Issue 2

Kidney

Newsletter

Keeping you up to date with the latest exciting advances and discussions in nephrology

Clinical challenges in kidney disease management

ANCA-associated vasculitis (AAV) is a group of rare, severe autoimmune diseases affecting small-medium blood vessels throughout the body1–4

There are three subtypes of AAV, which predict the course of disease:2–4
GPA:

granulomatosis with polyangiitis (previously called Wegener’s disease)

MPA:

microscopic polyangiitis

EGPA:

eosinophilic granulomatosis with polyangiitis(previously called Churg–Strauss syndrome)

AAV may cause irreversible damage to vital organs, with the kidneys and lungs as major targets2–4

Manifestations of severe disease:4,5
EYES

(GPA: 7–8%)
Severe inflammation of the sclera

LUNGS

(GPA & MPA: 60–80%)
Alveolar hemorrhage

NERVES

(GPA: 25%; MPA: 35%)
Painful neuropathy

NOSE

(GPA: 50–95%; MPA: 2–30%)
Nasal/sinus ulceration and destruction

EARS

(GPA: 50–95%; MPA: 2–30%)
Deafness

KIDNEYS

(GPA: 60–80%; MPA: 80%)
Renal impairment/failure

You can explore aav, its treatment challenges, and more here Understand AAV

Renal involvement in AAV increases risk of ESRD and mortality6–10

A substantial proportion of patients require dialysis:

At remission induction6

16%

More patients need dialysis as disease progresses

Within 5 years of diagnosis:7,8

15–38%
increased mortality risk in renal AAV with PR3-ANCA (vs non-renal AAV) over 12 years (95% CI 0.81–5.27; p=0.13)9,10
increased mortality risk in renal AAV without PR3-ANCA (vs non-renal AAV) over 12 years (95% CI 2.36–14.57; p<0.0005)9,10

What are the barriers to optimizing
clinical care across the ckd continuum?

Achieving and sustaining full remission is the prime goal in AAV management to optimize patient outcomes.2,11 EULAR/ERA-EDTA guidelines define full remission as the absence of disease activity attributable to active disease*2

Response to current standard of care (GCs in combination with an immunosuppressant)
is variable and relapse remains common in GPA/MPA patients12–14
1 in 2 GPA/MPA patients fail to achieve and sustain remission at 12 months without the use of GCs12,13
Long-term and/or high-dose GCs result in increased organ damage and other negative effects such as depression, anxiety and weight gain15–17
67% of GPA/MPA patients experience potentially treatment-related damage, such as hypertension, osteoporosis, malignancy and diabetes (at a mean of 7 years post diagnosis)15
Current standard of care is not selective and doesn’t target the root cause of inflammation18
*Absence of disease activity attributable to active disease is validated using a recognized scoring tool such as BVAS.2,11

Targeting the underlying inflammatory process may be vital to improve remission rates and reduce treatment-related adverse events in GPA/MPA12,13,15,18

You can explore aav, its treatment challenges, and more here Understand AAV
References and footnotes

FOOTNOTES

AAV, ANCA-associated vasculitis; ANCA, anti-neutrophil cytoplasmic auto-antibody; BVAS, Birmingham Vasculitis Activity Score; EGPA, eosinophilic granulomatosis with polyangiitis; ERA-EDTA, European Renal Association-European Dialysis and Transplant Association; ESRD, end-stage renal disease; EULAR, European Alliance Of Associations For Rheumatology;
GC, glucocorticoid; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis.

 

 

REFERENCES

  1. Al-Hussain T, et al. Adv Anat Pathol 2017;24:226–34;
  2. Yates M, et al. Ann Rheum Dis 2016;75:1583–94;
  3. Jennette JC, et al. Arthritis Rheum 2013;65:1–11;
  4. Wallace ZS, Miloslavsky EM. BMJ 2020;368:m421;
  5. Pagnoux C. Eur J Rheumatol 2016;3:122–33;
  6. Rutherford PA, Götte D. EMJ Nephrol 2020;8[Suppl 4]:2–16;
  7. Mohammed AJ, Segelmark M. J Rheumatol 2014;14:1366–73;
  8. Takala JH, et al. Scand J Rheumatol 2011;40:283–8;
  9. Hejil C, et al. RMD Open 2017;3:e000435–10;
  10. Mahr A, et al. Ann Rheum Dis 2013;72:1003–10;
  11. Hellmich B, et al. Ann Rheum Dis 2007;66:605–17;
  12. Stone JH, et al. N Engl J Med 2010;363:221–32;
  13. Specks U, et al. N Engl J Med 2013;369:417–27;
  14. Charles P, et al. Ann Int Med 2020;10.7326/M19-3827;
  15. Robson J, et al. Ann Rheum Dis 2015;74:177–84;
  16. Little MA, et al. Ann Rheum Dis 2010;69:1036−43;
  17. Robson J, et al. Rheumatol Int 2018;38:675–82;
  18. Jennette JC, Nachman PH. Clin J Am Soc Nephrol 2017;12:1680–91.

You are now heading to a third-party website that we do
not operate. We are not responsible for the content of the
third-party website. You are advised to review its privacy policy
as it may differ from ours.

 

Are you sure you want to leave?

Confirm

Cancel