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Table 1 Patients' characteristics and concomitant medication

From: Rituximab for the treatment of refractory pediatric autoimmune diseases: a case series

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]
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
  1. 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
  2. *1st rituximab course
  3. **2nd rituximab course
  4. ***3rd rituximab course
  5. 1 Rituximab was applied after the lymphoma protocol: 4 × 375 mg/m2 (two of the patients received single doses as well)
  6. 2 the second plasmapheresis could not be accomplished
  7. 3 B-cells were measured by fluorescence activated cell sorting (FACS) analysis
  8. Conditioning protocol prior to ASCT included: fludarabine 120 mg/m2, cyclophosphamide 120 mg/m2 and muromonab-CD3-10 mg.
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Cases Journal

ISSN: 1757-1626