Table 1 |
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Patients' characteristics and concomitant medication |
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Patient/Gender |
Diagnosis |
Age at Disease Onset [years] |
Disease Symptoms |
Disease Duration before Treatment [years] |
Medication before Rituximab treatment |
Rituximab Indication |
Rituximab Dosis [mg/m]2 |
Rituximab Courses [4 doses/course] |
Time to Return of peripheral B-cells3 |
Time to Relapse |
AE/SAE |
Follow-up Time [years] |
|
|
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1. female |
JDM |
14 |
heliotropic rash Gottron's patcnes muscul. weakness contractures difficult breathing difficult swallowing diffjcult speach |
1 |
MP(10 pulses) P MTX IVIG (10 pulses) CSA |
progressive disease despite therapy |
375 |
2 |
*11 months **progress without B-cells |
*3 months **3 months |
none/none |
2 |
|
2. male |
WG |
10 |
episcleritis arthralgia cough skin ulcers fever weight loss |
1 |
MP (2 pulses) CYC (6 pulses) P MTX AZA |
relapsing disease despite therapy |
375 |
1 |
5 months |
no relapse |
none/none |
2 |
|
3. male |
SLE |
7 |
malar rash glomerulonephrilis CNS vasculitis |
2 |
AZA MP (? pulses) CYC plasmapheresis |
severe CNS involvement |
375 |
1 single dose |
no data |
no relapse |
none/none |
6 |
|
4. female |
SLE WG |
13 for SLE 14 for MG |
thrombosis (DVTJ fever sweating fatigue weight loss malar rash loss of strength diplopic images |
4 |
P, AZA CYC (6 pulses) MP (6 pulses) MMF Pyridostigmine plasmapheresis1 NSAlDs |
*relapsing MG despite therapy **disease flare before ASCT ***disease flare after ASCT |
375 |
3 |
*6 months **no B-ceii retum[dis.flare without B-cells) ***2 months |
*response clinically insignificant **no response followed by ASCT ***no relapse |
mild bronchitis/none |
1.5 |
|
5. female |
MS SLE |
3 for MS 17 for SLE |
opticus neuritis hemiparesis malar rash arthritis |
1.5 |
MP (multiple pulses) intraocular Steroids CYC (12 pulses) (β-lnterferon AZA MMF NSAlDs |
*relapsing/progressive SLE nephritis despite therapy **autoimmune anemia/thrombo cytopenia after ASCT |
375 |
2 2 single doses |
*12 months (including time after ASCT) **no B-cell depletion after 2 single doses |
*partial to nituximab but development of glomerulonephritis **partial response, repeatedly low erythrocyties/thrombocytes |
none/none patient's death due to Evans syndrome/disease recurrence |
1 |
|
|
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JDM, juvenile dermatomyositis; WG, Wegener's granulomatosis; SLE, systemic lupus erythematodes; MG, myasthenia gravis; MS, multiple sclerosis; CNS, central nervous system; ASCT, autologous stem cell transplantation; MP, methylprednisolone (dosing regimen: 30 mg/kg-max. 1 g/day for 3 days); P, prednisolone (dosing regimen: 2 mg/kg/day); IVIG, iv immunoglobulins (dosing regimen: 1 g/kg/day for 3 days); CSA, cyclosporine A (dosing regimen: 3 mg/kg/day); MTX, methotrexate (iv-dosing regimen: 1 mg/kg/week; sc-dosing regimen: 15 mg/kg/week); AZA, azathioprine (dosing regimen: 3 mg/kg/day); CYC, cyclophosphamide (dosing regimen: iv 750 mg/m2 /every 4 weeks); MMF, mycophenolate mofetil (dosing regimen 1 - 3 g/day-depending on MMF blood levels); NSAIDs, non-steroidal anti-inflammatory drugs; AE, adverse event; SAE, serious adverse event *1st rituximab course **2nd rituximab course ***3rd rituximab course 1 Rituximab was applied after the lymphoma protocol: 4 × 375 mg/m2 (two of the patients received single doses as well) 2 the second plasmapheresis could not be accomplished 3 B-cells were measured by fluorescence activated cell sorting (FACS) analysis Conditioning protocol prior to ASCT included: fludarabine 120 mg/m2, cyclophosphamide 120 mg/m2 and muromonab-CD3-10 mg. |
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Tzaribachev et al. Cases Journal 2009 2:6609 doi:10.4076/1757-1626-2-6609 |
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