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How do your AAV patients present?
Ears and auditory4,5
GPA>MPA
Hearing loss, ear ache
Eyes4, 5
GPA>>MPA
Red, painful eyes
Nose and sinuses2,4,5
GPA>>MPA
Saddle nose deformity,
nasal septum deformity,
sinusitis
Skin2,5
GPA>MPA
Rashes, purpura, lesions
Lungs and upper airways2,4–6
GPA>MPA
Coughing, pain, lung
nodules (seen in X rays),
alveolar haemorrhage
Nervous system2,4,5
CNS (GPA=MPA), PNS
(MPA>GPA)
Mononeuritis,
paraesthesia, other
peripheral neuropathy
Kidneys4,5
MPA>GPA
Blood in the urine
QoL impairment7
Similar to other chronic diseases and includes anxiety, fatigue, sleep disturbance, depression and pain
Ears and auditory4,5
GPA>MPA
Hearing loss, ear ache
Eyes4, 5
GPA>>MPA
Red, painful eyes
Nose and sinuses2,4,5
GPA>>MPA
Saddle nose deformity,
nasal septum deformity,
sinusitis
Skin2,5
GPA>MPA
Rashes, purpura, lesions
Lungs and upper airways2,4–6
GPA>MPA
Coughing, pain, lung
nodules (seen in X rays),
alveolar haemorrhage
Nervous system2,4,5
CNS (GPA=MPA), PNS
(MPA>GPA)
Mononeuritis,
paraesthesia, other
peripheral neuropathy
Kidneys4,5
MPA>GPA
Blood in the urine
QoL impairment7
Similar to other chronic diseases and includes anxiety, fatigue, sleep disturbance, depression and pain
There is a need for a new and more targeted approach to AAV (GPA/MPA) treatment8–11
AAV (GPA/MPA) is a severe, relapsing disease that can cause irreversible damage to multiple organs1-3
Despite treatment, many AAV patients do not achieve or sustain remission and the risk of relapse persists, further exacerbating organ damage12–14
Established therapies for AAV (GPA/MPA) have relied on non-specic immunosuppressants alongside
high-dose and/or long-term GCs, which add to the burden of disease11,14–18
Activation of the alternative complement pathway plays a key role in AAV and no established therapies address this key driver of vascular inflammation9,11,19
AAV, ANCA-associated vasculitis; ANCA, anti-neutrophil cytoplasmic antibody; CNS, central nervous system; GC, glucocorticoid; GPA, granulomatosis with polyangiitis; MOA, mechanism of action; MPA, microscopic polyangiitis; PNS, peripheral nervous system; QoL, quality of life.
References
1. Wallace ZS, Miloslavsky EM. BMJ 2020;368:m421. 2. Pagnoux C. Eur J Rheumatol 2016;3(3):122–33. 4. Al Hussain T, et al. Adv Anat Pathol 2017;24(4):226–34. 4. Hunter RW, et al. BMJ 2020;369:m1070. 5. Kitching AR, et al. Nat Rev Dis Primers 2020;6(1):71. 6. Quartuccio L, et al. J Autoimmun 2020;108:102397. 7. Basu N, et al. Ann Rheum Dis 2014;73(1):207–11. 8. Hutton HL, et al. Semin Nephrol 2017;37(5):418–35. 9. Jennette JC, Nachman PH. Clin J Am Soc Nephrol 2017;12(10):1680–91. 10. Bekker P, et al. PLoS One 2016;11(10):e0164646. 11. Lamprecht P, et al. EMJ Rheumatol 2021;8(1):36–42. 12. Stone JH, et al. N Engl J Med 2010;363(3):221–32. 13. Specks U, et al. N Engl J Med 2013;369(5):417–27. 14. Robson J, et al. Rheumatology (Oxford) 2015;54(3):471–81. 15. Yates M, et al. Ann Rheum Dis 2016;75(9):1583–94. 16. Little MA, et al. Ann Rheum Dis 2010;69(6):1036–43. 17. McGregor JG, et al. Clin J Am Nephrol 2012;7(2):240–7. 18. Turnbull J, Harper L. Best Pract Res Clin Rheumatol 2009;23(3):391–401. 19. Liberman AC, et al. Front Endocrinol (Lausanne) 2018;9:235
