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Rituximab in the treatment of refractory scleritis in patients with granulomatosis with polyangiitis (Wegener's).

AbstractPURPOSE:
The purpose of this descriptive study was to evaluate the clinical response to rituximab (RTX) in patients with scleritis due to granulomatosis with polyangiitis (GPA), in patients who had proved refractory to treatment with systemic glucocorticoids and immunosuppressive agents.
METHODS:
Retrospective analysis of interventional case series. Single referral center study. Eight patients (12 affected eyes) due to scleritis secondary to GPA, refractory to conventional treatment were included to receive RTX as therapy for remission induction. RTX was administered as a 1-g infusion every 2 weeks, for a total of 2 g. Patient follow-up included clinical evaluation (systemic and ophthalmologic), B-cell subset (CD19, CD20, CD22) counts, proteinase-3 anti-neutrophil cytoplasmic antibody (PR-3 ANCA), and Birmingham Vasculitis Activity Score for Wegener's granulomatosis (BVAS-WG). Outcomes were response to treatment and achievement of remission, as well as number of ocular relapses.
RESULTS:
The main indication for treatment was refractory necrotising anterior scleritis. Four weeks after completion of treatment with RTX, all patients showed clear clinical improvement, with no further progression. In all patients, an absolute depletion of B cells was confirmed in the first 6 weeks after treatment. Seven patients (87.5 %) achieved remission of inflammatory activity in 7 months or less. However, three patients experienced ocular relapse, which comprised reactivation of the anterior scleritis, uveitis, and posterior scleritis, and two patients required a second dose of RTX, with immediate improvement.
CONCLUSIONS:
RTX is useful in the treatment of refractory necrotising scleritis in patients with GPA. Of note, in those who relapse after remission, RTX can be successfully used for retreatment.
AuthorsClaudia Recillas-Gispert, Juan Carlos Serna-Ojeda, Luis Felipe Flores-Suárez
JournalGraefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie (Graefes Arch Clin Exp Ophthalmol) Vol. 253 Issue 12 Pg. 2279-84 (Dec 2015) ISSN: 1435-702X [Electronic] Germany
PMID26507398 (Publication Type: Journal Article)
Chemical References
  • Glucocorticoids
  • Immunologic Factors
  • Rituximab
  • Myeloblastin
Topics
  • Adult
  • B-Lymphocyte Subsets (immunology)
  • Drug Resistance
  • Female
  • Flow Cytometry
  • Glucocorticoids (therapeutic use)
  • Granulomatosis with Polyangiitis (complications, drug therapy, metabolism)
  • Humans
  • Immunologic Factors (therapeutic use)
  • Infusions, Intravenous
  • Male
  • Middle Aged
  • Myeloblastin (metabolism)
  • Retrospective Studies
  • Rituximab (therapeutic use)
  • Scleritis (drug therapy, etiology, metabolism)
  • Young Adult

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