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2. Adding Azathioprine to Remission‐Induction Glucocorticoids for Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss), Microscopic Polyangiitis, or Polyarteritis Nodosa Without Poor Prognosis Factors: A Randomized, Controlled Trial

Author

Puéchal, Xavier, Pagnoux, Christian, Baron, Gabriel, Quémeneur, Thomas, Néel, Antoine, Agard, Christian, Lifermann, François, Liozon, Eric, Ruivard, Marc, Godmer, Pascal, Limal, Nicolas, Mékinian, Arsène, Papo, Thomas, Ruppert, Anne‐Marie, Bourgarit, Anne, Bienvenu, Boris, Geffray, Loïck, Saraux, Jean‐Luc, Diot, Elisabeth, Crestani, Bruno, Delbrel, Xavier, Sailler, Laurent, Cohen, Pascal, Le Guern, Véronique, Terrier, Benjamin, Groh, Matthieu, Le Jeunne, Claire, Mouthon, Luc, Ravaud, Philippe, Guillevin, Loïc, Lavigne, C., Gille, T., Le Guenno, G., Rieu, V., André, M., Goulenok, T., Federici, L., Szmania, I., Bonnotte, B., Vinzio, S., Bezanahary, H., Ly, K. H., Khouatra, C., Lega, J. C., Dunand, J. F., Hamidou, M., Ponge, T., Brihaye, B., Chauveheid, P., Dawidowicz, K., Bussone, G., Crabol, Y., Fois, E., de Menthon, M., Szwebel, T. A., Arlet, J. B., Israel‐Biet, D., Bloch‐Queyrat, C., Bachmeyer, C., Lavole, A., Wislez, M., Soria, P., Viallard, J.‐F., Landron, C., Poindron, V., Pourrat, J., Magnant, J., Kyndt, X., and Meckenstock, R.

Published
2017
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14. Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients

Author

Gusdorf, L., primary, Asli, B., additional, Barbarot, S., additional, Néel, A., additional, Masseau, A., additional, Puéchal, X., additional, Gottenberg, J-E., additional, Grateau, G., additional, Blanchard-Delaunay, C., additional, Rizzi, R., additional, Lifermann, F., additional, Kyndt, X., additional, Aubin, F., additional, Bessis, D., additional, Boye, T., additional, Gayet, S., additional, Rongioletti, F., additional, Sauleau, E., additional, Fermand, J-P., additional, and Lipsker, D., additional

Published
2016
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18. Granulomatose avec polyangéite (Wegener) et polyangéite microscopique : suivi à long terme de l’étude prospective WEGENT comparant l’azathioprine et le méthotrexate en traitement d’entretien chez 126 patients

Author

Puéchal, X., primary, Pagnoux, C., additional, Perrodeau, E., additional, Hamidou, M., additional, Boffa, J.J., additional, Kyndt, X., additional, Lifermann, F., additional, Merrien, D., additional, Smail, A., additional, Delaval, P., additional, Hanrotel-Saliou, C., additional, Imbert, B., additional, and Guillevin, L., additional

Published
2014
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20. [Extracapillary glomerulonephritis with anti-myeloperoxidase antibodies in 2 patients with systemic scleroderma treated with penicillamine D]

Author

Kyndt X, Ducq P, Frank Bridoux, Reumaux D, Makdassi R, Jd, Gheerbrant, and Vanhille P

Subjects
Proteinuria, Glomerulonephritis, Scleroderma, Systemic, Sex Factors, Penicillamine, Humans, Female, Raynaud Disease, Middle Aged, Aged, Antibodies, Antineutrophil Cytoplasmic, Autoimmune Diseases
Abstract

Side-effects, including autoimmune disorders, are frequent with D-penicillamine therapy. Proteinuria is observed in 10% of the patients, often secondary to extramembranous glomerulopathy. Necrotizing extracapillary glomerulonephritis is however exceptional.Two patients with systemic sclerodermia were treated with D-penicillamine for 7 and 14 years. Both developed necrotizing extracapillary glomerulonephritis with anti-myeloperoxidase antibodies (anti-MPO), associated with hemorrhagic alveolitis in one case. Partial regression of the renal failure was obtained after withdrawal of D-penicillamine and combination treatment with prednisone and cyclophosphamide.Extracapillary glomerulonephritis or a lung-kidney syndrome are frequently associated with anti-MPO antineutrophil cytoplasm antibodies (ANCA). In systemic sclerodermia, the presence of anti-MPO appears to define a group of patients at risk of pauci-immune extracapillary glomerulonephritis or a lung-kidney syndrome. In addition, the presence of ANCA in patients with renal failure would suggest extracapillary glomerulonephritis rather than sclerodermic microangiopathy. Development of extracapillary glomerulonephritis with anti-MPO in patients who are taking D-penicillamine suggests that inductor mechanisms other than D-penicillamine are involved in the pathogenesis of these glomerulopathies.

Published
1999

21. Treatment of systemic necrotizing vasculitides in patients≥65 years old: Results of the multicenter randomized CORTAGE trial

Author

Pagnoux, C., primary, Quéméneur, T., additional, Ninet, J., additional, Perrodeau, E., additional, Diot, E., additional, Kyndt, X., additional, De Wazières, B., additional, Reny, J.L., additional, Puéchal, X., additional, Leberruyer, P.Y., additional, Lidove, O., additional, Vanhille, P., additional, Godmer, P., additional, Albath-Sadiki, A., additional, Bienvenu, B., additional, Cohen, P., additional, Mouthon, L., additional, Ravaud, P., additional, and Guillevin, L., additional

Published
2013
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22. Traitement des vascularites systémiques nécrosantes chez les sujets plus ou moins 65ans : résultats de l’essai multicentrique randomisé CORTAGE

Author

Pagnoux, C., primary, Quéméneur, T., additional, Ninet, J., additional, Perrodeau, E., additional, Diot, E., additional, Kyndt, X., additional, de Wazieres, B., additional, Rény, J.-L., additional, Puéchal, X., additional, Leberruyer, P.-Y., additional, Lidove, O., additional, and Guillevin, L., additional

Published
2012
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26. Quelle est l’incidence des manifestations thrombotiques artérielles et veineuses après le diagnostic d’un syndrome des antiphospholipides obstétrical : étude rétrospective de 39 patientes

Author

Lefevre, G., primary, Lambert, M., additional, Bacri, J.L., additional, Quéméneur, T., additional, Dubucquoi, S., additional, Launay, D., additional, Kyndt, X., additional, Morell-Dubois, S., additional, Houfflin-Debarge, V., additional, Vanhille, P., additional, Hachulla, E., additional, and Hatron, P.Y., additional

Published
2009
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45. Alveolar haemorrhage in ANCA-associated vasculitides: 80 patients' features and prognostic factors

Author

Kostianovsky, A., Hauser, T., Pagnoux, C., Cohen, P., Eric DAUGAS, Mouthon, L., Miossec, P., Cordier, J. -F, Guillevin, L., Bachmeyer, C., Barbier, S., Belmatoug, N., Bielefeld, P., Bouredji, D., Bourgarit, A., Bruet, A., Cadranel, J., Chazerain, P., Chinet, T., Coëtmeur, D., Cottin, V., Demolombe-Rague, S., Dhôte, R., Girault, A., Fauchais, A. -L, Gobert, P., Godeau, B., Hillion, D., Janin-Magnificat, L., Kaloustian, E., Kouyoumdjian, L., Kyndt, X., Israël-Biet, D., Lassence, A., Leclerc, P., Le Jeunne, C., Le Noach, J., Lhote, F., Lifermann, F., Lobbedez, T., Longuet, O., Pastural, M., Plaisier, E., Ponge, T., Rogé, C., Rince, M., Rondeau, E., Rossert, J., Roux, M., Stern, M., Tanter, Y., Tirot, P., Valeyre, D., Vanhille, P., Vidal, E., Vrtovsnik, F., and Zoulim, A.

49. Serial measurements of antineutrophil cytoplasmic autoantibodies in patients with systemic vasculitis.

Author

Kyndt, Xavier, Reumaux, Dominique, Kyndt, X, Reumaux, D, Bridoux, F, Tribout, B, Bataille, P, Hachulla, E, Hatron, P Y, Duthilleul, P, and Vanhille, P

Subjects
*VASCULITIS, *AUTOANTIBODIES
Abstract

Purpose: To assess the value of serial determinations of antineutrophil cytoplasmic autoantibodies (ANCA) for monitoring disease activity in patients with systemic vasculitis.Patients and Methods: Forty-three patients with histologically proven vasculitis (21 with Wegener's granulomatosis, 17 with microscopic polyangiitis, and 5 with renal-limited vasculitis) were studied for a median follow-up of 22 months. Disease activity was prospectively assessed and quantified by the Birmingham Vasculitis Activity Score. A total of 347 sera were analyzed for ANCA determination.Results: Relapses occurred in 23 (54%) of 43 patients. Diagnostic category (Wegener's granulomatosis vs micropolyangiitis and renal-limited vasculitis), severity of initial symptoms (mean vasculitis activity score, mean number of organs involved), and ANCA pattern [cytoplasmic-ANCA (c-ANCA) vs perinuclear-ANCA (p-ANCA)] did not significantly differ between relapsers and nonrelapsers. Lung involvement was more frequent at onset among relapsers [16 of 23 (70%) vs 6 of 20 (30%); P = 0.02]. Relapses were slightly, but not significantly, more frequent in patients with Wegener's granulomatosis or a c-ANCA pattern. The percentage of relapsers was greater in patients with persistently positive ANCA than in patients with negative or decreasing ANCA titers (86% vs 20%, P = 0.0001). However, the predictive value of an increase in ANCA titers for the occurrence of a subsequent relapse was only 28% (4 of 14) for c-ANCA, 12% (2 of 17) for anti-proteinase 3-ANCA, and 43% (6 of 14) for anti-myeloperoxidase-ANCA. An increase in ANCA occurred before or during relapse in 33% (10 of 30) of cases for c-ANCA/anti-proteinase 3 antibodies, and 73% (11 of 15) of cases for anti-myeloperoxidase antibodies.Conclusion: The persistence of ANCA positivity is strongly associated with relapses. However, an increase in ANCA titers has a poor value for the early prediction of a subsequent relapse and should not be used as a sole parameter for therapeutic intervention. In addition, our results suggest that serial anti-myeloperoxidase determination may be useful as a prognostic marker in patients who are p-ANCA positive. [ABSTRACT FROM AUTHOR]

Published
1999
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50. Cytokine Signature in Schnitzler Syndrome: Proinflammatory Cytokine Production Associated to Th Suppression

Author

Marie Masson Regnault, Eric Frouin, Isabelle Jéru, Adriana Delwail, Sandrine Charreau, Sébastien Barbarot, Antoine Néel, Agathe Masseau, Xavier Puéchal, Xavier Kyndt, Stephane Gayet, François Lifermann, Bouchra Asli, Xavier Balguerie, Claire Blanchard-Delaunay, François Aubin, Rita Rizzi, Franco Rongioletti, Thierry Boyé, Laurence Gusdorf, Didier Bessis, Franck Morel, Ewa Hainaut, Dan Lipsker, Jean-Claude Lecron, Masson Regnault, M., Frouin, E., Jeru, I., Delwail, A., Charreau, S., Barbarot, S., Neel, A., Masseau, A., Puechal, X., Kyndt, X., Gayet, S., Lifermann, F., Asli, B., Balguerie, X., Blanchard-Delaunay, C., Aubin, F., Rizzi, R., Rongioletti, F., Boye, T., Gusdorf, L., Bessis, D., Morel, F., Hainaut, E., Lipsker, D., and Lecron, J. -C.

Subjects
lcsh:Immunologic diseases. Allergy, 0301 basic medicine, Adult, Lipopolysaccharides, Male, PBMC (peripheral blood mononuclear cells), medicine.medical_treatment, Immunology, Inflammation, Peripheral blood mononuclear cell, Proinflammatory cytokine, 03 medical and health sciences, 0302 clinical medicine, Schnitzler syndrome, inflammasome, Gammopathy, medicine, Immunology and Allergy, Humans, Cells, Cultured, Original Research, Aged, Aged, 80 and over, business.industry, IL-1, IL-1 antagonist, Interleukin, T-Lymphocytes, Helper-Inducer, Middle Aged, medicine.disease, cytokines, Interleukin 1 Receptor Antagonist Protein, 030104 developmental biology, Cytokine, 030228 respiratory system, Antirheumatic Agents, ex vivo, Tumor necrosis factor alpha, Female, medicine.symptom, lcsh:RC581-607, business
Abstract

BackgroundSchnitzler syndrome (SchS) is a rare autoinflammatory disease characterized by urticarial exanthema, bone and joint alterations, fever and monoclonal IgM gammopathy. Overactivation of the interleukin(IL)-1 system is reported, even though the exact pathophysiological pathways remain unknown.ObjectiveTo determine ex vivo cytokine profiles of Peripheral Blood Mononuclear Cells (PBMCs) from SchS patients prior to treatment and after initiation of anti-IL-1 therapy (anakinra). The sera cytokine profile was studied in parallel.MethodsWe collected blood samples from thirty-six untreated or treated SchS. PBMCs were cultured with and without LPS or anti-CD3/CD28. Cytokine levels were evaluated in serum and cell culture supernatants using Luminex technology.ResultsSpontaneous TNFα, IL-6, IL-1β, IL-1α, and IL-1RA release by PBMCs of SchS patients were higher than in controls. LPS-stimulation further induced the secretion of these cytokines. In contrast, after T-cell stimulation, TNFα, IL-10, IFNγ, IL-17A, and IL-4 production decreased in SchS patients compared to healthy controls, but less in treated patients. Whereas IL-1β serum level was not detected in most sera, IL-6, IL-10, and TNFα serum levels were higher in patients with SchS and IFNγ and IL-4 levels were lower. Of note, IL-6 decreased after treatment in SchS (p = 0.04).ConclusionOur data strengthen the hypothesis of myeloid inflammation in SchS, mediated in particular by IL-1β, TNFα, and IL-6, associated with overproduction of the inhibitors IL-1RA and IL-10. In contrast, we observed a loss of Th1, Th2, and Th17 cell functionalities that tends to be reversed by anakinra.

Published
2020

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