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Table 1 Rituximab use in refractory non-infectious orbital inflammation - summary of comprehensive literature review

From: Rituximab for treatment of non-infectious and non-malignant orbital inflammatory disease

Number of Studies 55
Total Patients 167
Age
(years)
Mean 48.0±16.5
Median 49
Range 4-86
Gender 0.89:1 (M:F)
Ocular Condition Treated with Rituximab
(Ratio, %)
Unspecified Orbital mass/granuloma (71/167, 42.5%);
Mass affecting one orbital structure (10/167, 6.0%);
Mass affecting two orbital structures (7/167, 4.2%);
Mass affecting three or more structures (1/167, 0.6%);
Unspecified orbital inflammation (42/167, 25.1%);
inflammation affecting one orbital structure (12/167, 7.2%);
inflammation affecting two orbital structures (14/167, 8.4%);
inflammation affecting three or more orbital structures (10/167, 6.0%)
Involved orbital structures when reported:
Lacrimal gland (39/54, 72.2%);
Extraocular muscles (27/54, 50.0%);
Preseptal/soft tissues (28/54, 51.9%);
Bone (4/54, 7.4%)
Underlying Systemic Condition
(Ratio, %)
GPA (99/167, 59.3%) [8, 9, 14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33];
IgG4-related disease (36/167, 21.6%) [34,35,36,37,38,39,40,41,42,43,44,45,46,47,48];
Idiopathic orbital inflammation (20/167, 12.0%) [18, 23, 34, 49,50,51,52,53,54,55,56,57];
Idiopathic sclerosing orbital inflammation (5/167, 3.0%) [58,59,60];
IgG4-related disease and AOXGD (4/167, 2.4%) [61,62,63,64];
AOXGD (1/167, 0.6%) [65];
GPA and IgG4-related disease (1/167, 0.6%) [66];
SLE (1/167, 0.6%) [67]
Treatment Prior to Rituximab
(Ratio, %)
None (14/122, 11.5%);
Corticosteroid only (27/122, 22.1%);
1 Steroid sparing agent (31/122, 25.4%);
2 Steroid sparing agents (25/122, 20.5%);
≥3 Steroid sparing agents (25/122, 20.5%)
Previously Utilized Immunosuppressants
(Ratio, %)
Corticosteroid (92/119, 77.3%);
Cyclophosphamide (49/119, 41.2%);
Methotrexate (44/119, 37.0%);
Azathioprine (34/119, 28.6%);
Mycophenolate mofetil (20/119, 16.8%);
Infliximab (7/119, 5.9%);
TNF-inhibitor NOS (6/119, 5.0%);
Cyclosporine (3/119, 2.5%);
Adalimumab (2/119, 1.7%);
Etanercept (2/119, 1.7%);
Chlorambucil (1/119, 0.8%);
IV immunoglobulin (1/119, 0.8%);
Leflunomide (1/119, 0.8%);
Tamoxifen (1/119, 0.8%);
Indomethacin (1/119, 0.8%);
Vedolizumab (1/119, 0.8%)
Line of Therapy
(Ratio, %)
First line (14/149, 9.4%);
Second line (27/149, 18.1%);
Third line or greater (108/149, 72.5%)
Treatment Regimen and Number of Cycles
(Ratio, %)
Rheumatologic (80/144, 55.6%);
Oncologic (51/144, 35.4%);
Other (13/144, 9.0%)
1 treatment cycle: (79/128, 61.7%);
2 treatment cycles: (28/128, 21.9%);
≥3 treatment cycles: (21/128, 16.4%)
Types of Responses
(Ratio, %)
Responsive (146/166, 88.0%)
- Disease Remission (93/146, 63.7%)
- Author report: (53/146, 36.3%)
Nonresponsive (20/166, 12.0%)
Incidence of Recurrence
(Ratio, %)
38/126, 30.2%
Adverse Events
(Ratio, %)
None (55/66, 83.3%);
Exacerbation of orbital disease (4/66, 6.1%);
Pneumonitis (2/66, 3.0%)a;
De novo hepatitis B (1/66, 1.5%);
Nausea (1/66, 1.5%);
Orbital discomfort with infusion (1/66, 1.5%);
Itching and breathlessness with infusion (1/66, 1.5%);
Fatigue (1/66, 1.5%)
  1. RTX rituximab, M male, F female, GPA granulomatosis with polyangiitis, AOXGD adult onset xanthogranulomatous disease, NOS not otherwise specified, Rheumatologic Two doses of 1000 mg separated by 14 days, Oncologic four doses of 375 mg/m2 weekly, Other all other RTX dosing regimens, TNF tumor necrosis factor, First line RTX initiated before or as same time as corticosteroids, Second line RTX initiated after corticosteroids, Third line RTX initiated after corticosteroids and another agent, such as nonbiologic or biologic disease modifying antirheumatic drug, anti-cancer medications, or intravenous immunoglobulins
  2. aone patient died from adenovirus pneumonitis