Improvements from baseline to end-point were also recorded for grip strength in the dominant hand (treatment difference 10·9 kPa; P = 0·0008) and the non-dominant Silmitasertib price hand (8·6 kPa; P = 0·005). Results were similar during the second cross-over period. During the extension phase, participants who continued to receive IVIG had

a longer time to relapse than did patients treated with placebo (P = 0·011). This is the first study that demonstrates clearly the long-term efficacy and tolerability of IVIG in CIDP. Another recent, multi-centre, randomized, double-blind, placebo controlled, parallel-group study in 45 patients with CIDP compared the efficacy and tolerability of IVIG (0·5 g/kg/day for 4 consecutive days) to intravenous methylprednisolone (0·5 g/day for 4 consecutive days) given every month for 6 months [37]. After therapy discontinuation, patients were followed-up for 6 months to

assess relapses. The primary outcome was the number of patients discontinuing either therapy owing to inefficacy or intolerance. MLN8237 order Secondary end-points included the proportion of patients experiencing adverse events or worsening after therapy discontinuation. More patients stopped methylprednisolone (52%) than IVIG (13%) (P = 0·0085). The reasons for discontinuation were lack of efficacy, adverse events or voluntary withdrawal. After therapy discontinuation, more patients on IVIG worsened and required further therapy (38%) than did those on methylprednisolone (none) (P = 0·0317). Thus, treatment of CIDP with IVIG for 6 months was discontinued less frequently because of inefficacy, adverse events or intolerance than treatment with intravenous methylprednisolone. Another recent prospective, multi-centre, single-arm, open-label Phase III study [Privigen® Impact on Mobility and Autonomy

(PRIMA) trial] evaluated the efficacy and safety of IVIG in 28 patients with CIDP [38]. Patients received one induction dose of IVIG (2 g/kg body weight) and up to seven maintenance doses (1 g/kg body weight) at 3-week intervals. The overall responder rate defined as an improvement of ≥1 point on the INCAT disability scale at completion Calpain was 60·7%. IVIG-pretreated patients demonstrated a higher responder rate than IVIG-naive patients (76·9 versus 46·7%). The INCAT score, the maximum grip strength and the Medical Research Council sum score all improved significantly at completion compared to baseline. Thus, these recent trials provide evidence for the long-term efficacy of IVIG in patients with CIDP. Adverse effects, frequent: headache, hypertension, allergic/anaphylactic reactions [especially in immunoglobulin (Ig)A-deficient patients], dermatitis; infrequent: infection (HIV or viral hepatitis) by contaminated blood product, pulmonary oedema from fluid overload, due to the high colloid oncotic pressure of IVIG, venous thrombosis, aseptic meningitis and haemolysis.