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

Tzaribachev, Nikolay; Koetter, Ina; Kuemmerle-Deschner, Jasmin B; Schedel, Joerg

doi:10.4076/1757-1626-2-6609

Cases Journal

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

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
**2^nd rituximab course
***3^rd 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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