Table 1.
Clinical features and treatment regimens for individual patients receiving ofatumumab
| Characteristics | Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | Case 6 | Case 7 | Case 8 |
|---|---|---|---|---|---|---|---|---|
| Age, years | 25 | 69 | 53 | 51 | 77 | 78 | 21 | 43 |
| Gender | F | M | F | F | M | M | M | M |
| Ethnicity | Caucasian | Caucasian | North African | Caucasian | South Asian | Caucasian | South Asian | Caucasian |
| Clinical diagnosis | GPA | MPA | EGPA | EGPA | MPA | GPA | GPA | MPA |
| ANCA IIF | cANCA ++ | pANCA+ | Negative | cANCA ++ | Negative | cANCA ++ | cANCA ++ | pANCA ++ |
| ANCA ELISA specificity | PR3 | Negative | Negative | PR3 | Negative | PR3 | PR3 | MPO |
| ANCA ELISA titre (NR <25) | 252 | Negative | Negative | 139 | Negative | 573 | 962 | 215 |
| Treatment indication | Relapse | Relapse | Relapse | Remission maintenance | New disease | New disease | New disease | New disease |
| For relapsing/remission maintenance patients | ||||||||
| Duration of AAV, years | 10 | 47 | 5 | 15 | — | — | — | — |
| Previous relapses | 3 | 1 | 5 | 3 | — | — | — | — |
| Major | 2 | 0 | 0 | 2 | ||||
| Minor | 1 | 1 | 5 | 1 | ||||
| Previous cumulative CYC, g | 3 | 0 | 0 | 10 | — | — | — | — |
| Previous cumulative RTX, g | 2 | 0 | 0 | 2 | — | — | — | — |
| Other previous treatments | CS | CS only | — | — | — | — | ||
| AZA | CS | CS | ||||||
| MMF | AZA | AZA | ||||||
| MTX | MMF | |||||||
| HCQ | ||||||||
| Time since last RTX (BCC at this presentation, cells/µl) | 62 months (175) | — | — | 15 months (38) | — | — | — | — |
| Organ Involvement at this Presentation | Renal | Eosinophilia, rising ANCA titre | Renal | Renal | Renal | |||
| ENT | Renal | ENT | Lung | ENT | ENT | Renal | ||
| Skin | Skin | Lung | Neuro | Lung | Lung | ENT | ||
| MSK | MSK | Neuro | GI | Hepatic | MSK | |||
| BVAS at this presentation | 19 | 16 | 10 | 0 | 26 | 24 | 23 | 18 |
| Treatment | ||||||||
| Ofatumumab dose (i.v.), mg | 2 × 700 | 2 × 700 | 2 × 700 | 2 × 700 | 2 × 700 | 2 × 700 | 2 × 700 | 2 × 700 |
| CYC dose (i.v.), g | 3.0 | 3.2 | — | — | 1.0 | 3.1 | 3.2 | 3.6 |
| Methylprednisolone dose (i.v.), mg | 1000 | — | — | — | — | 500 | — | — |
| Initial prednisolone, dose (p.o.), mg | 40 | 60 | 30 | 5 | 60 | 60 | 60 | 60 |
| PEX, n | — | — | — | — | — | 7 | — | 7 |
| Maintenance | MMF | MMF | AZA | MMF | MMF | AZA | AZA | AZA |
AAV: ANCA-associated vasculitis; ANCA: antineutrophil cytoplasm antibody; BCC: B cell count; BVAS: Birmingham vasculitis activity score; EGPA: eosinophilic granulomatosis with polyangiitis; ELISA: enzyme-linked immunosorbent assay; GI: gastrointestinal; GPA: granulomatosis with polyangiitis; IIF: indirect immunofluorescence; MPA: microscopic polyangiitis; MSK: musculoskeletal; NR: normal range; PEX: plasma exchange; RTX: rituximab.