Eighteen patients with definite, untreated chronic inflammatory demyel
inating polyradiculoneuropathy (CIDP) of chronic progressive (nine pat
ients) or relapsing course (nine patients) were randomized prospective
ly, to receive 10 plasma-exchange (PE) or sham plasma-exchange (SPE) t
reatments over 4 weeks in a double-blind trial. After a wash-out perio
d of 5 weeks or when they returned to baseline scores, patients were c
rossed over to the alternate treatments. Neurological function was ass
essed serially using a quantitative neurological disability score (NDS
), a functional clinical grade (CG) and grip strength (GS) measurement
s. Electrophysiological studies were done at the beginning and end of
each treatment. A primary 'intention to treat' analysis showed signifi
cant improvement with PE in all clinical outcome measures: NDS by 38 p
oints, P < 0.001; CG by 1.6 points, P < 0.001; GS by +13 kg, P < 0.003
and in selected electrophysiological measurements, Sigma proximal CMA
P, P < 0.01; Sigma motor conduction velocities, P < 0.006; Sigma dista
l motor latencies, P < 0.01. Fifteen patients completed the trial and
of those, 12 patients (80%) improved substantially with PE; i.e. Jive
out of seven patients with chronic progressive course and seven out of
eight patients with relapsing CIDP improved. There were three drop-ou
ts: one patient lost venous access; one patient suffered a stroke and
one patient left the trial to receive open treatment elsewhere. The im
provement in motor functions correlated with the electrophysiological
data, i.e. with improved motor conduction velocities and reversal of c
onduction block. Eight of 12 PE responders (66%) relapsed within 7-14
days after stopping PE. All improved with subsequent open label PE; al
l but two patients required long-term immunosuppressive drug therapy f
or stabilisation. The PE non-responders improved with prednisone. We c
onclude that PE is a very effective adjuvant therapy for CIDP of both
chronic progressive and relapsing course; concurrent immunosuppressive
drug treatment is required. Exchange treatments should be given two t
o three times per week until improvement is established; the treatment
frequency should then be tapered over several months.