The Birmingham Vasculitis Activity Score (BVAS) has been validated for initial assessment, with a high BVAS at baseline predictive of a worse outcome.[19]Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome: a prospective study in 342 patients. Medicine (Baltimore). 1996;75:17-28.
http://www.ncbi.nlm.nih.gov/pubmed/8569467?tool=bestpractice.com
[56]Gayraud M, Guillevin L, le Toumelin P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum. 2001;44:666-675.
http://www3.interscience.wiley.com/cgi-bin/fulltext/78003355/HTMLSTART
http://www.ncbi.nlm.nih.gov/pubmed/11263782?tool=bestpractice.com
[107]Luqmani RA, Bacon PA, Moots RJ, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87:671-678.
http://www.ncbi.nlm.nih.gov/pubmed/7820541?tool=bestpractice.com
The BVAS can be used to assess effectiveness of treatment over time. A flare of disease corresponds to a score above 0; remission is indicated by a score of 0.
A validated tool, such as the BVAS, or the Vasculitis Damage Index (VDI), are used to assess disease activity, the site and extent of involvement, and overall quality of life.[60]Ntatsaki E, Carruthers D, Chakravarty K, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014 Dec;53(12):2306-9.
http://rheumatology.oxfordjournals.org/content/53/12/2306.long
http://www.ncbi.nlm.nih.gov/pubmed/24729399?tool=bestpractice.com
Assessments should include clinical, laboratory, and investigations dependent on organ involvement.[60]Ntatsaki E, Carruthers D, Chakravarty K, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014 Dec;53(12):2306-9.
http://rheumatology.oxfordjournals.org/content/53/12/2306.long
http://www.ncbi.nlm.nih.gov/pubmed/24729399?tool=bestpractice.com
Use of cyclophosphamide, azathioprine, and other immunosuppressive agents requires specific blood and urine monitoring as per standard protocols.[60]Ntatsaki E, Carruthers D, Chakravarty K, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014 Dec;53(12):2306-9.
http://rheumatology.oxfordjournals.org/content/53/12/2306.long
http://www.ncbi.nlm.nih.gov/pubmed/24729399?tool=bestpractice.com
Repeat angiography is not routinely used in follow-up, because improvement in clinical features has been found to parallel the resolution of aneurysms.[20]Guillevin L, Mahr A, Callard P, et al. Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore). 2005;84:313-322.
http://www.ncbi.nlm.nih.gov/pubmed/16148731?tool=bestpractice.com
[114]Darras-Joly C, Lortholary O, Cohen P, et al. Regressing microaneurysms in 5 cases of hepatitis B virus related polyarteritis nodosa. J Rheumatol. 1995;22:876-880.
http://www.ncbi.nlm.nih.gov/pubmed/8587075?tool=bestpractice.com
Female patients should have an annual cervical smear for 3 years after cyclophosphamide therapy.[60]Ntatsaki E, Carruthers D, Chakravarty K, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014 Dec;53(12):2306-9.
http://rheumatology.oxfordjournals.org/content/53/12/2306.long
http://www.ncbi.nlm.nih.gov/pubmed/24729399?tool=bestpractice.com
[115]Ognenovski VM, Marder W, Somers EC, et al. Increased incidence of cervical intraepithelial neoplasia in women with systemic lupus erythematosus treated with intravenous cyclophosphamide. J Rheumatol. 2004;31:1763-1767.
http://www.ncbi.nlm.nih.gov/pubmed/15338497?tool=bestpractice.com
Yearly influenza vaccines are recommended.[113]Gluck T. Vaccinate your immunocompromised patients! Rheumatology (Oxford). 2006;45:9-10.
http://rheumatology.oxfordjournals.org/cgi/content/full/45/1/9
http://www.ncbi.nlm.nih.gov/pubmed/16361703?tool=bestpractice.com
Pneumococcal vaccine should be offered if antibody levels are low.[113]Gluck T. Vaccinate your immunocompromised patients! Rheumatology (Oxford). 2006;45:9-10.
http://rheumatology.oxfordjournals.org/cgi/content/full/45/1/9
http://www.ncbi.nlm.nih.gov/pubmed/16361703?tool=bestpractice.com
Live vaccines should not be used until 3 months after cyclophosphamide has been stopped.