I. Bodi et al., POLYNEUROPATHY WITH ENDONEURIAL IMMUNE-COMPLEX DEPOSITION AS THE FIRST MANIFESTATION OF SYSTEMIC LUPUS-ERYTHEMATOSUS, Acta Neuropathologica, 96(3), 1998, pp. 297-300
A 72-year-old male presented with progressive sensorimotor polyneuropa
thy. Later weight loss, proteinuria and deteriorating renal function w
ere noted. The electrophysiological examinations revealed extensive, s
ymmetrical demyelinating and axonal polyneuropathy. Frozen sections ob
tained from sural nerve biopsy sample showed the presence of immune co
mplexes and complements in the walls of the epi- and endoneurial blood
vessels, and perineurium suggestive of systemic lupus erythematous (S
LE). IgG and Clq deposits were also present along the basement membran
es of Schwann cells. The electron microscopy confirmed the presence of
immune complex deposition. Diagnosis of SLE was proven by positive se
rology (anti-nuclear antibodies, anti-Sm, anti-RNP, anti-double-strand
ed DNA) and renal biopsy showing membranous lupus nephritis with exten
sive immune complex deposition in the tubular basement membranes. Desp
ite combined immunosuppressive treatment for 10 months, the patient di
ed of complications of generalized immune complex vasculitis. The mani
festation of SLE in elderly patients, especially in males, is very rar
e. Moreover, the polyneuropathy is an unusual initial symptom of SLE.
Immune complex deposition in Schwann cell basement membrane probably p
lays an important role in the pathomechanism of sensorimotor polyneuro
pathy in SLE.