PMID- 15150209
OWN - NLM
STAT- MEDLINE
DCOM- 20040524
LR  - 20220311
IS  - 1538-3598 (Electronic)
IS  - 0098-7484 (Linking)
VI  - 291
IP  - 19
DP  - 2004 May 19
TI  - Intravenous immunoglobulin in autoimmune neuromuscular diseases.
PG  - 2367-75
AB  - CONTEXT: Intravenous immunoglobulin (IVIG) enhances immune homeostasis by 
      modulating expression and function of Fc receptors, interfering with activation 
      of complement and production of cytokines, providing anti-idiotypic antibodies, 
      and affecting the activation and effector functions of T and B cells. These 
      mechanisms may explain the effectiveness of IVIG in autoimmune neuromuscular 
      disorders. OBJECTIVE: To systematically review the current status of the 
      treatment of autoimmune neuromuscular diseases with IVIG, with emphasis on 
      controlled trials. DATA SOURCES: Peer-reviewed publications identified through 
      MEDLINE (1966-2003), EMBASE (1974-2003), and references from bibliographies of 
      pertinent articles. Each autoimmune neuromuscular disease term was searched in 
      combination with the term intravenous immunoglobulin. STUDY SELECTION AND DATA 
      EXTRACTION: Criteria for selection of studies included controlled study design, 
      English language, and clinical pertinence. Data quality was based on venue of 
      publication and relevance to clinical care. DATA SYNTHESIS: Outcomes of 
      controlled trials indicate that IVIG at a total dose of 2 g/kg is effective as 
      first-line therapy in Guillain-Barre syndrome, chronic inflammatory demyelinating 
      polyneuropathy, and multifocal motor neuropathy and as second-line therapy in 
      stiff-person syndrome, dermatomyositis, myasthenia gravis, and Lambert-Eaton 
      myasthenic syndrome. In other controlled studies, IVIG produced a modest, 
      variable, and transient but not statistically significant benefit in patients 
      with inclusion body myositis and paraproteinemic anti-myelin-associated 
      glycoprotein antibody demyelinating polyneuropathy. Intravenous immunoglobulin is 
      not effective in patients with multiple sclerosis who have established weakness 
      or optic neuritis. In myasthenia gravis, it should be reserved for difficult 
      cases or before thymectomy in lieu of plasma exchange. CONCLUSION: Intravenous 
      immunoglobulin is effective in many autoimmune neurologic diseases, but its 
      spectrum of efficacy, especially as first-line therapy, and the appropriate dose 
      for long-term maintenance therapy are not fully established. Further controlled 
      studies of IVIG, combined with a dose-finding effect, pharmacoeconomics, and 
      quality-of-life assessments, are warranted to improve the evidence base for 
      clinical practice.
FAU - Dalakas, Marinos C
AU  - Dalakas MC
AD  - Neuromuscular Diseases Section, National Institutes of Health, National Institute 
      of Neurological Disorders and Stroke, Bethesda, Md 20892, USA. 
      dalakasm@ninds.nih.gov
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PT  - Review
PL  - United States
TA  - JAMA
JT  - JAMA
JID - 7501160
RN  - 0 (Immunoglobulins, Intravenous)
SB  - IM
CIN - JAMA. 2004 Sep 22;292(12):1429; author reply 1429-30. PMID: 15383509
MH  - Autoimmune Diseases of the Nervous System/*drug therapy/immunology
MH  - Clinical Trials as Topic
MH  - Humans
MH  - Immunoglobulins, Intravenous/adverse effects/*therapeutic use
MH  - Neuromuscular Diseases/*drug therapy/immunology
RF  - 83
EDAT- 2004/05/20 05:00
MHDA- 2004/05/25 05:00
CRDT- 2004/05/20 05:00
PHST- 2004/05/20 05:00 [pubmed]
PHST- 2004/05/25 05:00 [medline]
PHST- 2004/05/20 05:00 [entrez]
AID - 291/19/2367 [pii]
AID - 10.1001/jama.291.19.2367 [doi]
PST - ppublish
SO  - JAMA. 2004 May 19;291(19):2367-75. doi: 10.1001/jama.291.19.2367.