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

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%)

Other^b

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%)

0^c

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%)