课件：白塞氏病.ppt

Recommendation 7 The pathergy test is simple and has a well-established role in BD diagnosis. We recommend pathergy testing in suspected NBD, since a positive result, especially with other systemic BD eatures, would contribute significantly toward NBD diagnosis. A negative test, however, will not exclude NBD Recommendation 8 BD is associated with the HLA-B5 allele and, more specifically, with HLA-B51. It is not clear if HLA-B51/B5 testing has a role in the diagnosis or prognosis of BD or NBD. Recommendation 9 Neurophysiologic tests are not routinely recommended for NBD. These may be useful if peripheral nervous system or optic nerve involvement is suspected. Asymptomatic neurophysiological findings are of doubtful clinical significance. The diagnosis of NBD should be avoided when solely based on asymptomatic neurophysiological findings Recommendation 10 Nervous tissue biopsy can occasionally be useful in the diagnosis of NBD. It is usually not recommended as a part of the diagnostic process. As it is an invasive procedure, we recommend considering it when all other diagnostic avenues have been exhausted, especially for tumour-like presentation. Management Recommendation 11 (a) There is no level I evidence on the treatment options of NBD. The following recommendations are mainly based on observational data (b) For acute/sub-acute parenchymal NBD attack, a course of corticosteroids is recommended, preferably IV methyl prednisolone for 3–10 days followed by a maintenance oral corticosteroid for a few months (up to 6 months) (c) We recommend considering a disease modifying therapy (DMT) after a significant parenchymal relapse depending on severity, response to steroid, previous neurological relapses,disease course, and other associated systemic BD features (d) Azathioprine is recommended as a first-line DMT; alternatives include mycophenolate mofetil, methotrexate, and cyclophosphamide (e) We recommend considering a biolo