anca-associated-vasculitis
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Defining ANCA-Associated Vasculitis

Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is a group of rare diseases that affects small- to medium-sized blood vessels.1,2 Once affected, these blood vessels become inflamed, leading to reduced blood flow and potentially impaired organ function. The exact cause of ANCA-associated vasculitis is currently unknown, but it is believed to be a combination of genetic and environmental factors.1-3

Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are the most common types of AAV and manifest as chronic, progressive autoimmune diseases that impact predominantly small-to-medium blood vessels throughout the body.4-6

GPA: granulomatous inflammation affecting blood vessels involving the upper and lower respiratory tracts and the kidneys; other organs can also be affected7,8

MPA: vasculitis often affecting the kidneys, lungs, and skin7

When are GPA and MPA Considered to be Active and Severe?

The American College of Rheumatology/Vasculitis Foundation (ACR/VF) defines active and severe as:

ACTIVE

New, worsening, or persistent clinical signs and/or symptoms attributed to GPA or MPA that are not related to prior damage9

SEVERE

Vasculitis with life- or organ-threatening manifestations9

Approximately 80-90% of patients with ANCA-associated vasculitis present with renal- or other organ-threatening manifestations, which can be considered severe active disease.9,10

Learn more about severe active GPA and MPA including a potential treatment option

Examining the Mechanism of Disease

Both the humoral pathway and the alternative complement pathway drive inflammatory vascular injury in GPA and MPA.4,5

Mechanism of disease for severe active ANCA-associated vasculitis
mechanism-disease-mobile-webp

The interaction between C5a and one of its receptors, C5aR, plays a critical role in amplifying inflammation and vascular injury in GPA and MPA.3-5

Hear from a vasculitis expert discussing mechanisms of disease for GPA and MPA

Severe active GPA and MPA can present as heterogeneous manifestations8

GPA and MPA manifest differently
throughout the body:
Eye
Eyes/Mucous Membranes

Sudden visual loss, uveitis, blurred vision, scleritis, episcleritis, conjunctivitis, blepharitis, keratitis, retinal changes, significant proptosis, mouth ulcers, genital ulcers, adnexal inflammation8,12

Ear
Ears

Conductive hearing loss, sensorineural hearing loss8,12

Nose
Nose/Throat

Bloody nasal discharge, crusts, ulcers, granulomata, paranasal sinus involvement, subglottic stenosis8,12

Heart
Cardiovascular

Loss of pulses, valvular heart disease, pericarditis, ischemic cardiac pain, cardiomyopathy, congestive cardiac failure8,12

Lungs
Chest

Wheezing, nodules or cavities, pleural effusion, pleurisy, infiltrates, endobronchial involvement, massive hemoptysis, alveolar hemorrhage, respiratory failure8,12

Abdominal
Abdominal

Peritonitis, bloody diarrhea, ischemic abdominal pain8,12

Renal
Renal

Hypertension, proteinuria, hematuria, changes in serum creatinine and creatinine clearance8,12

Skin
Cutaneous

Infarct, purpura, ulcer, gangrene, other skin vasculitis8,12

Nervous system
Nervous System

Headache, meningitis, seizures, cerebrovascular accident, organic confusion, spinal cord lesion, cranial nerve palsy, sensory peripheral neuropathy, mononeuritis multiplex8,12

Person icon
General

Myalgia, arthralgia, arthritis, fever, weight loss8,12

Please note these are just some of the signs and symptoms of severe active GPA or MPA. Severe active GPA or MPA can present differently in each patient.

Multisystem clinical assessment is critical for diagnosis2

Stethoscope and checklist Clinical Assessment
  • A detailed history and physical examination are key to disease management13
  • Due to the multisystemic nature of GPA and MPA, awareness of signs/symptoms of disease activity across all organ systems is important for diagnosis2,14
Test tube Serology
  • While a positive ANCA result can provide additional support for diagnosis, up to 20% of patients with GPA and MPA are ANCA-negative15
  • A positive ANCA can also be found in other conditions without associated vasculitis such as autoimmune hepatitis, ulcerative colitis, hepatitis C, HIV, or infectious endocarditis15
  • Serology alone cannot be used to diagnose GPA or MPA14
Syringe and petri dish Biopsy
  • Biopsy can be confirmatory, but treatment should not necessarily be delayed simply to get a biopsy2,15

Hear from a vasculitis expert discussing the diagnosis of GPA and MPA

Learn more about a treatment option for severe active GPA and MPA

Humoral and alternative complement pathways drive inflammatory vascular injury in GPA and MPA6,16,17

Inflammatory vascular injry in severe active GPA and MPA
humoral-inflammatory-mobile-webp

Opportunities to further care may start with understanding the humoral and complement mediated pathway of GPA and MPA pathophysiology6,16

Despite improvements in treating severe active GPA and MPA, challenges in care continue to exist from both disease activity and treatment4

ClickTap the icons below to learn more about the potential challenges healthcare professionals face when treating severe active GPA and MPA.

Stethoscope and clock
Timely Diagnosis2
Achieving remission
Achieving Remission22
Preventing relapse
Preventing Relapse23
Exclamation point
Glucocorticoid Toxicity24,25
Up and down arrows on human body outline
Impact on Organ Function26
Meditating human figure
Effect on Quality of Life25

Hear from a vasculitis expert discussing challenges in care of GPA and MPA

Considerations in Care for Your Patients With Severe Active GPA or MPA

Consider Disease and Treatment Burdens in GPA and MPA

To somebody that’s…diagnosed, the first thing I would tell them is ask a lot of questions. Fight for yourself because you’ve got to take care of your whole being.

– Brandon, living with GPA

Patients with GPA or MPA may have persistent symptoms,
which can negatively impact their quality of life27-29
Only about 25%

of patients are satisfied with their medication’s ability to control symptoms while maintaining a good quality of life30,*

For about 50%

of patients, even in remission, vasculitis symptoms are debilitating30,*

*

According to an online, self-administered survey of 100 patients with GPA or MPA from July 21-August 25, 2022.

Achieving the goal of remission while balancing burden of treatment toxicity can be challenging31
Syringe Syringe
Glucocorticoid toxicity can occur across multiple organ systems over time24
Human body outline Human body outline

BMI = Body Mass Index.

To somebody that’s…diagnosed, the first thing I would tell them is ask a lot of questions. Fight for yourself because you’ve got to take care of your whole being.

– Brandon, living with GPA

Hear from a vasculitis expert discussing goals of care for GPA and MPA

Facilitating open conversation with your patient

Open conversation between a patient and their HCP is crucial when it comes to treating conditions like severe active GPA or MPA, as each may have different expectations of what successful treatment looks like.27,30 Here we’ve provided some useful topics to help you get the most out of these conversations with your patient.

Your patients may have symptoms that impact their ability to communicate effectively with you, including hearing loss.8 Be sure to consider your patient’s abilities when talking with them, such as facing your patient so they can fully understand you and so that you are both able to read each other’s body language.

  • What response is your patient hoping to achieve from their treatment?
  • When does your patient expect to start seeing results from their treatment?
  • Has your patient’s quality of life improved or worsened since they started treatment?
  • If your patient could change one thing about their current treatment, what might it be?
  • Does your patient understand what GPA or MPA are and the effects it may be having on their body?
  • Help ensure your patient is aware of the potential side effects that could arise from their current treatment regimen

Hear what patients have to say about their own journey with GPA or MPA

Opportunities to further care

Watch a national broadcast Amgen® put together around opportunities to further care in AAV, with a focus on GPA and MPA.

Downloadable resources are available to help you and your patient

Resources

Birmingham Vasculitis Activity Score Resource

The Birmingham Vasculitis Activity Score (BVAS) is a validated tool used to measure disease activity in major studies of vasculitis, including ANCA-associated vasculitis. It captures a broad spectrum of clinical manifestations for new, worsening, or persistent disease across 9 organ systems (1 general and 8 tissue-specific).

Resources

ANCA101 Education Resource

This resource provides a summary of the ANCA-associated vasculitis disease mechanism, severe active GPA and MPA manifestations, and challenges with management.

Your patient is not alone

The following organizations provide education, support, and resources that may help your patient throughout their treatment journey.

Vasculitis Foundation logo
The Vasculitis Foundation

The Vasculitis Foundation supports, inspires, and empowers people and families living with vasculitis through a wide range of education, research, clinical, and awareness initiatives.

Visit them at vasculitisfoundation.org
NORD® logo
NORD (National Organization for Rare Disorders)

NORD (National Organization for Rare Disorders) is a patient advocacy organization committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and patient services.

Visit them at rarediseases.org
American Kidney Fund® logo
American Kidney Fund

The American Kidney Fund (AKF) works on behalf of Americans living with and at risk of kidney disease, with programs that support people from prevention through transplant and beyond.

Visit them at kidneyfund.org

References: 1.Tekin Mİ, Özdal MPC. Acta Medica. 2021;52(4):257-263. 2.Yates M, Watts R. Clin Med (Lond). 2017;17(1):60-64. 3. Shochet L, Holdsworth S, Kitching AR. Front Immunol. 2020;11:525. 4. Kitching AR, Anders H-J, Basu N, et al. Nat Rev Dis Primers. 2020;6(1):71. 5. Al-Hussain T, Hussein MH, Conca W, Al Mana H, Akhtar M. Adv Anat Pathol. 2017;24(4):226-234. 6. Jennette JC, Falk RJ, Bacon PA, et al. Arthritis Rheum 2013;65(1):1-11. doi: 10.1002/art.37715 7. Langford C. Cleve Clin J Med. 2012;79(suppl3):S3-15. 8. Supplement to: Jayne DRW, Merkel PA, Schall TJ, Bekker P; ADVOCATE Study Group. N Engl J Med. 2021;384(7):599-609. 9. Chung SA, Langford CA, Maz M, et al. Arthritis Rheumatol. 2021;73(8):1366-1383. 10. Lamprecht P, Kerstein A, Klapa S, et al. Front Immunol. 2018;9:1-10. 11. Jennette JC, Nachman PH. Clin J Am Soc Nephrol. 2017;12(10):1680-1691. 12. Mukhtyar CB. In: Watts RA, Scott DGI, eds. Vasculitis in Clinical Practice. Springer; 2010:13-19. 13. Qasim A, Patel JB. In: StatPearls [Internet]. StatPearls Publishing; 2023. Accessed October 3, 2023. https://www.ncbi.nlm.nigh.bov/books/NBK554372/ 14. Hunter RW, Welsh N, Farrah TE, et al. BMJ. 2020;369:m1070. 15. Pagnoux C. Eur J Rheumatol. 2016;3(3):122-133. 16. Chen M, Jayne DRW, Zhao M-H. Nat Rev Nephrol. 2017;13(6):359-367. 17. Jones RB, Ferraro AJ, Chaudhry AN, et al. Arthritis Rheum. 2009;60(7):2156-2168. 18. Winkelstein A. Blood. 1973;41(2):273-284. 19. Eickenberg S, Mickholz E, Jung E, et al. Arthritis Res Ther. 2012;14(3):R110. 20. Ogino MH, Prasanna T. In: StatPearls [Internet]. StatPearls Publishing; 2023. Accessed October 3, 2023. https://www.ncbi.nlm.nigh.bov/books/NBK5553087/ 21. Mohammadi O, Kassim TA. In: StatPearls [Internet]. StatPearls Publishing; 2023. 22. Stone JH, Merkel PA, Spiera R, et al; RAVE-ITN Research Group. N Engl J Med. 2010;363(3):221-232. 23. Terrier B, Pagnoux C, Perrodeau E, et al; French Vasculitis Study Group. Ann Rheum Dis. 2018;77(8):1151-1157. 24. Stone JH, McDowell PJ, Jayne DRW, et al. Semin Arthritis Rheum. 2022;55:152010 25. King C, Harper L. Current Treatment Options in Rheumatology. 2017;3(4):230-243. doi.org/10.1007/s40674-017-0082-y 26. Robson J, Doll H, Suppiah R, et al. Rheumatology (Oxford). 2015;54(3):471-481. 27. Robson JC, Dawson J, Cronholm PF, et al. Patient Relat Outcome Meas. 2018;9:17-34. 28. Jayne DRW, Merkel PA, Schall TJ, Bekker P; ADVOCATE Study Group. N Engl J Med. 2021;384(7):599-609. 29. Neumann I. Rheumatology (Oxford). 2020;59(suppl 3):iii60-iii67. 30. Data on file, Amgen; Harris Poll [91973]; 2022. 31. Furuta S, Nakagomi D, Kobayashi Y, et al. JAMA. 2021;325(21):2178-2187.

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Meditating human figure
Effect on Quality of Life

“Quality of Life” describes how well you’re doing and feeling day-to-day. If your treatment plan is working for you, it can improve your quality of life.

Only about 1/4 of people are satisfied with their medication’s ability to control symptoms while maintaining a good quality of life.*

*According to an online, self-administered survey of 100 patients with GPA or MPA from July 21-August 25, 2022.

Achieving Full Remission During Induction Remains a Challenge for Many Patients With GPA or MPA

Response to induction therapy is variable and many patients do not achieve full clinical remission (defined as a BVAS score of 0 with the patient off glucocorticoids)1

RAVE Clinical Trial: Patients Achieving Full Clinical Remission at 6 Months1

Challenges of Achieving Full Remission During Induction for Patients with Severe Active GPA and MPA Challenges of Achieving Full Remission During Induction for Patients with Severe Active GPA and MPA
*

Received one to three pulses of methylprednisolone (1000 mg each) followed by prednisone 1 mg/kg/day.

BVAS = Birmingham Vasculitis Activity Score;
CYC = cyclophosphamide; Q1W = weekly; RTX = rituximab.

Reference: 1. Stone JH, Merkel PA, Spiera R, et al. N Engl J Med. 2010;363(3):221-232.
Relapses Continue as Many Patients Struggle to Sustain Remission

MAINRITSAN-11,2

N = 115 patients with GPA or MPA in complete remission (BVAS = 0) after combined glucocorticoids and “pulse” intravenous CYC

AZA arm (n = 58) received:

2 mg/kg/day until month 12 1.5 mg/kg/day until month 18 1 mg/kg/day until month 22

RTX arm (n = 57) received:

500 mg on days 0 and 14, then months 6, 12, and 18

Prednisone treatment:

  • Started at 1 mg/kg/day followed by gradual tapering and then kept at a low dose (approximately 5 mg per day) for at least 18 months after randomization*

Major relapse: reappearance or worsening of disease with BVAS >0 and involvement of at least one major organ, a life-threatening manifestation, or both.


Minor relapse: reappearance or worsening of disease with BVAS >0, not corresponding to a major relapse but requiring mild treatment intensification.

MAINRITSAN-1 Relapse Rates1

Relapses May Continue as Patients Struggle to Sustain Remission
*

AZA = azathioprine; RTX = rituximab. Prednisone dose tapering and the decision to stop prednisone after month 18 were left to each site investigator’s discretion. AZA = azathioprine; BVAS = Birmingham Vasculitis Activity Score; CYC = cyclophosphamide; RTX = rituximab.

Patients do still experience relapse when receiving therapy, and as a result, many still receive glucocorticoids long term1,3
References: 1. Terrier B, Pagnoux C, Perrodeau E, et al; French Vasculitis Study Group.em Ann Rheum Dis. 2018;77(8):1151-1157. 2. Guillevin L, Pagnoux C, Karras A, et al; French Vasculitis Study Group. N Engl J Med. 2014;371(19):1771-1780. 3. Miloslavsky EM, Specks U, Merkel PA, et al. Arthritis Rheumatol. 2015;67(6):1629-1636.
Toxicity From Glucocorticoids Has Been Well Established

Even at lower doses, glucocorticoids can be associated with substantial morbidity1-3

Increased Infections2

Patients receiving glucocorticoid therapy for >6 months have a significantly greater incidence of infections (vs patients not using glucocorticoids after 6 months)*

Glucocorticoid Toxicity
*

Patients (N = 147) enrolled in the ANCA disease registry of the GDCN, with an initial diagnosis between 2000 and 2009, and remission on or off therapy attained for at least 1 month.

BMI = body mass index; GDCN = Glomerular Disease Collaborative Network.

Glucocorticoid Toxicity Index (GTI) Assesses Glucocorticoid Toxicity That Can Occur Across Multiple Organ Systems Over Time4

Glucocorticoid Toxicity

BMI = body mass index.

References: 1. Pujades-Rodriguez M, Morgan AW, Cubbon RM, et al. PLoS Med. 2020;17(12):e1003432. 2. McGregor JG, Hogan SL, Hu Y, et al. Clin J Am Soc Nephrol. 2012;7(2):240-247. 3. Waljee AK, Rogers MAM, Lin P, et al. BMJ. 2017;357:j1415. 4. Stone JH, McDowell PJ, Jayne DRW, et al. The glucocorticoid toxicity index: measuring change in glucocorticoid toxicity over time. Semin Arthritis Rheum. 2022;55:152010.
Disease Activity Can Impact Renal Function in Patients With GPA or MPA1,2

Renal impairment is common with many patients1,2

Disease Activity Impact on Renal Function in Patients with Severe Active GPA and MPA

GFR = glomerular filtration rate; LTFU = long-term follow-up.

Reference: 1. Robson J, Doll H, Suppiah R, et al. Rheumatology (Oxford). 2015;54(3):471-481. 2. Robson J, Doll H, Suppiah R, et al. Ann Rheum Dis. 2015;74:177-184.
Disease Activity and Treatment Toxicity Continue to Place a Burden
on Patients Who Prioritize QoL as Treatment Goal1

QoL in Patients With AAV vs the General Population2,3

Burden of Disease in Patients with Severe Active GPA and MPA
Burden of Disease in Patients with Severe Active GPA and MPA

AAV = ANCA-associated vasculitis; QoL = quality of life.

References: 1. Basu N, Jones GT, Fluck N, et al. Rheumatology (Oxford). 2010;49(7):1383-1390. 2. Benarous L, Terrier B, Laborde-Casterot H, et al. Clin Exp Rheumatol. 2017;35(suppl 103):40-46. 3. Basu N, McClean A, Harper L, et al. Ann Rheum Dis. 2014;73(1):207-211. 4. Basu N, McClean A, Harper L, et al. Rheumatology (Oxford). 2014;53(5):953-956. 5. Raimundo K, Farr AM, Kim G, et al. J Rheumatol. 2015;42(12):2383-2391. 6. Robson JC, Dawson J, Cronholm PF, et al. Patient Relat Outcome Meas. 2018;9:17-34.
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