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Treatment for IgG and IgA paraproteinaemic neuropathy

  • Review
  • Intervention

Authors


Abstract

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 methods

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.

摘要

背景

IgG及IgA副蛋白質血症神經病變的治療

副蛋白質血症神經病變是指那些與單株免疫球蛋白及副蛋白質相關的神經病變。通常它的表現是以慢性、感覺症狀為主、並且是對稱性的神經病變,類似慢性炎症脫髓鞘多發性神經病變 ,但在臨床和神經生理的變化上,感覺受損的情形均較為嚴重。目前對IgG、IgA未知臨床意義的單株免疫球蛋白神經病變的最佳治療仍舊不清楚。

目標

本回顧研究的目的在於檢驗IgG及IgA副蛋白質血症神經病變的治療療效。

搜尋策略

搜索範圍包括考科藍神經肌肉疾病組試驗紀錄(2005年5月) ,MEDLINE(從1966年1月至2005年5月),EMBASE(從1980年1月至2005年5月)。另外也檢閱了關於治療IgG及IgA副蛋白質血症神經病變的對照試驗的文獻目錄。

選擇標準

納入的研究包括了治療IgG及IgA副蛋白質血症神經病變的隨機和準隨機對照試驗。IgM副蛋白血症的病人被排除在外。另外也排除了其他疾病相關的次發性單株免疫球蛋白症的病人。不論年齡,只要診斷為未知臨床意義的單株免疫球蛋白神經病變合併有IgG或IgA副蛋白質的病人均被納入研究中。納入的患者不一定需要符合電生理學的診斷標準。

資料收集與分析

三位作者取得相關研究報告的全文,獨立進行評估和擷取數據。另外一位作者提供額外的數據和闡述資料。

主要結論

只有一項含有18名患者的隨機對照試驗符合預定的納入標準。另外也搜尋到相關的4項臨床試驗,不過這些皆非隨機對照試驗。結果顯示,在短期的追蹤發現,血漿置換術在治療IgG或IgA副蛋白質血症神經病變上,比起假裝安排的血漿置換治療,有些許的益處。

作者結論

隨機對照試驗的結果顯示,用於治療IgG及IgA副蛋白質血症神經病變的資料仍為不足,尚須更多的隨機對照試驗。之後的試驗應該包含更適當的追蹤期,和更多參與患者數。考慮到此疾病的罕見性,也許還需要透過多中心的合作來進行這些試驗。在觀察性試驗或開放性試驗的數據提供了有限的資料,支持血漿置換、環磷?胺合併類固醇、靜脈注射免疫球蛋白、及類固醇等治療。這些結果顯示了潛在的治療效果,但還必須把副作用造成的影響加以權衡在內。最佳的治療方式和長期利益,還需要設計良好的隨機對照試驗來加以驗證。

翻譯人

本摘要由新光醫院鍾禎智翻譯。

此翻譯計畫由臺灣國家衛生研究院(National Health Research Institutes, Taiwan)統籌。

總結

治療IgG及IgA副蛋白質血症神經病變: 副蛋白質血症神經病變是指那些合併有副蛋白質(一種在血液中過量的不正常的抗體或免疫球蛋白)的神經病變 。如果這些副蛋白質的產生並沒有合併其他疾病,便稱之為未知臨床意義的單株免疫球蛋白增高。 本篇回顧分析著重在於這些可能由IgG及IgA副蛋白質造成的神經病變之治療,但最佳的治療仍舊未知。針對免疫系統的治療方式,包括血漿置換、糖皮質激素或靜脈注射免疫球蛋白等,已在非隨機研究中被檢驗過。目前已確認的只有一項包含18位病人的血漿置換治療隨機對照試驗。此 試驗發現,短期追蹤下,治療得到一些輕微的改善。但長期的益處仍舊未明。目前還需要多一些有更多參與患者的隨機對照試驗。

Plain language summary

Treatment for IgG and IgA paraproteinaemic neuropathy

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.

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