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

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%)