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Table 2 Clinical characteristics of non-infectious orbital inflammation cases treated with Rituximab

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

Identified Cause
of Orbital Inflammation
Number
(Percentage)
Age
(years)
Gender
(M:F)
Anatomical
Localization
(Ratio, %)
Time from Diagnosis to Rituximab Treatment
(months)
Rituximab
Regimen
(Ratio, %)
Positive
Therapeutic
Response
(Ratio, %)
Recurrence
(Ratio, %)
Adverse Events
(Ratio, %)
Granulomatosis with Polyangiitis 99 (59.3%) Mean: 42.7 ± 15.6
Median: 42
Range: 4–72
0.89:1 Unspecified Orbital mass/granuloma (64/99, 64.6%);
Mass affecting one orbital structure (2/99, 2.0%);
Mass affecting three or more structures (1/99, 1.0%);
Unspecified orbital inflammation (31/99, 31.3%);
Inflammation affecting one orbital structure (1/99, 1.0%)
Involved orbital structures when reported:
Lacrimal gland (2/4, 50.0%);
Extraocular muscles (2/4, 50.0%);
Preseptal/soft tissues (2/4, 50.0%)
Mean: 59.4 ± 73.8
Median: 30.8
Range: 0–240
Rheumatologic (51/96, 53.1%);
Oncologic (42/96, 43.8%);
Other (3/96, 3.1%)
1 treatment cycle: (50/79, 63.3%);
2 treatment cycles: (18/79, 22.8%);
> 2 treatment cycles: (11/79, 13.9%)
range: 1–8 cycles
84/99, 84.8% 23/74, 31.1% None (32/38, 84.2%);
Exacerbation of disease (3/38, 7.9%);
De novo hepatitis (1/38, 2.6%);
Nausea (1/38, 2.6%);
Adenovirus pneumonitis (1/38, 2.6%)a
IgG4-related disease 36 (21.6%) Mean: 54.8 ± 14.7
Median: 54
Range: 12–83
1 to 0.80 Mass affecting one orbital structure (5/36, 13.9%);
Mass affecting two orbital structures (4/36, 11.1%);
Unspecified orbital inflammation (3/36, 8.3%);
Inflammation affecting one orbital structure (7/36, 19.4%);
Inflammation affecting two orbital structures (11/36, 30.6%);
Inflammation affecting three or more orbital structures (6/36, 16.7%)
Involved orbital structures when reported:
Lacrimal gland (23/33, 69.7%);
Extraocular muscles (16/33, 48.5%);
Preseptal/soft tissues (18/33, 54.5%)
Bone (3/33, 9.1%)
Mean: 51.8 ± 88.5
Median: 20.5
Range: 0–360
Rheumatologic (11/19, 57.9%);
Oncologic (4/19, 21.1%);
Other (4/19, 21.1%)
1 treatment cycle: (10/19, 52.6%);
2 treatment cycles: (2/19, 10.5%);
> 2 treatment cycles: (7/19, 36.8%)
range: 1–19 cycles
33/35, 94.3% 11/28, 39.3% None (15/17, 88.2%);
Fatigue (1/17, 5.9%);
Orbital discomfort with infusion
(1/17, 5.9%)
IgG4-related disease and Adult orbital xanthogranulomatous disease 4 (2.4%) Mean: 51.0
Median: 53.5
Range: 36–61
1 to 0.33 Inflammation affecting one orbital structure (1/4, 25.0%)
Inflammation affecting two orbital structures (1/4, 25.0%)
Inflammation affecting three or more orbital structures (2/4, 50.0%)
Involved orbital structures when reported:
Lacrimal gland (3/4, 75.0%);
Extraocular muscles (2/4, 50.0%);
Preseptal/soft tissues (4/4, 100%)
NR Rheumatologic (3/3, 100%)
1 cycle: (2/4, 50.0%)
2 cycles: (2/4, 50.0%)
4/4, 100% 0/4, 0% None (1/1, 100%)
Otherb 3 (1.8%) Mean: 53.7
Median: 51
Range: 49–61
0.50:1 Unspecified orbital inflammation (1/3, 33.3%)
Orbital inflammation affecting two orbital structures (2/3, 33.3%)
Involved orbital structures when reported:
Lacrimal gland (2/2, 100%)
Extraocular muscles (2/2, 100%)
0c Rheumatologic (2/3, 66.7%);
Other (1/3, 33.3%)
1 treatment cycle: (1/3, 33.3%);
2 treatment cycles: (1/3, 33.3%);
> 2 treatment cycles: (1/3, 33.3%)
3/3, 100% 0/3, 0% None (1/1100%)
Indeterminate 25 (15.0%) Mean: 48.0 ± 19.4
Median: 46
Range: 7–86
0.44:1 Unspecified Orbital mass/granuloma (6/25, 24.0%);
Mass affecting one orbital structure (4/25, 16.0%);
Mass affecting two orbital structures (2/25, 8.0%);
Unspecified orbital inflammation (7/25, 28.0%);
Inflammation affecting one orbital structure (3/25, 12.0%)
Inflammation affecting two orbital structures (1/25, 4.0%)
Inflammation affecting three or more orbital structures (2/25, 8.0%)
Involved orbital structures when reported:
Lacrimal gland (9/12, 75.0%);
Extraocular muscles (5/12, 41.7%);
Preseptal/soft tissues (4/12, 33.3%)
Bone (1/12, 8.3%)
Mean: 9.9 ± 10.8
Median: 5.0
Range: 1–30
Rheumatologic (12/23, 52.2%);
Oncologic (6/23, 26.1%);
4 weekly 10 mg intraorbital injections (3/23, 13.0%);
Other (2/23, 8.7%);
1 treatment cycle: (16/23, 69.6%);
2 treatment cycles: (4/23, 17.4%);
> 2 treatment cycles: (2/23, 8.7%);
range: 1–6 cycles
22/25, 88.0% 6/19, 31.6% None (6/9, 66.7%);
Interstitial pneumonitis (1/9, 11.1%);
Exacerbation of orbital disease
(1/9, 11.1%);
Itching and breathlessness
following infusion (1/9, 11.1%)
  1. M male, F female, NR not reported, Rheumatologic Two doses of 1000 mg separated by 14 days, Oncologic four doses of 375 mg/m2 weekly, Other all other RTX dosing regimens
  2. a = patient died from adenovirus pneumonitis
  3. b = includes one case each of systemic lupus erythematosus, concurrent IgG4-related disease and GPA, and necrobiotic xanthogranuloma
  4. c = 2/2 patients received RTX as first line therapy