Treatment of inflammatory and paraproteinemic
neuropathies.
Monaco S, Turri E, Zanusso G, Maistrello B.
Section of Clinical Neurology, Department of
Neurological and Visual Sciences, University of Verona,
Verona, Italy.
Acquired demyelinating and inflammatory neuropathies
encompass a number of acute and chronic autoimmune
conditions characterized by variable degrees of clinical
involvement. These disorders, including Guillain-Barre
syndrome (GBS), chronic inflammatory demyelinating
polyradiculoneuropathy (CIDP), multifocal motor
neuropathy (MMN), and paraprotein-associated neuropathy,
have an overall annual incidence of 2-4/100,000
worldwide and are potentially treatable. Over the last
few years, several investigations have helped clarify
the pathogenesis of immune neuropathies and the
definition of molecular targets involved in these
diseases, thus providing firmer grounds for treatment
with classical immunosuppressive drugs and new
biological agents. In GBS and related variants, which
are characterized by cellular inflammation and
alterations of the blood-nerve barrier, randomized
clinical trials show that plasma exchange (PE) and
intravenous immunoglobulin (IVIg) are equally effective
as disease-modifying treatments, although IVIg has been
adopted as the favourite treatment in most centres. In
CIDP, controlled clinical trials have established the
efficacy of oral prednisone, PE and IVIg, with
intermittent IVIg treatment or corticosteroids being
usually preferred. Adding azathioprine can help keep
lower the required dose of prednisone, while other
immunosuppressive agents, such as cyclophosphamide and
cyclosporin A may have side effects, limiting their use
to selected cases. Currently, the efficacy of interferon
beta and alfa is under evaluation. Controlled trials
support the view that IVIg is the treatment of choice in
MMN. Patients resistant to IVIg administration may
benefit of treatments which deplete B cells, such as
cyclophosphamide and rituximab. Demyelinating
neuropathies associated with circulating paraproteins
are clinically heterogeneous, depending on the
reactivity and type of the monoclonal (M) protein. In
many cases, neuropathies associated with IgM M proteins
are not treated because of their slow progression. In
patients with a disabling or rapid progression, small
trials have shown short-term benefits from IVIg or PE.
Recently, fludarabine and rituximab have been reported
as beneficial in selected cases.
Cur
Drug Targets Immune Endocr Metabol Disord. 2004
Jun;4(2):141-8.
Publication Types:
Review
Review, Tutorial
PMID: 15180454 [PubMed - indexed for MEDLINE] |
