Table 1

Patients' characteristics and concomitant medication

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


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.

Tzaribachev et al. Cases Journal 2009 2:6609   doi:10.4076/1757-1626-2-6609

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