Long-term Outcomes of Rituximab Therapy in Ocular Granulomatosis with Polyangiitis: Impact on Localized and Nonlocalized Disease.
OBJECTIVE
To evaluate the long-term outcomes of rituximab (RTX) treatment in patients with ocular granulomatosis with polyangiitis (GPA) with localized or generalized disease.


METHODS
Retrospective cohort.


METHODS
Thirty-seven patients with ocular GPA receiving RTX in a multidisciplinary vasculitis clinic between 2004 and 2013.


METHODS
A total of 100 patients who received a course of RTX were identified, and notes were reviewed. Baseline demographic details, clinical characteristics (including organ involvement), drugs used, and outcome measures were recorded.


METHODS
The percentage in remission (inactive disease with prednisolone ≤7.5 mg with or without maintenance treatment) at 6 months, time to remission, percentage relapsing, side effects, B-cell count, antineutrophil cytoplasm antibody titers, induction, and maintenance regimens.


RESULTS
The median follow-up time after the first RTX course was 36.5 months. Twenty patients had scleritis, and 17 patients had orbital disease; 86% achieved remission at 6 months. The percentage in remission versus partial remission was not statistically significant between patients with scleritis and patients with orbital disease (85% vs. 15% with scleritis and 82% vs. 18% with orbital disease; P = 1.00). The percentage relapsing was not statistically significant (P = 0.33) between scleritis (60%) and orbital disease (41%). Localized disease (ocular ± ear-nose-throat/lung) was observed in 57%, and generalized disease (ocular plus other organs) was observed in 43%, the former having a median duration of disease of 40 months. There was no statistically significant difference (P = 0.37) in the percentage in remission between localized and generalized ocular disease. Relapses occurred in 51%, with localized disease being a significant risk factor for relapse. Fifty percent of patients with generalized disease versus none with localized disease received cyclophosphamide (CYP) as part of the induction regimen. Patients who received CYP during induction had significantly (P = 0.027) lower ratios of baseline 12-month proteinase 3 titers than patients who did not have CYP. Infections were observed in 16% of patients, with 8% requiring hospital admission.


CONCLUSIONS
Our long-term data suggest that RTX is effective for inducing disease remission in localized and generalized ocular GPA. Localized disease is a significant risk factor for relapse, which may be related to less use of CYP in the induction regimen.
UCL Institute of Ophthalmology and Moorfields Eye Hospital, London, United Kingdom; Multidisciplinary Vasculitis Clinic, Imperial College Healthcare NHS Trust, London, United Kingdom.
Lavnish
L
Joshi
Multidisciplinary Vasculitis Clinic, Imperial College Healthcare NHS Trust, London, United Kingdom; Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, Hammersmith Hospital, London, United Kingdom.
Anisha
A
Tanna
Multidisciplinary Vasculitis Clinic, Imperial College Healthcare NHS Trust, London, United Kingdom; Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, Hammersmith Hospital, London, United Kingdom.
Stephen P
SP
McAdoo
Multidisciplinary Vasculitis Clinic, Imperial College Healthcare NHS Trust, London, United Kingdom; Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, Hammersmith Hospital, London, United Kingdom.
Nicholas
N
Medjeral-Thomas
Multidisciplinary Vasculitis Clinic, Imperial College Healthcare NHS Trust, London, United Kingdom; Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, Hammersmith Hospital, London, United Kingdom.
Simon R J
SR
Taylor
ENT Department, Imperial College Healthcare NHS Trust, Charing Cross Hospital, London, United Kingdom.
Gurpreet
G
Sandhu
Multidisciplinary Vasculitis Clinic, Imperial College Healthcare NHS Trust, London, United Kingdom; Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, Hammersmith Hospital, London, United Kingdom.
Ruth M
RM
Tarzi
Multidisciplinary Vasculitis Clinic, Imperial College Healthcare NHS Trust, London, United Kingdom; Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, Hammersmith Hospital, London, United Kingdom.
Charles D
CD
Pusey
UCL Institute of Ophthalmology and Moorfields Eye Hospital, London, United Kingdom; Multidisciplinary Vasculitis Clinic, Imperial College Healthcare NHS Trust, London, United Kingdom. Electronic address: s.lightman@ucl.ac.uk.
Sue
S
Lightman
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D001402:B-Lymphocytes / Q000276:immunology; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D018655:Lymphocyte Count; D008297:Male; D008875:Middle Aged; D016727:Orbital Pseudotumor / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D012008:Recurrence; D012189:Retrospective Studies; D000069283:Rituximab; D015423:Scleritis / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D016896:Treatment Outcome
D019268:Antibodies, Antineutrophil Cytoplasmic; D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide