Subcutaneous Immunoglobulin in De-novo CIDP (SIDEC)

Korábbi kezelés

• Other immune modulating treatment than low dose steroid (prednisolon \< 25 mg daily) within the last 6 months prior to inclusion
• Initiation of other immune modulating therapy

Más betegség

• Increased risk of thromboembolism
• Malignancy
• Severe medical disease
• Hepatitis B or C or HIV infection (screening at inclusion)
• Known allergy to consents in PRIVIGEN or HIZENTRA
• Serious medical disease that affects treatment or examinations

Egyéb

• Other causes of neuropathy
• Pregnancy (Plasma HCG is tested at inclusion in all fertile women)
• Breast feeding
• Known IgA deficiency
• Body weight \> 120 kg
  • After treatment initiation:
• Pregnancy
• Non-compliance to treatment
• Unacceptable side effects
• Withdrawal of consent to participate (drop-out)

• 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