Rituximab in the treatment of refractory scleritis in patients with granulomatosis with polyangiitis (Wegener's).
OBJECTIVE
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.
Ophthalmology, Instituto Nacional de Ciencias Medicas y Nutrición "Salvador Zubirán", Mexico City, Mexico.
Claudia
C
Recillas-Gispert
Instituto de Oftalmologia "Conde de Valenciana", Mexico City, Mexico.
Juan Carlos
JC
Serna-Ojeda
Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Calzada de Tlalpan 4502. Col. Sección XVI, C.P. 14080, Mexico City, Mexico. felipe98@prodigy.net.mx.
Luis Felipe
http://orcid.org/0000-0002-0172-8023
LF
Flores-Suárez
D000328:Adult; D016175:B-Lymphocyte Subsets / Q000276:immunology; D004351:Drug Resistance; D005260:Female; D005434:Flow Cytometry; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin / Q000378:metabolism; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use*; D015423:Scleritis / Q000188:drug therapy* / Q000209:etiology / Q000378:metabolism; D055815:Young Adult
D005938:Glucocorticoids; D007155:Immunologic Factors; D000069283:Rituximab; D053146:Myeloblastin
Granulomatosis with polyangiitis; Refractory; Rituximab; Scleritis; Treatment