial cell damage occurs during vasculitic processes in vivo. With the alteration of the endothelium, exposure to basement membrane components may occur with induction of humoral immunity. Methods. In the present study, we evaluated the prevalence of antibodies against the basement membrane antigen laminin (LMN) in patients with ANCA-associated systemic vasculitis (AASV), pathologic controls (systemic lupus erythematosus, mixed cryoglobulinaemia, Henoch Schonlein purpura, primary glomerulonephritis) and normal individuals. Results. By ELISA, 21.6% of AASV (16/74) and 10% of pathologic controls (3/30), but only one of the normal controls (2.8%) had these antibodies (P = 0.0…
ANCA-associated vasculitides: Recommendations of the French Vasculitis Study Group on the use of immunosuppressants and biotherapies for remission in…
2019
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…
Reducing the initial number of rituximab maintenance-therapy infusions for ANCA-associated vasculitides: randomized-trial post-hoc analysis
2020
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…
THU0045 IL-25/IL-17RB AXIS IS ACTIVATED AND ASSOCIATED WITH ILC2 EXPANSION IN GRANULOMATOSIS WITH POLYANGIITIS (GPA)
2019
Background: Pathogenesis of Granulomatosis with polyangiitis (GPA) is still unknown. However, it has been observed a skewing of circulating CD4+ T cells toward the Th17 and Th2 phenotype. The pro-inflammatory cytokine interleukin 25 (IL-25) is a member of IL-17 cytokine family associated to the Th2 immune phenotype. Through the receptor IL17RB, IL-25 further sustains the Th2-type immune response and elicits the expansion of the type 2 innate lymphoid cells (ILC2) and M2 macrophages. A pathogenic role of the innate lymphoid cells in GPA has been recently demonstrated; however, the relevance of IL-25 in this condition remains unexplored. Objectives: Aim of the study was to evaluate the expres…