title: Abatacept in the treatment of severe, longstanding, and refractory uveitis associated with juvenile idiopathic arthritis.
abstract: OBJECTIVE
  Abatacept (ABA), a selective T cell costimulation modulator that binds to CD80 and CD86 on antigen-presenting cells, was investigated for its antiinflammatory effect in treating severe chronic uveitis associated with juvenile idiopathic arthritis (JIA).
  
  
  METHODS
  Our retrospective study was conducted by members of the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC). Patients with JIA who are receiving ABA treatment for active uveitis were included. In all patients, uveitis had been refractory to previous topical and systemic corticosteroids, immunosuppressives, and at least 1 tumor necrosis factor-α inhibitor. A standardized protocol was used to document uveitis (MIWGUC) and arthritis. Baseline visit and visits at 3, 6, 9, and 12 months before and after ABA start were evaluated. Primary outcome measure was defined as achievement of uveitis inactivity; secondary outcome measures were tapering of corticosteroid and/or immunosuppressive treatment, and occurrence of complications.
  
  
  RESULTS
  In all, 21 patients (16 female) with active uveitis (n = 21) and arthritis (n = 18) were included (mean age 11.8 ± 3.6 yrs). In 7 of 18 patients with active arthritis at baseline, inactivity was achieved following ABA treatment. Uveitis inactivity was achieved in 11 patients, but recurred later in 8 of them, and remained active in another 10 cases. Systemic corticosteroids or immunosuppression were tapered in 3 patients, but uveitis recurred in all of them during further followup. Ocular complications secondary to uveitis were present in 17 patients at baseline, while 3 patients developed new ocular complications during followup.
  
  
  CONCLUSIONS
  A sustained response to ABA was uncommon in patients with severe and refractory uveitis.
authors: From the Department of Ophthalmology, Inselspital, University of Bern, Bern, Switzerland; Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen, and Department of Ophthalmology, and Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine, Interdisciplinary Uveitis Center, University of Heidelberg, Heidelberg, Germany; Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, and Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University, Milan, Italy; Department of Ophthalmology, Hôpital Pitié-Salpêtrière, and Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Paris, France; Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.C. Tappeiner, MD, Department of Ophthalmology, Inselspital, University of Bern, and Department of Ophthalmology, St. Franziskus Hospital, Münster; E. Miserocchi, MD, Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele; B. Bodaghi, MD, PhD, Department of Ophthalmology, Hôpital Pitié-Salpêtrière; K. Kotaniemi, MD, PhD, Department of Ophthalmology, Helsinki University Hospital; F. Mackensen, MD, Department of Ophthalmology and Interdisciplinary Uveitis Center, University of Heidelberg; V. Gerloni, MD, Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University;P. Quartier, MD, Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital; T. Lutz, MD, Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine and Interdisciplinary Uveitis Center, University of Heidelberg; A. Heiligenhaus, MD, Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen.
authors: Christoph
authors: C
authors: Tappeiner
authors: From the Department of Ophthalmology, Inselspital, University of Bern, Bern, Switzerland; Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen, and Department of Ophthalmology, and Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine, Interdisciplinary Uveitis Center, University of Heidelberg, Heidelberg, Germany; Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, and Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University, Milan, Italy; Department of Ophthalmology, Hôpital Pitié-Salpêtrière, and Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Paris, France; Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.C. Tappeiner, MD, Department of Ophthalmology, Inselspital, University of Bern, and Department of Ophthalmology, St. Franziskus Hospital, Münster; E. Miserocchi, MD, Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele; B. Bodaghi, MD, PhD, Department of Ophthalmology, Hôpital Pitié-Salpêtrière; K. Kotaniemi, MD, PhD, Department of Ophthalmology, Helsinki University Hospital; F. Mackensen, MD, Department of Ophthalmology and Interdisciplinary Uveitis Center, University of Heidelberg; V. Gerloni, MD, Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University;P. Quartier, MD, Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital; T. Lutz, MD, Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine and Interdisciplinary Uveitis Center, University of Heidelberg; A. Heiligenhaus, MD, Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen.
authors: Elisabetta
authors: E
authors: Miserocchi
authors: From the Department of Ophthalmology, Inselspital, University of Bern, Bern, Switzerland; Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen, and Department of Ophthalmology, and Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine, Interdisciplinary Uveitis Center, University of Heidelberg, Heidelberg, Germany; Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, and Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University, Milan, Italy; Department of Ophthalmology, Hôpital Pitié-Salpêtrière, and Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Paris, France; Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.C. Tappeiner, MD, Department of Ophthalmology, Inselspital, University of Bern, and Department of Ophthalmology, St. Franziskus Hospital, Münster; E. Miserocchi, MD, Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele; B. Bodaghi, MD, PhD, Department of Ophthalmology, Hôpital Pitié-Salpêtrière; K. Kotaniemi, MD, PhD, Department of Ophthalmology, Helsinki University Hospital; F. Mackensen, MD, Department of Ophthalmology and Interdisciplinary Uveitis Center, University of Heidelberg; V. Gerloni, MD, Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University;P. Quartier, MD, Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital; T. Lutz, MD, Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine and Interdisciplinary Uveitis Center, University of Heidelberg; A. Heiligenhaus, MD, Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen.
authors: Bahram
authors: B
authors: Bodaghi
authors: From the Department of Ophthalmology, Inselspital, University of Bern, Bern, Switzerland; Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen, and Department of Ophthalmology, and Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine, Interdisciplinary Uveitis Center, University of Heidelberg, Heidelberg, Germany; Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, and Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University, Milan, Italy; Department of Ophthalmology, Hôpital Pitié-Salpêtrière, and Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Paris, France; Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.C. Tappeiner, MD, Department of Ophthalmology, Inselspital, University of Bern, and Department of Ophthalmology, St. Franziskus Hospital, Münster; E. Miserocchi, MD, Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele; B. Bodaghi, MD, PhD, Department of Ophthalmology, Hôpital Pitié-Salpêtrière; K. Kotaniemi, MD, PhD, Department of Ophthalmology, Helsinki University Hospital; F. Mackensen, MD, Department of Ophthalmology and Interdisciplinary Uveitis Center, University of Heidelberg; V. Gerloni, MD, Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University;P. Quartier, MD, Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital; T. Lutz, MD, Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine and Interdisciplinary Uveitis Center, University of Heidelberg; A. Heiligenhaus, MD, Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen.
authors: Kaisu
authors: K
authors: Kotaniemi
authors: From the Department of Ophthalmology, Inselspital, University of Bern, Bern, Switzerland; Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen, and Department of Ophthalmology, and Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine, Interdisciplinary Uveitis Center, University of Heidelberg, Heidelberg, Germany; Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, and Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University, Milan, Italy; Department of Ophthalmology, Hôpital Pitié-Salpêtrière, and Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Paris, France; Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.C. Tappeiner, MD, Department of Ophthalmology, Inselspital, University of Bern, and Department of Ophthalmology, St. Franziskus Hospital, Münster; E. Miserocchi, MD, Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele; B. Bodaghi, MD, PhD, Department of Ophthalmology, Hôpital Pitié-Salpêtrière; K. Kotaniemi, MD, PhD, Department of Ophthalmology, Helsinki University Hospital; F. Mackensen, MD, Department of Ophthalmology and Interdisciplinary Uveitis Center, University of Heidelberg; V. Gerloni, MD, Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University;P. Quartier, MD, Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital; T. Lutz, MD, Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine and Interdisciplinary Uveitis Center, University of Heidelberg; A. Heiligenhaus, MD, Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen.
authors: Friederike
authors: F
authors: Mackensen
authors: From the Department of Ophthalmology, Inselspital, University of Bern, Bern, Switzerland; Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen, and Department of Ophthalmology, and Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine, Interdisciplinary Uveitis Center, University of Heidelberg, Heidelberg, Germany; Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, and Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University, Milan, Italy; Department of Ophthalmology, Hôpital Pitié-Salpêtrière, and Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Paris, France; Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.C. Tappeiner, MD, Department of Ophthalmology, Inselspital, University of Bern, and Department of Ophthalmology, St. Franziskus Hospital, Münster; E. Miserocchi, MD, Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele; B. Bodaghi, MD, PhD, Department of Ophthalmology, Hôpital Pitié-Salpêtrière; K. Kotaniemi, MD, PhD, Department of Ophthalmology, Helsinki University Hospital; F. Mackensen, MD, Department of Ophthalmology and Interdisciplinary Uveitis Center, University of Heidelberg; V. Gerloni, MD, Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University;P. Quartier, MD, Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital; T. Lutz, MD, Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine and Interdisciplinary Uveitis Center, University of Heidelberg; A. Heiligenhaus, MD, Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen.
authors: Valeria
authors: V
authors: Gerloni
authors: From the Department of Ophthalmology, Inselspital, University of Bern, Bern, Switzerland; Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen, and Department of Ophthalmology, and Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine, Interdisciplinary Uveitis Center, University of Heidelberg, Heidelberg, Germany; Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, and Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University, Milan, Italy; Department of Ophthalmology, Hôpital Pitié-Salpêtrière, and Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Paris, France; Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.C. Tappeiner, MD, Department of Ophthalmology, Inselspital, University of Bern, and Department of Ophthalmology, St. Franziskus Hospital, Münster; E. Miserocchi, MD, Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele; B. Bodaghi, MD, PhD, Department of Ophthalmology, Hôpital Pitié-Salpêtrière; K. Kotaniemi, MD, PhD, Department of Ophthalmology, Helsinki University Hospital; F. Mackensen, MD, Department of Ophthalmology and Interdisciplinary Uveitis Center, University of Heidelberg; V. Gerloni, MD, Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University;P. Quartier, MD, Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital; T. Lutz, MD, Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine and Interdisciplinary Uveitis Center, University of Heidelberg; A. Heiligenhaus, MD, Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen.
authors: Pierre
authors: P
authors: Quartier
authors: From the Department of Ophthalmology, Inselspital, University of Bern, Bern, Switzerland; Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen, and Department of Ophthalmology, and Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine, Interdisciplinary Uveitis Center, University of Heidelberg, Heidelberg, Germany; Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, and Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University, Milan, Italy; Department of Ophthalmology, Hôpital Pitié-Salpêtrière, and Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Paris, France; Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.C. Tappeiner, MD, Department of Ophthalmology, Inselspital, University of Bern, and Department of Ophthalmology, St. Franziskus Hospital, Münster; E. Miserocchi, MD, Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele; B. Bodaghi, MD, PhD, Department of Ophthalmology, Hôpital Pitié-Salpêtrière; K. Kotaniemi, MD, PhD, Department of Ophthalmology, Helsinki University Hospital; F. Mackensen, MD, Department of Ophthalmology and Interdisciplinary Uveitis Center, University of Heidelberg; V. Gerloni, MD, Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University;P. Quartier, MD, Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital; T. Lutz, MD, Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine and Interdisciplinary Uveitis Center, University of Heidelberg; A. Heiligenhaus, MD, Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen.
authors: Thomas
authors: T
authors: Lutz
authors: From the Department of Ophthalmology, Inselspital, University of Bern, Bern, Switzerland; Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen, and Department of Ophthalmology, and Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine, Interdisciplinary Uveitis Center, University of Heidelberg, Heidelberg, Germany; Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, and Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University, Milan, Italy; Department of Ophthalmology, Hôpital Pitié-Salpêtrière, and Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Paris, France; Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.C. Tappeiner, MD, Department of Ophthalmology, Inselspital, University of Bern, and Department of Ophthalmology, St. Franziskus Hospital, Münster; E. Miserocchi, MD, Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele; B. Bodaghi, MD, PhD, Department of Ophthalmology, Hôpital Pitié-Salpêtrière; K. Kotaniemi, MD, PhD, Department of Ophthalmology, Helsinki University Hospital; F. Mackensen, MD, Department of Ophthalmology and Interdisciplinary Uveitis Center, University of Heidelberg; V. Gerloni, MD, Department of Rheumatology, Pediatric Rheumatology Unit, Istituto Ortopedico G. Pini University;P. Quartier, MD, Paris-Descartes University, IMAGINE and Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital; T. Lutz, MD, Department of General Pediatrics, Centre for Pediatric and Adolescent Medicine and Interdisciplinary Uveitis Center, University of Heidelberg; A. Heiligenhaus, MD, Department of Ophthalmology, St. Franziskus Hospital, Münster, and University Duisburg-Essen. arnd.heiligenhaus@uveitis-zentrum.de.
authors: Arnd
authors: A
authors: Heiligenhaus
mesh_terms: D000069594:Abatacept / Q000627:therapeutic use*; D000293:Adolescent; D001171:Arthritis, Juvenile / Q000150:complications*; D002648:Child; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome; D014605:Uveitis / Q000150:complications / Q000188:drug therapy*
chemical_list: D007166:Immunosuppressive Agents; D000069594:Abatacept
keywords: ABATACEPT; BIOLOGICALS; JUVENILE IDIOPATHIC ARTHRITIS; UVEITIS