References

Andrews M, Edmunds M, Campbell A, Walls J, Feehally J. Systemic vasculitis in the 1980s—is there an increasing incidence of Wegener's granulomatosis and microscopic polyarteritis?. J R Coll Physicians Lond. 1990; 24:(4)284-288

Vasculitis, 2nd edn. In: Ball GV, Bridges SL (eds). Oxford: Oxford University Press; 2008

Ball GV, Fessler BJ, Bridges SL. Oxford textbook of vasculitis, 3rd edn. Oxford: Oxford University Press; 2014

Berden A, Göçeroglu A, Jayne D Diagnosis and management of ANCA associated vasculitis. BMJ. 2012; 344 https://doi.org/10.1136/bmj.e26

Berti A, Cornec D, Crowson CS, Specks U, Matteson EL. The epidemiology of antineutrophil cytoplasmic autoantibody-associated vasculitis in Olmsted County, Minnesota: a twenty-year US population-based study. Arthritis Rheumatol. 2017; 69:(12)2338-2350 https://doi.org/10.1002/art.40313

Biscetti F, Carbonella A, Parisi F The prognostic significance of the Birmingham Vasculitis Activity Score (BVAS) with systemic vasculitis patients transferred to the intensive care unit (ICU). Medicine (Baltimore). 2016; 95:(48) https://doi.org/10.1097/MD.0000000000005506

Improving outcomes for patients with ANCA-associated vasculitis. 2017. https://tinyurl.com/yxv7ehtm (accessed 24 November 2020)

Calatroni M, Buzio C, Vaglio A. The evolving paradigm of cancer risk related to cyclophosphamide therapy in granulomatosis with polyangiitis: Table 1. Rheumatology. 2015; 54:(8)1339-1341 https://doi.org/10.1093/rheumatology/kev196

Cameron JS. Bright's disease today: the pathogenesis and treatment of glomerulonephritis—I. BMJ. 1972; 4:(5832)87-90 https://doi.org/10.1136/bmj.4.5832.87

De Joode A, Middelkoop S, Sanders J, Stegeman C. Microscopic haematuria in anti-neutrophil cytoplasm antibody-associated vasculitis with glomerulonephritis during treatment and remission. Journal of Vasculitis. 2016; 2:(3) https://doi.org/10.4172/2471-9544.100116

Garrity J. Ocular manifestations of small-vessel vasculitis. Cleveland Clinic Journal of Medicine. 2012; 79:(3)S31-S33

Geetha D, Kallenberg C, Stone JH Current therapy of granulomatosis with polyangiitis and microscopic polyangiitis: the role of rituximab. J Nephrol. 2015; 28:(1)17-27 https://doi.org/10.1007/s40620-014-0135-3

Assessment of nephrotic syndrome. BMJ Best Practice. 2018. https://bestpractice.bmj.com/topics/en-gb/356 (accessed 24 November 2020)

Guillevin L, Dörner T. Vasculitis: mechanisms involved and clinical manifestations. Arthritis Res Ther. 2007; 9 https://doi.org/10.1186/ar2193

Hamour S, Salama AD, Pusey CD. Management of ANCA-associated vasculitis: Current trends and future prospects. Ther Clin Risk Manag. 2010; 6:253-64 https://doi.org/10.2147/TCRM.S6112

Havill JP, Levine SM, Kuperman M, Hellmann DB, Geetha D. Falling through the cracks of vasculitis classification-a report of three patients. NDT Plus. 2011; 4:(5)327-330 https://doi.org/10.1093/ndtplus/sfr054

Heijl C, Harper L, Flossmann O Incidence of malignancy in patients treated for antineutrophil cytoplasm antibody-associated vasculitis: follow-up data from European Vasculitis Study Group clinical trials. Ann Rheum Dis. 2011; 70:(8)1415-1421 https://doi.org/10.1136/ard.2010.145250

Hebert LA, Parikh S, Prosek J, Nadasdy T, Rovin BH. Differential diagnosis of glomerular disease: a systematic and inclusive approach. Am J Nephrol. 2013; 38:(3)253-266 https://doi.org/10.1159/000354390

Hilhorst M, Wilde B, van Paassen P, Winkens B, van Breda Vriesman P, Cohen Tervaert JW Improved outcome in anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis: a 30-year follow-up study. Nephrol Dial Transplant. 2013; 28:(2)373-379 https://doi.org/10.1093/ndt/gfs428

Hunder GG, Arend WP, Bloch DA The American College of Rheumatology 1990 criteria for the classification of vasculitis: introduction. Arthritis Rheum. 1990; 33:(8)1065-1067 https://doi.org/10.1002/art.1780330802

Jayne D. Vasculitis—when can biopsy be avoided?. Nephrol Dial Transplant. 2017; 32:(9)1454-1456 https://doi.org/10.1093/ndt/gfx248

Jennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Clinical and Experimental Nephrology. 2013; 17:(5)603-606 https://doi.org/10.1007%2Fs10157-013-0869-6

Jennette JC, Falk RJ, Andrassy K Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994; 37:(2)187-192 https://doi.org/10.1002/art.1780370206

Jennette JC, Falk RJ, Bacon PA 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum. 2013; 65:(1)1-11 https://doi.org/10.1002/art.37715

John Hopkins Vasculitis Centre. Diagnosing vasculitis. 2019. https://tinyurl.com/y3836udp (accessed 24 November 2020)

Kermani TA, Cuthbertson D, Carette S The Birmingham Vasculitis Activity Score as a measure of disease activity in patients with giant cell arteritis. J Rheumatol. 2016; 43:(6)1078-1084 https://doi.org/10.3899/jrheum.151063

Kermani TA, Warrington KJ, Amin S. Malignancy risk in vasculitis. Ther Adv Musculoskelet Dis. 2011; 3:(1)55-63 https://doi.org/10.1177/1759720X10387460

Kobari Y, Nagasawa T. Otitis media with anti-neutrophil cytoplasm antibody-associated vasculitis: a new concept and the associated criteria. Intern Med. 2017; 56:(24)3365-3367 https://doi.org/10.2169/internalmedicine.8704-16

Lane SE, Watts R, Scott DGI. Epidemiology of systemic vasculitis. Curr Rheumatol Rep. 2005; 7:(4)270-275 https://doi.org/10.1007/s11926-005-0036-5

Leclair V, Lundberg IE. New myositis classification criteria—what we have learned since Bohan and Peter. Curr Rheumatol Rep. 2018; 20:(4) https://doi.org/10.1007/s11926-018-0726-4

Luqmani RA, Suppiah R, Grayson PC, Merkel PA, Watts R. Nomenclature and classification of vasculitis—update on the ACR/EULAR Diagnosis and Classification of Vasculitis Study (DCVAS). Clin Exp Immunol. 2011; 164:11-13 https://doi.org/10.1111/j.1365-2249.2011.04358.x

Lv L, Chang DY, Li ZY, Chen M, Hu Z, Zhao MH. Persistent hematuria in patients with antineutrophil cytoplasmic antibody-associated vasculitis during clinical remission: chronic glomerular lesion or low-grade active renal vasculitis?. BMC Nephrol. 2017; 18:(1) https://doi.org/10.1186/s12882-017-0763-7

Mahr A, de Menthon M. Classification and classification criteria for vasculitis: achievements, limitations and prospects. Curr Opin Rheumatol. 2015; 27:(1)1-9 https://doi.org/10.1097/BOR.0000000000000134

Martinez Valenzuela L, Bordignon Draibe J, Fulladosa Oliveras X, Bestard Matamoros O, Cruzado Garrit JM, Torras Ambrós J. T-lymphocyte in ANCA-associated vasculitis: what do we know? A pathophysiological and therapeutic approach. Clin Kidney J. 2019; 12:(4)503-511 https://doi.org/10.1093/ckj/sfz029

McFadden R. Introducing pharmacology for nursing and healthcare, 3rd edn. Abingdon: Routledge; 2019

Miller A, Chan M, Wiik A, Misbah SA, Luqmani RA. An approach to the diagnosis and management of systemic vasculitis. Clin Exp Immunol. 2010; 160:(2)143-160 https://doi.org/10.1111/j.1365-2249.2009.04078.x

Monach PA. ANCA-associated vasculitis: a prothrombotic state even in remission?. J Rheumatol. 2013; 40:(12)1935-1937 https://doi.org/10.3899/jrheum.131069

Mooney J, Spalding N, Poland F The informational needs of patients with anti-neutrophil cytoplasm antibody-associated vasculitis--development of an informational needs questionnaire. Rheumatology. 2014; 53:(8)1414-1421 https://doi.org/10.1093/rheumatology/keu026

Mortaz E, Alipoor SD, Adcock IM, Mumby S, Koenderman L. Update on neutrophil function in severe inflammation. Front Immunol. 2018; 9:(2171) https://doi.org/10.3389/fimmu.2018.02171

Using the Birmingham Vasculitis Activity Score (BVAS) to screen for vasculitis. Poster. 2008. https://tinyurl.com/yyul5u5s

Blood in urine linked to higher risk of kidney disease relapse in AAV patients, study finds. ANCA Vasculitis News. 2019. https://ancavasculitisnews.com/2019/05/28/hematuria-linked-kidney-disease-relapse-aav-patient/ (accessed: 24 November 2020)

National Institute for Health and Care Excellence. Rituximab in combination with glucocorticoids for treating anti-neutrophil cytoplasmic antibody-associated vasculitis. Technology appraisal guidance TA308. 2014. https://www.nice.org.uk/guidance/ta308 (accessed 24 November 2020)

National Institute for Health and Care Excellence. Sepsis: recognition, diagnosis and early management. NICE guideline NG51. 2017 (revised version of 2016 document). https://www.nice.org.uk/guidance/ng51 (accessed: 24 November 2020)

National Kidney Foundation. Focal segmental glomerulosclerosis (FSGS). 2019. https://www.kidney.org/atoz/content/focal (accessed 24 November 2020)

Naz N, Chiu Y, Edwards M, Redfern A. Investigating whether socio-economic factors influence incidence of vasculitis in West Cheshire, UK. Rheumatology. 2019; 58 https://doi.org/10.1093/rheumatology/kez059.031

Pearce F, Lanyon P, Grainge M Incidence of ANCA-associated vasculitis in a UK mixed ethnicity population. Rheumatology. 2016; 55:(9)1656-1663 https://doi.org/10.1093/rheumatology/kew232

Peces R, de Sousa E, Peces C. Renal biopsies in special situations. Nefrologia. 2011; 31:(6)627-629 https://doi.org/10.3265/Nefrologia.pre2011.Jun.10925

Pullen RL. Managing cutaneous vasculitis in a patient with lupus erythematosus. Dermatol Nurs. 2007; 19:(1)212-216

Ramírez-Assad C, Cardiel M, Flores-Suárez L. Validation of the Birmingham Vasculitis Activity Score (BVAS) and the Vasculitis Damage Index (VDI) in a population of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis in Mexico. La Presse Médicale. 2013; 42:(4,2) https://doi.org/10.1016/j.lpm.2013.02.203

Rang H, Ritter J, Flower R, Henderson G. Rang & Dale's pharmacology, 8th edn. London: Elsevier Health Sciences; 2015

Rhee RL, Davis JC, Ding L The utility of urinalysis in determining the risk of renal relapse in ANCA-associated vasculitis. Clin J Am Soc Nephrol. 2018; 13:(2)251-257 https://doi.org/10.2215/CJN.04160417

Scott DG, Watts RA. Systemic vasculitis: epidemiology, classification and environmental factors. Ann Rheum Dis. 2000; 59:(3)161-3 https://doi.org/10.1136/ard.59.3.161

APACHE II calculator. Acute physiology and chronic health evaluation score to predict hospital mortality. 2019. https://clincalc.com/IcuMortality/APACHEII.aspx (accessed 24 November 2020)

Seeliger B, Sznajd J, Robson JC Are the 1990 American College of Rheumatology vasculitis classification criteria still valid?. Rheumatology. 2017; 56:(7)1154-1161 https://doi.org/10.1093/rheumatology/kex075

Selamet U, Kovaliv YB, Savage CO, Harper L. ANCA-associated vasculitis: new options beyond steroids and cytotoxic drugs. Expert Opin Investig Drugs. 2007; 16:(5)689-703 https://doi.org/10.1517/13543784.16.5.689

Shang W, Ning Y, Xu X Incidence of cancer in ANCA-associated vasculitis: a meta-analysis of observational studies. PLOS One. 2015; 10:(5) https://doi.org/10.1371/journal.pone.0126016

Spearpoint P, Götte D, Deichmann A, Wengler M, Schoenermark M, Rutherford P. the economic burden of anca-associated vasculitis in Germany: a claims data study. Annals of the Rheumatic Diseases. 2019; 78:(622)

Specks U. Accurate relapse prediction in ANCA-associated vasculitis: the search for the holy grail. J Am Soc Nephrol. 2015; 26:(3)505-507 https://doi.org/10.1681/ASN.2014080817

Stassen PM, Derks RPH, Kallenberg CGM, Stegeman CA. Venous thromboembolism in ANCA-associated vasculitis—incidence and risk factors. Rheumatology. 2007; 47:(4)530-534 https://doi.org/10.1093/rheumatology/ken035

Steddon S, Ashman N, Chesser A, Cunningham J. The Oxford handbook of nephrology and hypertension, 2nd edn. Oxford: Oxford University Press; 2014

Suresh E. Diagnostic approach to patients with suspected vasculitis. Postgrad Med J. 2006; 82:(970)483-488 https://doi.org/10.1136/pgmj.2005.042648

Szekanecz Z, Koch AE. Chemokines and angiogenesis. Curr Opin Rheumatol. 2001; 13:(3)202-208 https://doi.org/10.1097/00002281-200105000-00009

Tatsis E, Reinhold-Keller E, Steindorf K, Feller AC, Gross WL. Wegener's granulomatosis associated with renal cell carcinoma. Arthritis Rheum. 1999; 42:(4)751-756 https://doi.org/10.1002/1529-0131(199904)42:4<751::AID-ANR19>3.0.CO;2-D

University Hospitals of Derby and Burton. Acute kidney injury clinical guidelines. 2019. https://tinyurl.com/y2m2eksw (accessed 24 November 2020)

University of Oxford. American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) diagnostic and classification criteria for primary systemic vasculitis. 2016. https://clinicaltrials.gov/ct2/show/NCT01066208 (accessed 24 November 2020)

van Paassen P, Cohen Tervaert J, Heeringa P. Mechanisms of vasculitis: how pauci-immune is anti-neutrophil cytoplasmic antibody-associated renal vasculitis?. Nephron Exp Nephrol. 2007; 105:(1)e10-e16 https://doi.org/10.1159/000096960

Watts RA, Scott DGI, Pusey CD, Lockwood CM. Vasculitis—aims of therapy. An overview. Rheumatology. 2000; 39:(3)229-232 https://doi.org/10.1093/rheumatology/39.3.229

Watts RA, Mooney J, Skinner J, Scott DG, Macgregor AJ. The contrasting epidemiology of granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis. Rheumatology (Oxford). 2012; 51:(5)926-31 https://doi.org/10.1093/rheumatology/ker454

Watts RA, Mooney J, Scott DGI. The epidemiology of vasculitis in the UK. Rheumatology. 2014; 53 https://doi.org/10.1093/rheumatology/keu129.008

Watts RA, Robson J, Pearce F. The global burden of anti-neutrophil cytoplasmic antibody vasculitis: high but unquantified. Rheumatology (Oxford). 2017; 56:(9)1439-1440 https://doi.org/10.1093/rheumatology/kew438

World Health Organization. Guillain–Barré syndrome. 2016. https://www.who.int/news-room/fact-sheets/detail/guillain-barr%C3%A9-syndrome (accessed 24 November 2020)

Wiik A. What you should know about PR3-ANCA. An introduction. Arthritis Res. 2000; 2:(4)252-254 https://doi.org/10.1186/ar96

Xiao H, Hu P, Falk RJ, Jennette JC. Overview of the Pathogenesis of ANCA-Associated Vasculitis. Kidney Dis. 2016; 1:(4)205-215 https://doi.org/10.1159/000442323

Yoshida N, Lino Y. Pathogenesis and diagnosis of otitis media with ANCA-associated vasculitis. Allergol Int. 2014; 63:(4)523-32 https://doi.org/10.2332/allergolint.14-RAI-0774

Anti-neutrophil cytoplasmic antibodies-associated vasculitis: a guide and case study

10 December 2020
Volume 29 · Issue 22

Abstract

Vasculitis is a relatively rare and poorly understood condition causing inflammation of the blood vessels, which in turn can affect a patient's respiratory and renal systems. In some cases, ocular involvement can cause loss of sight and hearing loss may also be a red flag for vasculitis, which, if not treated early, can cause complete hearing loss. Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a group comprising granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis (EGP). AAV is fatal if untreated and as increased risk escalates with age, coupled with a decline in renal function, these are the principal predictors of poor outcome. Vital roles for nursing vasculitis patients lie in managing inflammation and pain, as these distressing symptoms are prevalent in the disease. Because of the multiple complications that can occur with vasculitis, treatment-related information is a high priority for these patients. As nurses are well placed to deliver information, value lies in their role in reducing the negative impacts on treatment regimens and compliance that accompany patients' poor insight into their condition.

To help nurses increase their knowledge of vasculitis conditions, this article will discuss vasculitis, with a particular focus on anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), which comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis (EGP). It will examine the prevalence, causes and clinical symptoms, as well as discuss some of the treatment options available. This discussion will include the case study of a patient seen in clinical practice (Table 1). Because of the multiple complications that can occur with vasculitis, treatment-related information is a high priority for these patients. Nurses are well placed to deliver this to help reduce the negative impacts on treatment regimens and compliance, often problematic in this patient group.

BD=twice daily; OD=once daily; PO=orally; TDS=three times daily

The term vasculitis refers to a relatively rare and poorly understood set of conditions, causing inflammation of the blood vessels, and AAV is a sub-group within this collection (Ball et al, 2014). According to guidance from the National Institute for Health and Care Excellence (NICE) (2014), the majority of AAV affects small- and medium-sized blood vessels, commonly in the respiratory system or, as in the case of the patient described in this article, the renal system.

Register now to continue reading

Thank you for visiting British Journal of Nursing and reading some of our peer-reviewed resources for nurses. To read more, please register today. You’ll enjoy the following great benefits:

What's included

  • Limited access to clinical or professional articles

  • Unlimited access to the latest news, blogs and video content