Table. Characteristics of Patients With Neuromyelitis Optica Spectrum Disorder Receiving Bortezomib Therapy.

Characteristic	Patient 1	Patient 2	Patient 3	Patient 4	Patient 5
Before BTZ treatment
Age at disease onset, y	41	27	43	36	43
Age at BTZ initiation, y	42	43	43	37	53
Anti-AQP4-ab	+	+	+	+	+
Duration of disease at BTZ initiation, y	1.7	16.5	0.4	1.8	10.9
Relapses, No.	5	14	2	4	15
Coexisting autoimmune disease	None	None	None	Myasthenia gravis	Sjögren syndrome
Coexisting autoantibodies	ANA, SSA	ANA, SSA, Ro-52, RF, and nRNP	ANA	ANA and AChR	ANA, SSA, Ro-52, and SSB
EDSS score at BTZ initiation	6.5	5.5	6.0	2.5	5.0
VAS score at BTZ initiation	6.0	6.0	6.5	4.0	7.0
Attacks in the year before BTZ treatment, No.	3 (1 attack of corpus callosum, 1 of ON, and 1 of LETM)	3 (1 attack of brain stem attack, 1 of ON, and 1 of LETM)	2 (1 attack of ON and 1 of LETM)	3 (2 attacks of LETM and1 of ON)	3 (2 Attacks of ON and 1 brain stem and subcortical attack)
Attack prevention 2 y before BTZ treatment	Prednisone,15-20 mg/d; rituximab to deplete B cell countsa	IV cyclophosphamide, 0.4 g/w, total 10.0 g; prednisone, 15 mg/d; AZA, 3 mg/kg/d	Prednisone, 15 mg/d; AZA, 3 mg/kg/d	Prednisone,15-20 mg/d; AZA, 3 mg/kg/d	Prednisone, 20-30 mg/d; AZA, 3 mg/kg/d; rituximab to deplete B cell countsa
Responses to prior therapies 2 y before BTZ treatment	Poor; 1 attack experienced while using rituximab	Poor; 1 attack experienced even while using a combination of prednisone and AZA	Poor; 1 attack experienced when using a combination of prednisone and AZA (unable to tolerate AZA)b	Poor; 2 attacks experienced when combination of prednisone and AZA, but unable to tolerate AZAb	Poor; 2 attacks experienced while using rituximab, or 2 relapses experienced while using a combination of prednisone and AZA
Peripheral CD19+ B cells, counts/μLc	230	270	374	194	156
Peripheral CD138+ plasma cells, counts/μL	6	11	6	7	14
1-Year follow-up after initial BTZ treatment
Attacks, No.	1 (Myelitis)	0	0	0	0
EDSS score	3.5	4.0	1.0	0.5	3.5
VAS score	3.5	3.0	2.0	2.5	4.0
Peripheral CD19+ B cells, counts/μL	41	25	211	105	16
Peripheral CD138+ plasma cells, counts/μL	2	0	3	2	1
Attack prevention after discontinuation of BTZ	Prednisone, 15 mg/d	AZA, 3 mg/kg/d	Prednisone, 15 mg/d	Prednisone, 15 mg/d	Prednisone, 20 mg/d
BTZ-related adverse effects	Headache, common cold	Headache, enterocolitis	Macula-papular rash at the injection site	Common cold	Macula-papular rash at the injection site

Abbreviations: AChR+, antiacetylcholine receptor antibody; ANA, anti-nuclear antibody; AQP4-ab, antiaquaporin-4 autoantibody; AZA, azathioprine; BTZ, bortezomib; CD, cluster of differentiation; EDSS, Expanded Disability Status Scale; IV, intravenous; LETM, longitudinal extensive transverse myelitis; nRNP, antinuclear ribonucleoprotein antibody; ON, optica neuritis; RF, rheumatic factor; Ro-52, anti-Ro-52 antibody; SSA, anti–Sjögren syndrome A antibody; SSB, anti–Sjögren syndrome B antibody; VAS, visual analog scale.

^aThe patient had ever received rituximab treatment, total 400 mg/cycle, 2 cycles to ensure that CD19⁺ and CD27⁺ memory B cells were less than 0.05% of the peripheral blood mononuclear cell count.

^bThe reason for discontinuation of AZA treatment was hepatic dysfunction.

^cBefore receiving a bortezomib infusion, the peripheral blood CD19⁺ B cells counts of all the 5 patients were in the normal range.