Overview
SIDEC - (Subcutaneous Immunoglobulin in De-novo CIDP) ia a study designed as a randomized, parallel study with an open-label extension phase. The aims are to compare the effect of SCIG and IVIG in 60 treatment-naïve CIDP patients, and to detect the lowest effective dosage for maintenance treatment.
Description
In fase I the patients are followed for 26 weeks on a fixed dose of 0.54 g/kg/week in the SCIG group (total 14 g/kg) and 2 g/kg/4week in the IVIG group (total 14 g/kg). The patients automatically continue in fase II in which treatment is reduced every 12 weeks (90%, 75%, 50%, 25% and 0%) over a course 60 weeks. The patients are evaluated at every visit with overall disability sum score (ODSS), grip strength, medical research council score (MRC-score), INCAT-Sensory Sum Score (ISS), 10-meter-walk test (10-MWT), 6-spot-step test (6-SST), 9-hole-peg test (9-HPT), quality of life (EQ-5D-5L), Fatigue Severity Scale (FSS), Neuropathic Pain Symptom Inventory (NPSI), Rasch built overall disability scale (RODS) and Life Quality Index (LQI) and blood samples.
Eligibility
Inclusion Criteria:
- Fulfilling EFNS/PNS criteria for definite, probable or pure motor CIDP.
- No previous treatment with IVIG or SCIG.
- Age ≥ 18.
- ODSS ≥ 2 - either (arm/leg): 1/1, 2/0 or 0/2 at the time of inclusion.
Clinical criteria for typical CIDP
- Chronically progressive, stepwise, or recurrent symmetric proximal and distal weakness and sensory dysfunction of all extremities, developing over at least 2 months; cranial nerves may be affected.
- Absent or reduced tendon reflexes in all extremities.
Criteria for pure motor CIDP • Pure motor affection; otherwise as for typical CIDP.
Electrophysiological criteria for CIDP
- Motor distal latency prolongation ≥50% above ULN in two nerves (excluding median neuropathy at the wrist from carpal tunnel syndrome), or
- Reduction of motor conduction velocity ≥30% below LLN in two nerves, or
- Prolongation of F-wave latency ≥30% above ULN in two nerves (≥50% if amplitude of distal negative peak CMAP ≤80% of LLN values), or
- Absence of F-waves in two nerves of these nerves have distal negative peak CMAP amplitudes ≥20% of LLN + ≥1 other demyelinating parameter in ≥1 other nerve, or
- Partial motor conduction block: ≥50% amplitude reduction of the proximal negative peak CMAP relative to distal, if distal negative peak CMAP >20% of LLN, in two nerves, or in one nerve + ≥1 other demyelinating parameter in ≥1 other nerve, or
- Abnormal dispersion (≥30% duration increase between the proximal and distal negative peak CMAP) in ≥2 nerves, or
- Distal CMAP duration (interval between onset of the first negative peak an return to baseline of the last negative peak) increase in ≥1 nerve (median ≥6.6 ms, ulnar ≥6.7 ms, peroneal ≥7.6 ms, tibial ≥8.8 ms) + ≥1 other demyelinating parameter in ≥1 other nerve
Electrophysiological criteria for probable CIDP
(a) ≥30% amplitude reduction of the proximal negative peak CMAP relative to distal,
excluding the posterior tibial nerve, if distal negative peak CMAP ≥20% of LLN, in two
nerves, or in one nerve + ≥1 other demyelinating parameter in ≥1 other nerve
Exclusion Criteria:
- Other causes of neuropathy
- Increased risk of thromboembolism
- Pregnancy (Plasma HCG is tested at inclusion in all fertile women)
- Breast feeding
- Malignancy
- Severe medical disease
- Other immune modulating treatment than low dose steroid (prednisolon < 25 mg daily)
within the last 6 months prior to inclusion
- Hepatitis B or C or HIV infection (screening at inclusion)
- Known IgA deficiency
- Known allergy to consents in PRIVIGEN or HIZENTRA
- Body weight > 120 kg
After treatment initiation:
- Pregnancy
- Serious medical disease that affects treatment or examinations
- Non-compliance to treatment
- Initiation of other immune modulating therapy
- Unacceptable side effects
- Withdrawal of consent to participate (drop-out)