BMJ Open Ophthalmology (Sep 2024)

Cellular pattern and orbital fat involvement are possible risk factors for the failure of corticosteroids in patients with pure idiopathic orbital inflammation syndrome: lessons from the French prospective SIOI cohort study (part II)

  • ,
  • David Saadoun,
  • Eric Vicaut,
  • Valérie Touitou,
  • Jacques Lagier,
  • Frédéric Mouriaux,
  • Pierre-Yves Robert,
  • Antoine Rousseau,
  • Nicolas Schleinitz,
  • Jean-Marie Michot,
  • Loic Guillevin,
  • Matthieu Groh,
  • Antoine Martin,
  • Philippe Blanche,
  • Sébastien Abad,
  • Robin Dhote,
  • Felix Ackermann,
  • Boris Bienvenu,
  • Olivier Galatoire,
  • Sophie Coffin-Pichonnet,
  • Pierre Aucouturier,
  • Françoise Heran,
  • Bertrand Vabres,
  • Ambre La Rosa,
  • Stéphane Tran Ba,
  • Nahla Cucherousset,
  • Neila Sedira,
  • Emmanuel Héron,
  • Aïcha Abbas,
  • Mboup Bassirou,
  • Isabelle Badelon,
  • Mathieu Zmuda,
  • Alain Ducasse,
  • Isabelle Larré,
  • Amélie Brabant-Viau,
  • Jean Luc George,
  • Jean Maalouf,
  • Anne-Laure Fisch,
  • Nathalie Tieulé,
  • Juliette Delmas,
  • Alexandre Vallon,
  • Natahlie Massart,
  • Marie Alexandra Alyanakian,
  • Pierre Olivier Schischmanoff,
  • Nabila Pizzi,
  • Nathalie Kingue-Elessa,
  • Jad Abou Ghantous,
  • Solohaja-Faniaha Dimby

DOI
https://doi.org/10.1136/bmjophth-2024-001663
Journal volume & issue
Vol. 9, no. 1

Abstract

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Purpose To better characterise the effects of corticosteroids on the course of pure idiopathic orbital inflammation syndrome (pIOIS).Methods This was a national, multicentre, prospective, non-interventional cohort study (SIOI). Among the 35 patients with histologically proven orbital inflammation who had previously been studied for their IgG4 immunostaining status, we selected those with a negative IgG4 status (ie, pIOIS) who received corticosteroids as single first-line treatment. Clinical, morphological and pathological findings at diagnosis and during follow-up from treatment initiation to study completion were analysed. Patients were assessed for their response to prednisone after the 24-month prospective phase in terms of remission (≤10 mg/d) or failure (>10 mg/d). Daily standard doses of prednisone (DSDP) were calculated at different time-points and compared between response groups.Results Of the 17 patients with pIOIS included in the final analysis, two-thirds received corticosteroids only. DSDP (mg/kg-day) were significantly higher at the time of failure in eight patients (47%) than in nine (53%) remitting at M24 (0.16 vs 0.045; p: 0.03). Notably, patients with pIOIS with a cellular pattern or orbital fat involvement tended to receive higher daily corticosteroid doses in the event of failure than remission (0.16 vs 0.045 and 0.12 vs 0.042, respectively). During treatment, maximal DSDP was 0.52 in failed patients.Conclusion The highest corticosteroid doses were insufficient to prevent failure in patients with pIOIS, particularly in those with a cellular pattern or orbital fat involvement. Large-scale interventional studies are now necessary to clarify prognostic factors and optimise corticosteroid management in patients with pIOIS.