Rituximab as a monotherapy or in combination therapy for the treatment of non-paraneoplastic autoimmune retinopathy

Figures & data

Table 1 Non-paraneoplastic autoimmune retinopathy treatment regimens

Patient no/sex/age	Treatment regimen	Duration of treatment (months)	Reason for treatment termination	Total duration of follow-up (months)
1/F/30	R + C + B	10	Side effect to R, C, and B	17
2/F/47	R + C	18	Continued to the last visit	18
3/F/51	R	20	Continued to the last visit	20
4/F/73	R	11	Insurance issue	49
5/M/54	R + C	18	Side effect to C	18
6/F/40	R + B	8	Continued to the last visit	8

Abbreviations: B, bortezomib; C, cyclophosphamide; F, female; M, male; R, rituximab.

Figure 1 Changes in vision, visual field parameters, and electroretinography parameters from the initial visit to the most recent visit in 12 eyes of six patients with non-paraneoplastic autoimmune retinopathy. (A) Changes in LogMAR visual acuity. (B) Mean deviation changes on Humphrey visual field. (C) Pattern standard deviation changes on Humphrey visual field. (D) Changes in scotopic rod response amplitude on Ganzfeld electroretinography. (E) Changes in maximal response a-wave amplitude on Ganzfeld electroretinography. (F) Changes in maximal response b-wave amplitude on Ganzfeld electroretinography. (G) Changes in 30-Hz flicker amplitude on Ganzfeld electroretinography. (H) Changes in 30-Hz flicker implicit time on Ganzfeld electroretinography. The central box shows the 25th–75th percentile. The middle line represents the median. Vertical line extends from the minimum to the maximum. Separate triangles show far out values (larger than the upper quartile plus three times the interquartile range). The solid line connects the median values.

Abbreviation: logMAR, logarithm of the minimum angle of resolution.

Table 2 Visual acuity, visual field parameters, and electroretinography parameters changes from the initial visit to the most recent visit in patients with non-paraneoplastic autoimmune retinopathy

Patient no/sex/age/eye	Changea in V/A	Changea in HVF, MD	Changea in HVF, PSD	Changea in HVF, TD	Changea in HVF, PD	Changea in ERG, Sc b-wave	Changea in ERG, C a-wave	Changea in ERG, C b-wave	Changea in ERG, 30-Hz Am	Changea in ERG, 30-Hz Im	Negative wave-forms
1/F/30/OD	W	S	S	I	S	W	W	I	W	S	No
1/F/30/OS	W	S	S	S	S	W	W	W	W	W	No
2/F/47/OD	S	S	S	S	S	W	S	I	I	S	Yes
2/F/47/OS	W	S	S	S	I	I	W	I	I	W	Yes
3/F/51/OD	S	W	S	W	W	S	W	S	I	I	No
3/F/51/OS	S	S	S	W	W	I	S	S	W	S	Yes
4/F/73/OD	W	S	S	S	S	W	W	W	S	S	No
4/F/73/OS	S	S	S	S	S	W	W	W	W	I	No
5/M/54/OD	S	I	S	I	I	I	S	S	W	I	No
5/M/54/OS	S	S	W	S	W	I	I	S	W	I	No
6/F/40/OD	S	S	S	W	W	S	S	S	I	I	No
6/F/40/OS	S	I	I	I	I	S	W	W	I	I	No

Note:

a Change from the initial visit to the most recent visit.

Abbreviations: Am, amplitude; C, combined; ERG, electroretinography; F, female; HVF, Humphrey visual field; I, improved; Im, implicit time; M, male; MD, mean deviation; OD, right eye; OS, left eye; PD, pattern deviation; PSD, pattern standard deviation; S, stable; Sc, Scotopic; TD, total deviation; V/A, visual acuity; W, worse.

Figure 2 Changes in visual field parameters (improvement in the mean deviation and stability of pattern standard deviation) in one of the patients from the initial visit before starting rituximab (upper panel) to the last visit (lower panel).

Abbreviations: MD, mean deviation; PSD, pattern standard deviation.

Figure 3 Changes in 30-Hz flicker electroretinography parameters (improvement in the Amp and IT) in one of the patients from the initial visit before starting rituximab (upper panel) to the last visit (lower panel).

Abbreviations: Amp, amplitude; IT, implicit time.

Table 3 Changes in visual acuity, HVF and electroretinography as single tests modalities from the initial visit to the most recent visit in patients with non-paraneoplastic autoimmune retinopathy

Patient no/sex/age/eye	Changea in V/A	Changea in HVF	Changea in ERG
1/F/30/OD	W	S	W
1/F/30/OS	W	S	W
2/F/47/OD	S	S	S/I
2/F/47/OS	W	S	I
3/F/51/OD	S	W	S/I
3/F/51/OS	S	S/W	S
4/F/73/OD	W	S	W
4/F/73/OS	S	S	W
5/M/54/OD	S	I	S/I
5/M/54/OS	S	S/W	I
6/F/40/OD	S	S/W	S
6/F/40/OS	S	I	S/I

Note:

a Change from the initial visit to the most recent visit.

Abbreviations: ERG, electroretinography; F, female; HVF, Humphrey visual field; I, improved; M, male; OD, right eye; OS, left eye; S, stable; S/I, stable/improved; S/W, stable/worse; V/A, visual acuity; W, worse.

Table 4 Anti-retinal and anti-optic nerve autoantibody bands in non-paraneoplastic autoimmune retinopathy

Patient no/sex/age	ARA bands (pathologic), first visit (kDa)	ARA bands (pathologic), last visit (kDa)	ARA bands (nonspecific), first visit (kDa)	ARA bands (nonspecific), last visit (kDa)	AOA bands (pathologic), first visit (kDa)	AOA bands (pathologic), last visit (kDa)	AOA bands (nonspecific), first visit (kDa)	AOA bands (nonspecific), last visit (kDa)
1/F/30	30, 46, 48	30, 45	31, 43, 62, 122	43, 60, 62, 112	N	N	46, 62, 64	N
2/F/47	22	22	N	N	22	22	N	131, 132, 135
3/F/51	40, 46	46	N	N	N	N	46, 50, 62	36, 62
4/F/73	33–35	N	N	N	N	N	2, 104, 110	62
5/M/54	30, 46	30, 46	50, 72	72	N	N	46, 72	72
6/F/40	N	N	62, 76	62, 76	N	N	32, 96, 136	135

Notes: 22, ARA and AOA pathologic; 30, carbonic anhydrase II; 33–35, Muller cell associated; 40, aldolase; 46, enolase; 48, arrestin.

Abbreviations: AOA, anti-optic nerve antibody; ARA, anti-retinal antibody; F, female; M, male; N, negative.