ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2485

Orbital Pseudotumor in Granulomatosis with Polyangiitis. Interim Analysis of Clinical Outcomes from a European Collaborative Observational Study

Luca Moroni1, Gabriele Gallina2, Marco Lanzillotta2, Sara Maggioni2, Giovanni Benanti2, Adriana Cariddi2, Paolo Delvino3, Sara Monti4, Alessandra Milanesi5, Filippo Fagni6, Marco Fornaro7, Camille Taille8, Elena Treppo9, Grégory Pugnet10, Emanuele Chiara11, Roberto Padoan12, Luca Iorio13, Federica Davanzo12, Eleonora Fiorin14, Alvise Berti15, Roberto Bortolotti16, Sergey Moiseev17, Pavel Novikov17, Askin Ates18, Ahmet Omma19, Tahir Saygin Ogut20, Gozde Sevgi Kart-Bayram21, Tuba Demirci22, Riza Can Kardas23, Ömer Karadağ21, Marco Matucci-Cerinic24 and Lorenzo Dagna25, 1IRCCS San Raffaele Scientific Institute, Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Milano, Italy, Milano, Italy, 2IRCCS Ospedale San Raffaele, Milano, Italy, 3University of Milano-Bicocca, Milan, Milan, Italy, 4IRCCS Istituto Auxologico Italiano, Milan, Italy, 5Unit of Rheumatology, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy, 6Department of Internal Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, Deutsches Zentrum für Immuntherapie (DZI), Friedrich Alexander University Erlangen-Nuremberg and Universitätsklinikum Erlangen, Erlangen, Germany, Erlangen, Germany, 7Università di Bari, Bari, Italy, 8Bichat Hospital, Paris, France, 9Division of Rheumatology, Department of Medicine (DMED), University of Udine, Udine, Italy, Udine, Italy, 10Toulouse Rangueil University Hospital, Toulouse, France, 11Ospedale Careggi, Firenze, Italy, 12Azienda Ospedaliera-Universitaria di Padova, Padova, Italy, 13Rheumatology Unit, Department of Medicine DIMED, University of Padua, Padova, Italy, Padova, Italy, 14Azienda Ospedaliera-Universitaria di Padova, Padova, 15Rheumatology Unit Santa Chiara Hospital of Trento, Trento, Italy, 16Rheumatology Unit Santa Chiara Hospital of Trento, Trento, 17Sechenov First Moscow State Medical University, Moscow, Russia, 18Ankara University, Ankara, Turkey, 19Ankara Bilkent City Hospital, Ankara, Turkey, 20Akdeniz University, Antalya, Turkey, 21Hacettepe University School of Medicine, Ankara, Turkey, 22Dokuz Eylül University, Izmir, Turkey, 23Gazi University, Ankara, Turkey, 24University San Raffaele Milano, Milano, Milan, Italy, 25Ospedale San Raffaele, Milano, Italy

Meeting: ACR Convergence 2024

Keywords: ,

Session Information

Date: Monday, November 18, 2024

Title: Vasculitis – ANCA-Associated Poster III

Session Type: Poster Session C

Session Time: 10:30AM-12:30PM

Background/Purpose: Orbital Pseudotumor (OPT) is a rare organ-threatening manifestation of Granulomatosis with Polyangiitis (GPA), with severe prognosis and historically refractory to most treatments. This is an interim report from the STOP-GPA study, a collaborative European study on difficult-to-treat manifestations of GPA.  

Methods: This retrospective, multicentre study included OPT patients who fulfilled 2022 ACR/EULAR criteria for GPA, diagnosed between 1980 and 2023. OPT relapse was defined as worsening symptoms or dimensional increase of orbital inflammatory tissue at imaging. Chi-square, student’s t-test or Mann-Whitney tests were used to compare categorical and continuous variables respectively. We compared clinical variables and treatments between relapsing and non-relapsing patients. Time to relapse (TTR) was evaluated by survival analysis and Breslow test.

Results: The study included 61 patients (mean age at GPA diagnosis: 43 years, BVAS: 10), with positive ANCA in 44 (72%). ANCA positivity is associated with relapse (85% vs 63%, p=0.04) with a trend towards a stronger association with anti-PR3 (Table 1). Relapse is also associated with sight loss (46% in relapsers vs. 20% in non-relapsers, p=0.03).

Twenty-eight patients (46%) received glucocorticoid pulses as part of induction treatment with apparently no impact on relapsing behaviour of the disease over time. Metothrexate is the only agent significantly associated with a reduced relapse risk if used in the induction phase (10%, p=0.02), followed by rituximab (26%, p=0.08).  

When used as a maintenance drug, RTX is associated with the lowest relapse risk (19%, p=0.03), while azathioprine is associated with the worst disease course (relapse rate 72%, p=0.03).

Moreover, during the maintenance phase with a median follow-up of 87 months (IQR 32-177 months), RTX significantly prolongs the time to relapse as displayed in Figure 1.  

Conclusion: This preliminary analysis of the STOP-GPA study demonstrates that relapses in OPT are strongly associated with sight loss. ANCA positivity, and particularly anti-PR3 antibodies may detect patients at risk for relapse. In absence of higher level evidence, and waiting for more detailed and complete results, we suggest that combination treatment with RTX and MTX can be considered for induction and maintenance in patients with GPA-related OPT in order to avoid irreversible organ damage.  

Supporting image 1

Clinical characteristics of the OPT cohort sorted by relapsing behavior

Supporting image 2

Relapse-free survival in patients receiving RTX-based vs. other maintenance treatment regimens

Disclosures: L. Moroni: AstraZeneca, 1, 2, GlaxoSmithKlein(GSK), 1, 2; G. Gallina: None; M. Lanzillotta: Amgen Inc., 12, MITIGATE Committee Member; S. Maggioni: None; G. Benanti: None; A. Cariddi: None; P. Delvino: GlaxoSmithKlein(GSK), 6; S. Monti: AstraZeneca, 6, GlaxoSmithKlein(GSK), 6; A. Milanesi: None; F. Fagni: None; M. Fornaro: None; C. Taille: None; E. Treppo: None; G. Pugnet: None; E. Chiara: None; R. Padoan: None; L. Iorio: None; F. Davanzo: None; E. Fiorin: None; A. Berti: None; R. Bortolotti: None; S. Moiseev: None; P. Novikov: None; A. Ates: None; A. Omma: None; T. Ogut: None; G. Kart-Bayram: None; T. Demirci: None; R. Kardas: None; Ö. Karadağ: None; M. Matucci-Cerinic: None; L. Dagna: None.

To cite this abstract in AMA style:

Moroni L, Gallina G, Lanzillotta M, Maggioni S, Benanti G, Cariddi A, Delvino P, Monti S, Milanesi A, Fagni F, Fornaro M, Taille C, Treppo E, Pugnet G, Chiara E, Padoan R, Iorio L, Davanzo F, Fiorin E, Berti A, Bortolotti R, Moiseev S, Novikov P, Ates A, Omma A, Ogut T, Kart-Bayram G, Demirci T, Kardas R, Karadağ Ö, Matucci-Cerinic M, Dagna L. Orbital Pseudotumor in Granulomatosis with Polyangiitis. Interim Analysis of Clinical Outcomes from a European Collaborative Observational Study [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/orbital-pseudotumor-in-granulomatosis-with-polyangiitis-interim-analysis-of-clinical-outcomes-from-a-european-collaborative-observational-study/. Accessed .

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