Evidence report — Behçet’s syndrome with vascular-inflammatory central nervous system involvement




НазваниеEvidence report — Behçet’s syndrome with vascular-inflammatory central nervous system involvement
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Criteria for the Clinical Use of Intravenous Immunoglobulin (IVIg) in Australia


Evidence report: Behçet’s syndrome


Report prepared by Biotext for
National Blood Authority




© 2010, National Blood Authority, Canberra.

Report researched, written and prepared by Meg Heaslop, Janet Salisbury, Julie Irish, Jacinda Wilson, Biotext Pty Ltd, Canberra.

Evidence report — Behçet’s syndrome with vascular-inflammatory central nervous system involvement


PICO question

In patients with Behçet’s syndrome with vascular-inflammatory central nervous system involvement, does IVIg improve the clinical or laboratory markers, compared to no IVIg or standard treatment?

Status in current criteria

Currently unlisted

Evidence classification

4b (no included studies)
[However, the search retrieved several general reviews of Behçet’s syndrome that did not include IVIg as a treatment.]

Search strategy


We searched the following electronic databases:

  • Cochrane Database of Systematic Reviews (the Cochrane Collaboration: Cochrane Reviews)

  • Database of Abstracts of Reviews of Effects (DARE) (non-Cochrane reviews)

  • PubMed/MEDLINE, EMBASE

  • Cochrane Controlled Clinical Trials Registry (CENTRAL)

  • US National Institutes of Health international clinical trials registry (www.clinicaltrials.gov).

We also used a range of other methods, including checking references cited in identified articles; hand searching recent issues of relevant journals (see the list of journals, below); and checking all other relevant sources, including papers suggested in the submissions to the National Blood Authority.

Searches were limited to a publication date of 2004 onwards, and to humans.

In PubMed, we used the following search string:

  • Behcet’s syndrome OR Behcets syndrome OR Behcet syndrome OR Behcet’s disease OR Behcet disease OR vasculitis OR Behcet syndrome[MH] OR nervous system OR nervous system involvement OR central nervous system OR Nervous System Diseases OR Ocular disease OR eye involvement OR refractory eye involvement OR ocular involvement AND Immunoglobulins, Intravenous[MH] OR intravenous immunoglobulin

In MEDLINE and EMBASE, we used the following search string (limited to clinical trials) to make sure no papers were missed:

  • autoimmune encephalopathy OR encephalopathy OR encephalopathies OR brain disease OR brain inflammation OR Brain Diseases[MH] OR Brain Inflammation[MH] AND Autoimmune Diseases[MH] OR autoimmune disease OR disease, autoimmune OR diseases, autoimmune AND Immunoglobulins, Intravenous[MH] OR intravenous immunoglobulin

In the Cochrane Library (including CENTRAL and DARE) and www.clinicaltrials.gov, we used the following search terms:

  • ocular involvement AND intravenous immunoglobulin

  • ocular involvement AND immunoglobulin.

After running the search string in each database (see below), we selected articles that appeared relevant on the basis of their title (being careful to include papers where there was doubt). These papers were downloaded into an EndNote library, and duplicates were discarded. We then winnowed them further, by reviewing abstracts. Finally, we selected only those that were most relevant by a more thorough review of titles and abstracts, and — where relevant — whole papers. We used the following rules when selecting papers:

  • randomised controlled trials and systematic reviews were chosen preferentially, where available

  • if no randomised controlled trials or systematic reviews were available, case series were chosen (and the whole article was downloaded)

  • if no case series were available, case studies were included, but only abstracts were viewed (papers in languages other than English were excluded)

  • finally, if only case studies were available, general reviews were also included (but not if they were superseded by a Cochrane review).
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