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Efficacy of rituximab in systemic manifestations of primary Sjögren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry

Identifieur interne : 002324 ( Istex/Corpus ); précédent : 002323; suivant : 002325

Efficacy of rituximab in systemic manifestations of primary Sjögren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry

Auteurs : Jacques-Eric Gottenberg ; Gael Cinquetti ; Claire Larroche ; Bernard Combe ; Eric Hachulla ; Olivier Meyer ; Edouard Pertuiset ; Guy Kaplanski ; Laurent Chiche ; Jean-Marie Berthelot ; Bruno Gombert ; Philippe Goupille ; Christian Marcelli ; Séverine Feuillet ; Jean Leone ; Jean Sibilia ; Charles Zarnitsky ; Philippe Carli ; Stephanie Rist ; Philippe Gaudin ; Carine Salliot ; Muriel Piperno ; Adeline Deplas ; Maxime Breban ; Thierry Lequerre ; Pascal Richette ; Charles Ghiringhelli ; Mohamed Hamidou ; Philippe Ravaud ; Xavier Mariette

Source :

RBID : ISTEX:52A3B80A2058E94C2AC1BECDAEDC9D33451FAAA8

English descriptors

Abstract

Objectives To evaluate the efficacy and safety of rituximab in patients with primary Sjögren's syndrome (pSS). Methods The AutoImmune and Rituximab registry has included 86 patients with pSS treated with rituximab, prospectivey followed up every 6 months for 5 years. Results Seventy-eight patients with pSS (11 men, 67 women), who already had at least one follow-up visit, were analysed. Median age was 59.8 years (29–83), median duration of disease was 11.9 years (3–32). Indications for treatment were systemic involvement for 74 patients and only severe glandular involvement in four patients. The median European Sjögren's Syndrome disease activity index (ESSDAI) was 11 (2–31). 17 patients were concomitantly treated with another immunosuppressant agent. Median follow-up was 34.9 months (6–81.4) (226 patient-years). Overall efficacy according to the treating physician was observed in 47 patients (60%) after the first cycle of rituximab. Median ESSDAI decreased from 11 (2–31) to 7.5 (0–26) (p<0.0001). Median dosage of corticosteroid decreased from 17.6 mg/day (3–60) to 10.8 mg/day (p=0.1). Forty-one patients were retreated with rituximab. Four infusion reactions and one delayed serum sickness-like disease resulted in rituximab discontinuation. Three serious infections (1.3/100 patient-years) and two cancer-related deaths occurred. Conclusions In common practice, the use of rituximab in pSS is mostly restricted to patients with systemic involvement. This prospective study shows good efficacy and tolerance of rituximab in patients with pSS and systemic involvement.

Url:
DOI: 10.1136/annrheumdis-2012-202293

Links to Exploration step

ISTEX:52A3B80A2058E94C2AC1BECDAEDC9D33451FAAA8

Le document en format XML

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<name sortKey="Combe, Bernard" sort="Combe, Bernard" uniqKey="Combe B" first="Bernard" last="Combe">Bernard Combe</name>
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<name sortKey="Hachulla, Eric" sort="Hachulla, Eric" uniqKey="Hachulla E" first="Eric" last="Hachulla">Eric Hachulla</name>
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<name sortKey="Meyer, Olivier" sort="Meyer, Olivier" uniqKey="Meyer O" first="Olivier" last="Meyer">Olivier Meyer</name>
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<name sortKey="Kaplanski, Guy" sort="Kaplanski, Guy" uniqKey="Kaplanski G" first="Guy" last="Kaplanski">Guy Kaplanski</name>
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<name sortKey="Chiche, Laurent" sort="Chiche, Laurent" uniqKey="Chiche L" first="Laurent" last="Chiche">Laurent Chiche</name>
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<name sortKey="Berthelot, Jean Marie" sort="Berthelot, Jean Marie" uniqKey="Berthelot J" first="Jean-Marie" last="Berthelot">Jean-Marie Berthelot</name>
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<name sortKey="Gombert, Bruno" sort="Gombert, Bruno" uniqKey="Gombert B" first="Bruno" last="Gombert">Bruno Gombert</name>
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<name sortKey="Goupille, Philippe" sort="Goupille, Philippe" uniqKey="Goupille P" first="Philippe" last="Goupille">Philippe Goupille</name>
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<name sortKey="Marcelli, Christian" sort="Marcelli, Christian" uniqKey="Marcelli C" first="Christian" last="Marcelli">Christian Marcelli</name>
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<mods:affiliation>Service de Rhumatologie, CHU de Caen, Caen, France</mods:affiliation>
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<name sortKey="Feuillet, Severine" sort="Feuillet, Severine" uniqKey="Feuillet S" first="Séverine" last="Feuillet">Séverine Feuillet</name>
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<mods:affiliation>Service de Pneumologie, Hôpital Saint Louis, Paris, France</mods:affiliation>
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<name sortKey="Leone, Jean" sort="Leone, Jean" uniqKey="Leone J" first="Jean" last="Leone">Jean Leone</name>
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<mods:affiliation>Service de Médecine Interne, Hôpital Debré, Reims, France</mods:affiliation>
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<name sortKey="Sibilia, Jean" sort="Sibilia, Jean" uniqKey="Sibilia J" first="Jean" last="Sibilia">Jean Sibilia</name>
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<name sortKey="Zarnitsky, Charles" sort="Zarnitsky, Charles" uniqKey="Zarnitsky C" first="Charles" last="Zarnitsky">Charles Zarnitsky</name>
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<name sortKey="Carli, Philippe" sort="Carli, Philippe" uniqKey="Carli P" first="Philippe" last="Carli">Philippe Carli</name>
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<name sortKey="Rist, Stephanie" sort="Rist, Stephanie" uniqKey="Rist S" first="Stephanie" last="Rist">Stephanie Rist</name>
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<name sortKey="Gaudin, Philippe" sort="Gaudin, Philippe" uniqKey="Gaudin P" first="Philippe" last="Gaudin">Philippe Gaudin</name>
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<mods:affiliation>Service de Rhumatologie, CHU Hôpital Sud, Grenoble, Echirolles, France</mods:affiliation>
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<name sortKey="Salliot, Carine" sort="Salliot, Carine" uniqKey="Salliot C" first="Carine" last="Salliot">Carine Salliot</name>
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<name sortKey="Piperno, Muriel" sort="Piperno, Muriel" uniqKey="Piperno M" first="Muriel" last="Piperno">Muriel Piperno</name>
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<name sortKey="Deplas, Adeline" sort="Deplas, Adeline" uniqKey="Deplas A" first="Adeline" last="Deplas">Adeline Deplas</name>
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<mods:affiliation>Service de Rhumatologie, CH Niort, Niort France</mods:affiliation>
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<name sortKey="Breban, Maxime" sort="Breban, Maxime" uniqKey="Breban M" first="Maxime" last="Breban">Maxime Breban</name>
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<mods:affiliation>Service de Rhumatologie, CHU Ambroise Paré, Boulogne Billancourt, France</mods:affiliation>
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<name sortKey="Lequerre, Thierry" sort="Lequerre, Thierry" uniqKey="Lequerre T" first="Thierry" last="Lequerre">Thierry Lequerre</name>
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<mods:affiliation>Service de Rhumatologie, CHU Rouen, Rouen, France</mods:affiliation>
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<name sortKey="Richette, Pascal" sort="Richette, Pascal" uniqKey="Richette P" first="Pascal" last="Richette">Pascal Richette</name>
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<mods:affiliation>Service de Rhumatologie, Hôpital Lariboisière, Paris, France</mods:affiliation>
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<name sortKey="Ghiringhelli, Charles" sort="Ghiringhelli, Charles" uniqKey="Ghiringhelli C" first="Charles" last="Ghiringhelli">Charles Ghiringhelli</name>
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<mods:affiliation>Service de Médecine Interne, Hôpital Saint André, Bordeaux, France</mods:affiliation>
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<name sortKey="Hamidou, Mohamed" sort="Hamidou, Mohamed" uniqKey="Hamidou M" first="Mohamed" last="Hamidou">Mohamed Hamidou</name>
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<mods:affiliation>Service de Médecine Interne, CHU de Nantes, Nantes, France</mods:affiliation>
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<name sortKey="Ravaud, Philippe" sort="Ravaud, Philippe" uniqKey="Ravaud P" first="Philippe" last="Ravaud">Philippe Ravaud</name>
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<mods:affiliation>INSERM U738, Département d'Epidémiologie, Biostatistiques et Recherche Clinique, Hôtel Dieu, Centre d'épidémiologie clinique, Paris, France</mods:affiliation>
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<name sortKey="Mariette, Xavier" sort="Mariette, Xavier" uniqKey="Mariette X" first="Xavier" last="Mariette">Xavier Mariette</name>
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<mods:affiliation>Service de Rhumatologie, Université Paris Sud, AP-HP, Hôpitaux Universitaires Paris Sud, Paris, France</mods:affiliation>
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<title level="a">Efficacy of rituximab in systemic manifestations of primary Sjögren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry</title>
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<name sortKey="Cinquetti, Gael" sort="Cinquetti, Gael" uniqKey="Cinquetti G" first="Gael" last="Cinquetti">Gael Cinquetti</name>
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<name sortKey="Larroche, Claire" sort="Larroche, Claire" uniqKey="Larroche C" first="Claire" last="Larroche">Claire Larroche</name>
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<name sortKey="Combe, Bernard" sort="Combe, Bernard" uniqKey="Combe B" first="Bernard" last="Combe">Bernard Combe</name>
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<name sortKey="Hachulla, Eric" sort="Hachulla, Eric" uniqKey="Hachulla E" first="Eric" last="Hachulla">Eric Hachulla</name>
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<mods:affiliation>Service de Médecine interne, CHU Lille, Lille, France</mods:affiliation>
</affiliation>
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<name sortKey="Meyer, Olivier" sort="Meyer, Olivier" uniqKey="Meyer O" first="Olivier" last="Meyer">Olivier Meyer</name>
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<mods:affiliation>Service de Rhumatologie, Hôpital Bichat, Paris, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Pertuiset, Edouard" sort="Pertuiset, Edouard" uniqKey="Pertuiset E" first="Edouard" last="Pertuiset">Edouard Pertuiset</name>
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<mods:affiliation>Service de Rhumatologie, CH Pontoise, Pontoise, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Kaplanski, Guy" sort="Kaplanski, Guy" uniqKey="Kaplanski G" first="Guy" last="Kaplanski">Guy Kaplanski</name>
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<mods:affiliation>Service de Médecine interne, Hôpital de la conception, Marseille, France</mods:affiliation>
</affiliation>
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<name sortKey="Chiche, Laurent" sort="Chiche, Laurent" uniqKey="Chiche L" first="Laurent" last="Chiche">Laurent Chiche</name>
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<mods:affiliation>Service de Médecine interne, Hôpital de la conception, Marseille, France</mods:affiliation>
</affiliation>
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<name sortKey="Berthelot, Jean Marie" sort="Berthelot, Jean Marie" uniqKey="Berthelot J" first="Jean-Marie" last="Berthelot">Jean-Marie Berthelot</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, INSERM U957, CHU Nantes, Nantes, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Gombert, Bruno" sort="Gombert, Bruno" uniqKey="Gombert B" first="Bruno" last="Gombert">Bruno Gombert</name>
<affiliation>
<mods:affiliation>Service de Médecine Polyvalente - Rhumatologie, CH de La Rochelle, La Rochelle, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Goupille, Philippe" sort="Goupille, Philippe" uniqKey="Goupille P" first="Philippe" last="Goupille">Philippe Goupille</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, CHU Tours, Tours, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Marcelli, Christian" sort="Marcelli, Christian" uniqKey="Marcelli C" first="Christian" last="Marcelli">Christian Marcelli</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, CHU de Caen, Caen, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Feuillet, Severine" sort="Feuillet, Severine" uniqKey="Feuillet S" first="Séverine" last="Feuillet">Séverine Feuillet</name>
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<mods:affiliation>Service de Pneumologie, Hôpital Saint Louis, Paris, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Leone, Jean" sort="Leone, Jean" uniqKey="Leone J" first="Jean" last="Leone">Jean Leone</name>
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<mods:affiliation>Service de Médecine Interne, Hôpital Debré, Reims, France</mods:affiliation>
</affiliation>
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<name sortKey="Sibilia, Jean" sort="Sibilia, Jean" uniqKey="Sibilia J" first="Jean" last="Sibilia">Jean Sibilia</name>
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<mods:affiliation>Service de Rhumatologie, Centre National de Références des Maladies Auto-Immunes Systémiques Rares, Hôpitaux Universitaires de Strasbourg, Immunorhumathologie Moléculaire, INSERM UMR S 1109, Université de Strasbourg, Strasbourg, France</mods:affiliation>
</affiliation>
</author>
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<name sortKey="Zarnitsky, Charles" sort="Zarnitsky, Charles" uniqKey="Zarnitsky C" first="Charles" last="Zarnitsky">Charles Zarnitsky</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, Groupe Hospitalier du Havre, Le Havre, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Carli, Philippe" sort="Carli, Philippe" uniqKey="Carli P" first="Philippe" last="Carli">Philippe Carli</name>
<affiliation>
<mods:affiliation>Service de Médecine Interne, HIA Sainte-Anne, Toulon, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Rist, Stephanie" sort="Rist, Stephanie" uniqKey="Rist S" first="Stephanie" last="Rist">Stephanie Rist</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, CHR Orléans, Orléans, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Gaudin, Philippe" sort="Gaudin, Philippe" uniqKey="Gaudin P" first="Philippe" last="Gaudin">Philippe Gaudin</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, CHU Hôpital Sud, Grenoble, Echirolles, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Salliot, Carine" sort="Salliot, Carine" uniqKey="Salliot C" first="Carine" last="Salliot">Carine Salliot</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, CHR Orléans, Orléans, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Piperno, Muriel" sort="Piperno, Muriel" uniqKey="Piperno M" first="Muriel" last="Piperno">Muriel Piperno</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, CHU Lyon Sud France,</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Deplas, Adeline" sort="Deplas, Adeline" uniqKey="Deplas A" first="Adeline" last="Deplas">Adeline Deplas</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, CH Niort, Niort France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Breban, Maxime" sort="Breban, Maxime" uniqKey="Breban M" first="Maxime" last="Breban">Maxime Breban</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, CHU Ambroise Paré, Boulogne Billancourt, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Lequerre, Thierry" sort="Lequerre, Thierry" uniqKey="Lequerre T" first="Thierry" last="Lequerre">Thierry Lequerre</name>
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<mods:affiliation>Service de Rhumatologie, CHU Rouen, Rouen, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Richette, Pascal" sort="Richette, Pascal" uniqKey="Richette P" first="Pascal" last="Richette">Pascal Richette</name>
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<mods:affiliation>Service de Rhumatologie, Hôpital Lariboisière, Paris, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Ghiringhelli, Charles" sort="Ghiringhelli, Charles" uniqKey="Ghiringhelli C" first="Charles" last="Ghiringhelli">Charles Ghiringhelli</name>
<affiliation>
<mods:affiliation>Service de Médecine Interne, Hôpital Saint André, Bordeaux, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Hamidou, Mohamed" sort="Hamidou, Mohamed" uniqKey="Hamidou M" first="Mohamed" last="Hamidou">Mohamed Hamidou</name>
<affiliation>
<mods:affiliation>Service de Médecine Interne, CHU de Nantes, Nantes, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Ravaud, Philippe" sort="Ravaud, Philippe" uniqKey="Ravaud P" first="Philippe" last="Ravaud">Philippe Ravaud</name>
<affiliation>
<mods:affiliation>INSERM U738, Département d'Epidémiologie, Biostatistiques et Recherche Clinique, Hôtel Dieu, Centre d'épidémiologie clinique, Paris, France</mods:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Mariette, Xavier" sort="Mariette, Xavier" uniqKey="Mariette X" first="Xavier" last="Mariette">Xavier Mariette</name>
<affiliation>
<mods:affiliation>Service de Rhumatologie, Université Paris Sud, AP-HP, Hôpitaux Universitaires Paris Sud, Paris, France</mods:affiliation>
</affiliation>
</author>
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<series>
<title level="j">Annals of the Rheumatic Diseases</title>
<title level="j" type="abbrev">Ann Rheum Dis</title>
<idno type="ISSN">0003-4967</idno>
<idno type="eISSN">1468-2060</idno>
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<publisher>BMJ Publishing Group Ltd and European League Against Rheumatism</publisher>
<date type="published" when="2013-06">2013-06</date>
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<div type="abstract">Objectives To evaluate the efficacy and safety of rituximab in patients with primary Sjögren's syndrome (pSS). Methods The AutoImmune and Rituximab registry has included 86 patients with pSS treated with rituximab, prospectivey followed up every 6 months for 5 years. Results Seventy-eight patients with pSS (11 men, 67 women), who already had at least one follow-up visit, were analysed. Median age was 59.8 years (29–83), median duration of disease was 11.9 years (3–32). Indications for treatment were systemic involvement for 74 patients and only severe glandular involvement in four patients. The median European Sjögren's Syndrome disease activity index (ESSDAI) was 11 (2–31). 17 patients were concomitantly treated with another immunosuppressant agent. Median follow-up was 34.9 months (6–81.4) (226 patient-years). Overall efficacy according to the treating physician was observed in 47 patients (60%) after the first cycle of rituximab. Median ESSDAI decreased from 11 (2–31) to 7.5 (0–26) (p<0.0001). Median dosage of corticosteroid decreased from 17.6 mg/day (3–60) to 10.8 mg/day (p=0.1). Forty-one patients were retreated with rituximab. Four infusion reactions and one delayed serum sickness-like disease resulted in rituximab discontinuation. Three serious infections (1.3/100 patient-years) and two cancer-related deaths occurred. Conclusions In common practice, the use of rituximab in pSS is mostly restricted to patients with systemic involvement. This prospective study shows good efficacy and tolerance of rituximab in patients with pSS and systemic involvement.</div>
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<abstract>Objectives To evaluate the efficacy and safety of rituximab in patients with primary Sjögren's syndrome (pSS). Methods The AutoImmune and Rituximab registry has included 86 patients with pSS treated with rituximab, prospectivey followed up every 6 months for 5 years. Results Seventy-eight patients with pSS (11 men, 67 women), who already had at least one follow-up visit, were analysed. Median age was 59.8 years (29–83), median duration of disease was 11.9 years (3–32). Indications for treatment were systemic involvement for 74 patients and only severe glandular involvement in four patients. The median European Sjögren's Syndrome disease activity index (ESSDAI) was 11 (2–31). 17 patients were concomitantly treated with another immunosuppressant agent. Median follow-up was 34.9 months (6–81.4) (226 patient-years). Overall efficacy according to the treating physician was observed in 47 patients (60%) after the first cycle of rituximab. Median ESSDAI decreased from 11 (2–31) to 7.5 (0–26) (p>0.0001). Median dosage of corticosteroid decreased from 17.6 mg/day (3–60) to 10.8 mg/day (p=0.1). Forty-one patients were retreated with rituximab. Four infusion reactions and one delayed serum sickness-like disease resulted in rituximab discontinuation. Three serious infections (1.3/100 patient-years) and two cancer-related deaths occurred. Conclusions In common practice, the use of rituximab in pSS is mostly restricted to patients with systemic involvement. This prospective study shows good efficacy and tolerance of rituximab in patients with pSS and systemic involvement.</abstract>
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<note>J-EG and GC contributed equally to this study.</note>
<note>Correspondence to Professor Jacques-Eric Gottenberg, ImmunoRhumatologie Moléculaire, Service de Rhumatologie, INSERM UMR_S 1109, Université de Strasbourg, Centre National de Référence des Maladies Auto-Immunes, Hôpital Hautepierre, 1 Avenue Molière, 67000 Strasbourg 67098, France; jacques-eric.gottenberg@chru-strasbourg.fr</note>
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<forename type="first">Gael</forename>
<surname>Cinquetti</surname>
</persName>
<affiliation>Service de Médecine Interne et Maladies Infectieuses, HIA Legouest, Metz, France</affiliation>
</author>
<author xml:id="author-0002">
<persName>
<forename type="first">Claire</forename>
<surname>Larroche</surname>
</persName>
<affiliation>Service de Médecine interne, CHU Avicenne, Bobigny, France</affiliation>
</author>
<author xml:id="author-0003">
<persName>
<forename type="first">Bernard</forename>
<surname>Combe</surname>
</persName>
<affiliation>Service d'Immunorhumathologie, CHU Montpellier, Montpellier, France</affiliation>
</author>
<author xml:id="author-0004">
<persName>
<forename type="first">Eric</forename>
<surname>Hachulla</surname>
</persName>
<affiliation>Service de Médecine interne, CHU Lille, Lille, France</affiliation>
</author>
<author xml:id="author-0005">
<persName>
<forename type="first">Olivier</forename>
<surname>Meyer</surname>
</persName>
<affiliation>Service de Rhumatologie, Hôpital Bichat, Paris, France</affiliation>
</author>
<author xml:id="author-0006">
<persName>
<forename type="first">Edouard</forename>
<surname>Pertuiset</surname>
</persName>
<affiliation>Service de Rhumatologie, CH Pontoise, Pontoise, France</affiliation>
</author>
<author xml:id="author-0007">
<persName>
<forename type="first">Guy</forename>
<surname>Kaplanski</surname>
</persName>
<affiliation>Service de Médecine interne, Hôpital de la conception, Marseille, France</affiliation>
</author>
<author xml:id="author-0008">
<persName>
<forename type="first">Laurent</forename>
<surname>Chiche</surname>
</persName>
<affiliation>Service de Médecine interne, Hôpital de la conception, Marseille, France</affiliation>
</author>
<author xml:id="author-0009">
<persName>
<forename type="first">Jean-Marie</forename>
<surname>Berthelot</surname>
</persName>
<affiliation>Service de Rhumatologie, INSERM U957, CHU Nantes, Nantes, France</affiliation>
</author>
<author xml:id="author-0010">
<persName>
<forename type="first">Bruno</forename>
<surname>Gombert</surname>
</persName>
<affiliation>Service de Médecine Polyvalente - Rhumatologie, CH de La Rochelle, La Rochelle, France</affiliation>
</author>
<author xml:id="author-0011">
<persName>
<forename type="first">Philippe</forename>
<surname>Goupille</surname>
</persName>
<affiliation>Service de Rhumatologie, CHU Tours, Tours, France</affiliation>
</author>
<author xml:id="author-0012">
<persName>
<forename type="first">Christian</forename>
<surname>Marcelli</surname>
</persName>
<affiliation>Service de Rhumatologie, CHU de Caen, Caen, France</affiliation>
</author>
<author xml:id="author-0013">
<persName>
<forename type="first">Séverine</forename>
<surname>Feuillet</surname>
</persName>
<affiliation>Service de Pneumologie, Hôpital Saint Louis, Paris, France</affiliation>
</author>
<author xml:id="author-0014">
<persName>
<forename type="first">Jean</forename>
<surname>Leone</surname>
</persName>
<affiliation>Service de Médecine Interne, Hôpital Debré, Reims, France</affiliation>
</author>
<author xml:id="author-0015">
<persName>
<forename type="first">Jean</forename>
<surname>Sibilia</surname>
</persName>
<affiliation>Service de Rhumatologie, Centre National de Références des Maladies Auto-Immunes Systémiques Rares, Hôpitaux Universitaires de Strasbourg, Immunorhumathologie Moléculaire, INSERM UMR S 1109, Université de Strasbourg, Strasbourg, France</affiliation>
</author>
<author xml:id="author-0016">
<persName>
<forename type="first">Charles</forename>
<surname>Zarnitsky</surname>
</persName>
<affiliation>Service de Rhumatologie, Groupe Hospitalier du Havre, Le Havre, France</affiliation>
</author>
<author xml:id="author-0017">
<persName>
<forename type="first">Philippe</forename>
<surname>Carli</surname>
</persName>
<affiliation>Service de Médecine Interne, HIA Sainte-Anne, Toulon, France</affiliation>
</author>
<author xml:id="author-0018">
<persName>
<forename type="first">Stephanie</forename>
<surname>Rist</surname>
</persName>
<affiliation>Service de Rhumatologie, CHR Orléans, Orléans, France</affiliation>
</author>
<author xml:id="author-0019">
<persName>
<forename type="first">Philippe</forename>
<surname>Gaudin</surname>
</persName>
<affiliation>Service de Rhumatologie, CHU Hôpital Sud, Grenoble, Echirolles, France</affiliation>
</author>
<author xml:id="author-0020">
<persName>
<forename type="first">Carine</forename>
<surname>Salliot</surname>
</persName>
<affiliation>Service de Rhumatologie, CHR Orléans, Orléans, France</affiliation>
</author>
<author xml:id="author-0021">
<persName>
<forename type="first">Muriel</forename>
<surname>Piperno</surname>
</persName>
<affiliation>Service de Rhumatologie, CHU Lyon Sud France,</affiliation>
</author>
<author xml:id="author-0022">
<persName>
<forename type="first">Adeline</forename>
<surname>Deplas</surname>
</persName>
<affiliation>Service de Rhumatologie, CH Niort, Niort France</affiliation>
</author>
<author xml:id="author-0023">
<persName>
<forename type="first">Maxime</forename>
<surname>Breban</surname>
</persName>
<affiliation>Service de Rhumatologie, CHU Ambroise Paré, Boulogne Billancourt, France</affiliation>
</author>
<author xml:id="author-0024">
<persName>
<forename type="first">Thierry</forename>
<surname>Lequerre</surname>
</persName>
<affiliation>Service de Rhumatologie, CHU Rouen, Rouen, France</affiliation>
</author>
<author xml:id="author-0025">
<persName>
<forename type="first">Pascal</forename>
<surname>Richette</surname>
</persName>
<affiliation>Service de Rhumatologie, Hôpital Lariboisière, Paris, France</affiliation>
</author>
<author xml:id="author-0026">
<persName>
<forename type="first">Charles</forename>
<surname>Ghiringhelli</surname>
</persName>
<affiliation>Service de Médecine Interne, Hôpital Saint André, Bordeaux, France</affiliation>
</author>
<author xml:id="author-0027">
<persName>
<forename type="first">Mohamed</forename>
<surname>Hamidou</surname>
</persName>
<affiliation>Service de Médecine Interne, CHU de Nantes, Nantes, France</affiliation>
</author>
<author xml:id="author-0028">
<persName>
<forename type="first">Philippe</forename>
<surname>Ravaud</surname>
</persName>
<affiliation>INSERM U738, Département d'Epidémiologie, Biostatistiques et Recherche Clinique, Hôtel Dieu, Centre d'épidémiologie clinique, Paris, France</affiliation>
</author>
<author xml:id="author-0029">
<persName>
<forename type="first">Xavier</forename>
<surname>Mariette</surname>
</persName>
<affiliation>Service de Rhumatologie, Université Paris Sud, AP-HP, Hôpitaux Universitaires Paris Sud, Paris, France</affiliation>
</author>
<idno type="istex">52A3B80A2058E94C2AC1BECDAEDC9D33451FAAA8</idno>
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<idno type="DOI">10.1136/annrheumdis-2012-202293</idno>
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<idno type="article-id">annrheumdis-2012-202293</idno>
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<monogr>
<title level="j">Annals of the Rheumatic Diseases</title>
<title level="j" type="abbrev">Ann Rheum Dis</title>
<idno type="pISSN">0003-4967</idno>
<idno type="eISSN">1468-2060</idno>
<idno type="publisher-id">ard</idno>
<idno type="PublisherID-hwp">annrheumdis</idno>
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<publisher>BMJ Publishing Group Ltd and European League Against Rheumatism</publisher>
<date type="published" when="2013-06"></date>
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<date>2012-12-21</date>
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<abstract>
<p>Objectives To evaluate the efficacy and safety of rituximab in patients with primary Sjögren's syndrome (pSS). Methods The AutoImmune and Rituximab registry has included 86 patients with pSS treated with rituximab, prospectivey followed up every 6 months for 5 years. Results Seventy-eight patients with pSS (11 men, 67 women), who already had at least one follow-up visit, were analysed. Median age was 59.8 years (29–83), median duration of disease was 11.9 years (3–32). Indications for treatment were systemic involvement for 74 patients and only severe glandular involvement in four patients. The median European Sjögren's Syndrome disease activity index (ESSDAI) was 11 (2–31). 17 patients were concomitantly treated with another immunosuppressant agent. Median follow-up was 34.9 months (6–81.4) (226 patient-years). Overall efficacy according to the treating physician was observed in 47 patients (60%) after the first cycle of rituximab. Median ESSDAI decreased from 11 (2–31) to 7.5 (0–26) (p<0.0001). Median dosage of corticosteroid decreased from 17.6 mg/day (3–60) to 10.8 mg/day (p=0.1). Forty-one patients were retreated with rituximab. Four infusion reactions and one delayed serum sickness-like disease resulted in rituximab discontinuation. Three serious infections (1.3/100 patient-years) and two cancer-related deaths occurred. Conclusions In common practice, the use of rituximab in pSS is mostly restricted to patients with systemic involvement. This prospective study shows good efficacy and tolerance of rituximab in patients with pSS and systemic involvement.</p>
</abstract>
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<list>
<head>keywords</head>
<item>
<term>Sjøgren's Syndrome</term>
</item>
<item>
<term>Autoimmunity</term>
</item>
<item>
<term>Autoimmune Diseases</term>
</item>
</list>
</keywords>
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<change when="2012-12-21">Created</change>
<change when="2013-06">Published</change>
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<journal-meta>
<journal-id journal-id-type="hwp">annrheumdis</journal-id>
<journal-id journal-id-type="nlm-ta">Ann Rheum Dis</journal-id>
<journal-id journal-id-type="publisher-id">ard</journal-id>
<journal-title>Annals of the Rheumatic Diseases</journal-title>
<abbrev-journal-title abbrev-type="publisher">Ann Rheum Dis</abbrev-journal-title>
<abbrev-journal-title>Ann Rheum Dis</abbrev-journal-title>
<issn pub-type="ppub">0003-4967</issn>
<issn pub-type="epub">1468-2060</issn>
<publisher>
<publisher-name>BMJ Publishing Group Ltd and European League Against Rheumatism</publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="publisher-id">annrheumdis-2012-202293</article-id>
<article-id pub-id-type="doi">10.1136/annrheumdis-2012-202293</article-id>
<article-id pub-id-type="other">annrheumdis;72/6/1026</article-id>
<article-id pub-id-type="other">annrheumdis;annrheumdis-2012-202293</article-id>
<article-id pub-id-type="pmid">23264337</article-id>
<article-id pub-id-type="other">1026</article-id>
<article-id pub-id-type="other">annrheumdis-2012-202293</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Clinical and epidemiological research</subject>
</subj-group>
<series-title>Concise report</series-title>
</article-categories>
<title-group>
<article-title>Efficacy of rituximab in systemic manifestations of primary Sjögren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname>Gottenberg</surname>
<given-names>Jacques-Eric</given-names>
</name>
<xref ref-type="aff" rid="af1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Cinquetti</surname>
<given-names>Gael</given-names>
</name>
<xref ref-type="aff" rid="af2">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Larroche</surname>
<given-names>Claire</given-names>
</name>
<xref ref-type="aff" rid="af3">3</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Combe</surname>
<given-names>Bernard</given-names>
</name>
<xref ref-type="aff" rid="af4">4</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Hachulla</surname>
<given-names>Eric</given-names>
</name>
<xref ref-type="aff" rid="af5">5</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Meyer</surname>
<given-names>Olivier</given-names>
</name>
<xref ref-type="aff" rid="af6">6</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Pertuiset</surname>
<given-names>Edouard</given-names>
</name>
<xref ref-type="aff" rid="af7">7</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Kaplanski</surname>
<given-names>Guy</given-names>
</name>
<xref ref-type="aff" rid="af8">8</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Chiche</surname>
<given-names>Laurent</given-names>
</name>
<xref ref-type="aff" rid="af8">8</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Berthelot</surname>
<given-names>Jean-Marie</given-names>
</name>
<xref ref-type="aff" rid="af9">9</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Gombert</surname>
<given-names>Bruno</given-names>
</name>
<xref ref-type="aff" rid="af10">10</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Goupille</surname>
<given-names>Philippe</given-names>
</name>
<xref ref-type="aff" rid="af11">11</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Marcelli</surname>
<given-names>Christian</given-names>
</name>
<xref ref-type="aff" rid="af12">12</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Feuillet</surname>
<given-names>Séverine</given-names>
</name>
<xref ref-type="aff" rid="af13">13</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Leone</surname>
<given-names>Jean</given-names>
</name>
<xref ref-type="aff" rid="af14">14</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Sibilia</surname>
<given-names>Jean</given-names>
</name>
<xref ref-type="aff" rid="af1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Zarnitsky</surname>
<given-names>Charles</given-names>
</name>
<xref ref-type="aff" rid="af15">15</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Carli</surname>
<given-names>Philippe</given-names>
</name>
<xref ref-type="aff" rid="af16">16</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Rist</surname>
<given-names>Stephanie</given-names>
</name>
<xref ref-type="aff" rid="af17">17</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Gaudin</surname>
<given-names>Philippe</given-names>
</name>
<xref ref-type="aff" rid="af18">18</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Salliot</surname>
<given-names>Carine</given-names>
</name>
<xref ref-type="aff" rid="af17">17</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Piperno</surname>
<given-names>Muriel</given-names>
</name>
<xref ref-type="aff" rid="af19">19</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Deplas</surname>
<given-names>Adeline</given-names>
</name>
<xref ref-type="aff" rid="af20">20</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Breban</surname>
<given-names>Maxime</given-names>
</name>
<xref ref-type="aff" rid="af21">21</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Lequerre</surname>
<given-names>Thierry</given-names>
</name>
<xref ref-type="aff" rid="af22">22</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Richette</surname>
<given-names>Pascal</given-names>
</name>
<xref ref-type="aff" rid="af23">23</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Ghiringhelli</surname>
<given-names>Charles</given-names>
</name>
<xref ref-type="aff" rid="af24">24</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Hamidou</surname>
<given-names>Mohamed</given-names>
</name>
<xref ref-type="aff" rid="af25">25</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Ravaud</surname>
<given-names>Philippe</given-names>
</name>
<xref ref-type="aff" rid="af26">26</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Mariette</surname>
<given-names>Xavier</given-names>
</name>
<xref ref-type="aff" rid="af27">27</xref>
</contrib>
<contrib contrib-type="contributor">
<on-behalf-of>for the Club Rhumatismes et Inflammations and the French Society of Rheumatology</on-behalf-of>
</contrib>
</contrib-group>
<aff id="af1">
<label>1</label>
Service de Rhumatologie, Centre National de Références des Maladies Auto-Immunes Systémiques Rares, Hôpitaux Universitaires de Strasbourg, Immunorhumathologie Moléculaire, INSERM UMR S 1109, Université de Strasbourg, Strasbourg, France</aff>
<aff id="af2">
<label>2</label>
<addr-line>Service de Médecine Interne et Maladies Infectieuses</addr-line>
,
<institution>HIA Legouest</institution>
,
<addr-line>Metz</addr-line>
,
<country>France</country>
</aff>
<aff id="af3">
<label>3</label>
<addr-line>Service de Médecine interne</addr-line>
,
<institution>CHU Avicenne</institution>
,
<addr-line>Bobigny</addr-line>
,
<country>France</country>
</aff>
<aff id="af4">
<label>4</label>
<addr-line>Service d'Immunorhumathologie</addr-line>
,
<institution>CHU Montpellier</institution>
,
<addr-line>Montpellier</addr-line>
,
<country>France</country>
</aff>
<aff id="af5">
<label>5</label>
<addr-line>Service de Médecine interne</addr-line>
,
<institution>CHU Lille</institution>
,
<addr-line>Lille</addr-line>
,
<country>France</country>
</aff>
<aff id="af6">
<label>6</label>
<addr-line>Service de Rhumatologie</addr-line>
,
<institution>Hôpital Bichat</institution>
,
<addr-line>Paris</addr-line>
,
<country>France</country>
</aff>
<aff id="af7">
<label>7</label>
<addr-line>Service de Rhumatologie</addr-line>
,
<institution>CH Pontoise</institution>
,
<addr-line>Pontoise</addr-line>
,
<country>France</country>
</aff>
<aff id="af8">
<label>8</label>
<institution>Service de Médecine interne, Hôpital de la conception</institution>
,
<addr-line>Marseille</addr-line>
,
<country>France</country>
</aff>
<aff id="af9">
<label>9</label>
<addr-line>Service de Rhumatologie, INSERM U957</addr-line>
,
<institution>CHU Nantes</institution>
,
<addr-line>Nantes</addr-line>
,
<country>France</country>
</aff>
<aff id="af10">
<label>10</label>
<addr-line>Service de Médecine Polyvalente - Rhumatologie</addr-line>
,
<institution>CH de La Rochelle</institution>
,
<institution>La Rochelle</institution>
,
<country>France</country>
</aff>
<aff id="af11">
<label>11</label>
<addr-line>Service de Rhumatologie</addr-line>
,
<institution>CHU Tours</institution>
,
<addr-line>Tours</addr-line>
,
<country>France</country>
</aff>
<aff id="af12">
<label>12</label>
<addr-line>Service de Rhumatologie</addr-line>
,
<institution>CHU de Caen</institution>
,
<addr-line>Caen</addr-line>
,
<country>France</country>
</aff>
<aff id="af13">
<label>13</label>
<addr-line>Service de Pneumologie</addr-line>
,
<institution>Hôpital Saint Louis</institution>
,
<addr-line>Paris</addr-line>
,
<country>France</country>
</aff>
<aff id="af14">
<label>14</label>
<addr-line>Service de Médecine Interne</addr-line>
,
<institution>Hôpital Debré</institution>
,
<addr-line>Reims</addr-line>
,
<country>France</country>
</aff>
<aff id="af15">
<label>15</label>
<institution>Service de Rhumatologie, Groupe Hospitalier du Havre</institution>
,
<addr-line>Le Havre</addr-line>
,
<country>France</country>
</aff>
<aff id="af16">
<label>16</label>
<addr-line>Service de Médecine Interne</addr-line>
,
<institution>HIA Sainte-Anne</institution>
,
<addr-line>Toulon</addr-line>
,
<country>France</country>
</aff>
<aff id="af17">
<label>17</label>
<addr-line>Service de Rhumatologie</addr-line>
,
<institution>CHR Orléans</institution>
,
<addr-line>Orléans</addr-line>
,
<country>France</country>
</aff>
<aff id="af18">
<label>18</label>
<addr-line>Service de Rhumatologie</addr-line>
,
<institution>CHU Hôpital Sud, Grenoble</institution>
,
<addr-line>Echirolles</addr-line>
,
<country>France</country>
</aff>
<aff id="af19">
<label>19</label>
<addr-line>Service de Rhumatologie</addr-line>
,
<institution>CHU Lyon Sud France</institution>
, </aff>
<aff id="af20">
<label>20</label>
<addr-line>Service de Rhumatologie</addr-line>
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<institution>CH Niort</institution>
,
<country>Niort France</country>
</aff>
<aff id="af21">
<label>21</label>
Service de Rhumatologie, CHU Ambroise Paré, Boulogne Billancourt, France</aff>
<aff id="af22">
<label>22</label>
Service de Rhumatologie, CHU Rouen, Rouen, France</aff>
<aff id="af23">
<label>23</label>
Service de Rhumatologie, Hôpital Lariboisière, Paris, France</aff>
<aff id="af24">
<label>24</label>
Service de Médecine Interne, Hôpital Saint André, Bordeaux, France</aff>
<aff id="af25">
<label>25</label>
Service de Médecine Interne, CHU de Nantes, Nantes, France</aff>
<aff id="af26">
<label>26</label>
<addr-line>INSERM U738, Département d'Epidémiologie</addr-line>
,
<institution>Biostatistiques et Recherche Clinique, Hôtel Dieu, Centre d'épidémiologie clinique</institution>
,
<addr-line>Paris</addr-line>
,
<country>France</country>
</aff>
<aff id="af27">
<label>27</label>
<addr-line>Service de Rhumatologie, Université Paris Sud</addr-line>
,
<institution>AP-HP, Hôpitaux Universitaires Paris Sud</institution>
,
<addr-line>Paris</addr-line>
,
<country>France</country>
</aff>
<author-notes>
<corresp>
<label>Correspondence to</label>
Professor Jacques-Eric Gottenberg, ImmunoRhumatologie Moléculaire, Service de Rhumatologie, INSERM UMR_S 1109, Université de Strasbourg, Centre National de Référence des Maladies Auto-Immunes, Hôpital Hautepierre, 1 Avenue Molière, 67000 Strasbourg 67098, France; jacques-eric.gottenberg@chru-strasbourg.fr </corresp>
<fn>
<p>J-EG and GC contributed equally to this study.</p>
</fn>
</author-notes>
<pub-date pub-type="ppub">
<month>6</month>
<year>2013</year>
</pub-date>
<pub-date pub-type="epub-original">
<day>21</day>
<month>12</month>
<year>2012</year>
</pub-date>
<pub-date pub-type="epub">
<day>21</day>
<month>12</month>
<year>2012</year>
</pub-date>
<volume>72</volume>
<volume-id pub-id-type="other">72</volume-id>
<volume-id pub-id-type="other">72</volume-id>
<issue>6</issue>
<issue-id pub-id-type="other">annrheumdis;72/6</issue-id>
<issue-id pub-id-type="other">6</issue-id>
<issue-id pub-id-type="other">72/6</issue-id>
<fpage>1026</fpage>
<history>
<date date-type="received">
<day>2</day>
<month>7</month>
<year>2012</year>
</date>
<date date-type="rev-recd">
<day>12</day>
<month>10</month>
<year>2012</year>
</date>
<date date-type="accepted">
<day>18</day>
<month>11</month>
<year>2012</year>
</date>
</history>
<permissions>
<copyright-statement>Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions</copyright-statement>
<copyright-year>2013</copyright-year>
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<self-uri content-type="pdf" xlink:role="full-text" xlink:href="annrheumdis-72-1026.pdf"></self-uri>
<abstract>
<sec>
<title>Objectives</title>
<p>To evaluate the efficacy and safety of rituximab in patients with primary Sjögren's syndrome (pSS).</p>
</sec>
<sec>
<title>Methods</title>
<p>The AutoImmune and Rituximab registry has included 86 patients with pSS treated with rituximab, prospectivey followed up every 6 months for 5 years.</p>
</sec>
<sec>
<title>Results</title>
<p>Seventy-eight patients with pSS (11 men, 67 women), who already had at least one follow-up visit, were analysed. Median age was 59.8 years (29–83), median duration of disease was 11.9 years (3–32). Indications for treatment were systemic involvement for 74 patients and only severe glandular involvement in four patients. The median European Sjögren's Syndrome disease activity index (ESSDAI) was 11 (2–31). 17 patients were concomitantly treated with another immunosuppressant agent. Median follow-up was 34.9 months (6–81.4) (226 patient-years). Overall efficacy according to the treating physician was observed in 47 patients (60%) after the first cycle of rituximab. Median ESSDAI decreased from 11 (2–31) to 7.5 (0–26) (p<0.0001). Median dosage of corticosteroid decreased from 17.6 mg/day (3–60) to 10.8 mg/day (p=0.1). Forty-one patients were retreated with rituximab. Four infusion reactions and one delayed serum sickness-like disease resulted in rituximab discontinuation. Three serious infections (1.3/100 patient-years) and two cancer-related deaths occurred.</p>
</sec>
<sec>
<title>Conclusions</title>
<p>In common practice, the use of rituximab in pSS is mostly restricted to patients with systemic involvement. This prospective study shows good efficacy and tolerance of rituximab in patients with pSS and systemic involvement.</p>
</sec>
</abstract>
<kwd-group>
<kwd>Sjøgren's Syndrome</kwd>
<kwd>Autoimmunity</kwd>
<kwd>Autoimmune Diseases</kwd>
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</front>
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<name type="personal">
<namePart type="given">Christian</namePart>
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<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Séverine</namePart>
<namePart type="family">Feuillet</namePart>
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<roleTerm type="text">author</roleTerm>
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<namePart type="given">Jean</namePart>
<namePart type="family">Leone</namePart>
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<role>
<roleTerm type="text">author</roleTerm>
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<name type="personal">
<namePart type="given">Jean</namePart>
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<name type="personal">
<namePart type="given">Philippe</namePart>
<namePart type="family">Carli</namePart>
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<roleTerm type="text">author</roleTerm>
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<namePart type="given">Xavier</namePart>
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<abstract>Objectives To evaluate the efficacy and safety of rituximab in patients with primary Sjögren's syndrome (pSS). Methods The AutoImmune and Rituximab registry has included 86 patients with pSS treated with rituximab, prospectivey followed up every 6 months for 5 years. Results Seventy-eight patients with pSS (11 men, 67 women), who already had at least one follow-up visit, were analysed. Median age was 59.8 years (29–83), median duration of disease was 11.9 years (3–32). Indications for treatment were systemic involvement for 74 patients and only severe glandular involvement in four patients. The median European Sjögren's Syndrome disease activity index (ESSDAI) was 11 (2–31). 17 patients were concomitantly treated with another immunosuppressant agent. Median follow-up was 34.9 months (6–81.4) (226 patient-years). Overall efficacy according to the treating physician was observed in 47 patients (60%) after the first cycle of rituximab. Median ESSDAI decreased from 11 (2–31) to 7.5 (0–26) (p<0.0001). Median dosage of corticosteroid decreased from 17.6 mg/day (3–60) to 10.8 mg/day (p=0.1). Forty-one patients were retreated with rituximab. Four infusion reactions and one delayed serum sickness-like disease resulted in rituximab discontinuation. Three serious infections (1.3/100 patient-years) and two cancer-related deaths occurred. Conclusions In common practice, the use of rituximab in pSS is mostly restricted to patients with systemic involvement. This prospective study shows good efficacy and tolerance of rituximab in patients with pSS and systemic involvement.</abstract>
<note type="footnotes">J-EG and GC contributed equally to this study.</note>
<note type="author-notes">Correspondence to Professor Jacques-Eric Gottenberg, ImmunoRhumatologie Moléculaire, Service de Rhumatologie, INSERM UMR_S 1109, Université de Strasbourg, Centre National de Référence des Maladies Auto-Immunes, Hôpital Hautepierre, 1 Avenue Molière, 67000 Strasbourg 67098, France; jacques-eric.gottenberg@chru-strasbourg.fr</note>
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