We assessed the clinicopathological features of 28 patients with peripheral
neuropathy associated with Churg-Strauss syndrome. Initial symptoms attrib
utable to neuropathy were acute painful dysaesthesiae and oedema in the dys
aesthetic portion of the distal limbs. Sensory and motor involvement mostly
showed a pattern of mononeuritis multiplex in the initial phase, progressi
ng into asymmetrical polyneuropathy, restricted to the limbs. Parallel loss
of myelinated and unmyelinated fibres due to axonal degeneration was evide
nt as decreased or absent amplitudes of sensory nerve action potentials and
compound muscle action potentials, indicating acute massive axonal loss. E
pineurial necrotizing vasculitis was seen in 54% of cases; infiltrates cons
isted mainly of CD8-positive suppressor/cytotoxic and CD4-positive helper T
lymphocytes. Eosinophils were present in infiltrates, but in smaller numbe
rs than lymphocytes. CD20-positive B lymphocytes were seen only occasionall
y. Deposits of IgG, C3d, IgE and major basic protein were scarce. The mean
follow-up period was 4.2 years, with a range of 8 months to 10 years. Fatal
outcome was seen only in a single patient, indicating a good survival rate
. The patients who responded well to the initial corticosteroid therapy wit
hin 4 weeks regained self-controlled functional status in longterm follow-u
p (modified Rankin score was less than or equal to 2), while those not resp
onding well to the initial corticosteroid therapy led a dependent existence
(P < 0.01), In addition the patients with poor functional outcomes had sig
nificantly more systemic organ damage caused by vasculitis (P < 0.05), Necr
otizing vasculitis mediated by cytotoxic T cells, leading to ischaemic chan
ges, appears to be a major cause of Churg-Strauss syndrome-associated neuro
pathy, The initial clinical course and the extent of systemic vasculitic le
sions may influence the long-term functional prognosis.