Treatment for IgG and IgA paraproteinaemic neuropathy

Allen D, Lunn MPT, Niermeijer J, Nobile-Orazio E

Published Online:

January 21, 2009

Paraproteinaemic neuropathy refers to those neuropathies associated with a paraprotein (an abnormal antibody or immunoglobulin present in relative excess in the blood). If the paraprotein is present without underlying evidence of disease, this is known as a monoclonal gammopathy of uncertain significance. This review looked at the treatments for neuropathy associated with and possibly caused by IgG and IgA paraproteins. The optimal treatment is not known. Treatments that act on the immune system such as plasma exchange, corticosteroids or intravenous immunoglobulin have been examined in non-randomised studies of people with IgG and IgA paraproteinaemic neuropathy. We identified only one randomised controlled trial of 18 participants treated with plasma exchange. The trial demonstrated a modest benefit over a short follow-up period. The long-term benefit is unclear. Further randomised controlled trials of this and other treatments with larger numbers of participants are needed.

Abstract (click to read)

Background:

Paraproteinaemic neuropathy refers to those neuropathies associated with a monoclonal gammopathy or paraprotein. Typically it presents with a chronic predominantly sensory, symmetrical neuropathy, similar to chronic inflammatory demyelinating polyradiculoneuropathy but with relatively more sensory involvement, both clinically and neurophysiologically. The optimal treatment for IgG and IgA monoclonal gammopathy of uncertain significance neuropathies is not known.

Objectives:

The objective of this review is to examine the efficacy of any treatment for IgG or IgA paraproteinaemic peripheral neuropathy.

Search strategy:

We performed searches of the Cochrane Neuromuscular Disease Group Trials register (May 2005), MEDLINE (from January 1966 to May 2005), EMBASE (from January 1980 to May 2005). We also checked bibliographies for controlled trials of treatments for IgG or IgA paraproteinaemic peripheral neuropathy.

Selection criteria:

We included randomised and quasi-randomised controlled trials using any treatment for IgG or IgA paraproteinaemic peripheral neuropathy. People with IgM paraproteins were excluded. We excluded participants where the monoclonal gammopathy was considered secondary to an underlying disorder. We included participants of any age with a diagnosis of monoclonal gammopathy of uncertain significance with a paraprotein of the IgG or IgA class and a neuropathy. Included participants were not required to fulfil specific electrophysiological diagnostic criteria.

Data collection and analysis:

The full texts of potentially relevant studies were obtained and assessed and independent data extraction was performed by three authors. Additional data and clarification were received from one author.

Main results:

We identified only one randomised controlled trial with 18 participants which fulfilled the predetermined inclusion criteria. Four other trials were identified but these were not randomised controlled trials. The included trial revealed a modest short-term benefit of plasma exchange in IgG or IgA paraproteinaemic neuropathy, over a short follow-up period, when compared to sham plasma exchange.

Authors' conclusions:

The evidence from randomised controlled trials for the treatment of IgG or IgA paraproteinaemic neuropathy is currently inadequate. More randomised controlled trials of treatments are required. These should have adequate follow-up periods and contain larger numbers of participants, perhaps through multicentre collaboration, considering the relative infrequency of this condition. Observational or open trial data provide limited support for the use of treatments such as plasma exchange, cyclophosphamide combined with prednisolone, intravenous immunoglobulin and corticosteroids. These show potential therapeutic promise but the potential benefits must be weighed against adverse effects. Their optimal use and the long-term benefits need to be considered and validated with well-designed randomised controlled trials.

This record should be cited as:

Allen D, Lunn MPT, Niermeijer J, Nobile-Orazio E. Treatment for IgG and IgA paraproteinaemic neuropathy. Cochrane Database of Systematic Reviews 2007, Issue 1. Art. No.: CD005376. DOI: 10.1002/14651858.CD005376.pub2

Assessed as up to date:

October 24, 2006

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Primary Review Group:

Neuromuscular Disease Group

Health topics:

Neurology > Peripheral neuropathy > Paraneoplastic neuropathy

Neurology > Peripheral neuropathy > Paraproteinaemic neuropathy

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Treatment for IgG and IgA paraproteinaemic neuropathy

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