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. 2021 Nov 12;12:20406207211046428. doi: 10.1177/20406207211046428

Table 4.

Bortezomib use in systemic autoimmune diseases.

Study type Autoimmune disease Study population N Comments References
Case report SLE and MM Adult (63 years) 1 • Bortezomib 1.5 mg/sqm on days 1, 4, 8 and 11 + steroids (28-day cycles) for three cycles
• Response: clinical improvement
• No toxicity
Fröhlich et al. 49
Case report SLE and APS in IgA MM Adult (70 years) 1 • Bortezomib 1.3 mg/sqm IV on days 1, 8, 15 and 22 (for four cycles) + dexamethasone
• Response: PR of MM and disappearance of SLE autoantibodies, then MM progression after 3 years
• Toxicity: G1 peripheral neuropathy
Quartuccio et al. 50
Case series Refractory SLE 20 • Bortezomib 1.3 mg/sqm IV in 21-day cycles + dexamethasone (N = 16) for a median of two cycles (range: 1–4)
• Response: clinical improvement (N = 20)
• Toxicity: G3-4 infections (N = 3), fever (N = 2), G1-2 nausea (N = 3), headache (N = 3), polyneuropathy (N = 2), allergic skin reactions (N = 2)
Alexander et al. 51
Case series SLE 8 • Bortezomib 1.3 mg/sqm for four injections in two cycles
• Response: gradual and significant depletion of short- and long-lived PCs in peripheral blood and BM by 50%, accompanied by a significant reduction of autoantibodies and total Ig
Alexander et al. 10
Case series Refractory lupus nephritis Adult (median age 27.8 years, range: 21–43) 5 • Bortezomib 1.3 mg/sqm IV on days 1, 4, 8 and 11 of 21-day cycles for four cycles + steroids
• Response: CR (N = 3), PR (N = 1), NR (N = 1)
• Toxicity: G1-2 thrombocytopenia (N = 5), gastrointestinal (N = 3), neurotoxicity (N = 3), transient elevated creatinine (N = 2), fever (N = 3), zoster (N = 1), hypertension (N = 1)
Zhang et al. 52
Case series Resistant lupus nephritis 12 • Bortezomib for a median of six (range: 5–7) weekly cycles
• Response: CR (N = 1), PR (N = 10), NR (N = 1)
• Toxicity: G2 polyneuropathy (N = 2)
Segarra et al. 53
Case report Refractory primary Sjögren syndrome Adult (44 years) 1 • Bortezomib 1.3 mg/sqm on days 1, 4, 8, 11, 22, 29, 36, 43, 50 and 57
• Response: clinical improvement
• No toxicity
Jakez-Ocampo et al. 54
Case series RA and MM Adult (78, 72 and 60 years) 3 • Bortezomib 1.3 mg/sqm IV on days 1, 4, 8 and 11 + steroids for eight cycles for two patients, three cycles for one patient
• Response: improvement of joint disease
• No toxicity
Lassoued et al. 55

APS, antiphospholipid syndrome; BM, bone marrow; CR, complete remission; Ig, immunoglobulins; IV, intravenous; MM, multiple myeloma; NR, non-response; PC, plasma cell; PR, partial remission; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus.