Rapidly progressive glomerulonephritides (crescentic glomerulonephritides)


Authors: O. Zakiyanov 1,2;  J. Vachek 1,3;  V. Tesař 1
Authors‘ workplace: Klinika nefrologie VFN a 1. LF UK v Praze 1;  Ústav lékařské biochemie a laboratorní diagnostiky VFN a 1. LF UK v Praze 2;  Farmakologický ústav 1. LF UK v Praze 3
Published in: Kardiol Rev Int Med 2014, 16(1): 62-66
Category: Internal Medicine

Overview

Rapidly progressive glomerulonephritides (RPGN) are the most severe form of the nephritic syndromes. In this case, glomerular injury is so acute that irreversible renal failure develops over the course of a few days to weeks. RPGN usually presents as proteinuria < 3g/ day, hematuria with dysmorphic red blood cells and/ or red cell casts, with or without signs of systemic vasculitis. A specific finding upon kidney bio­psy is a crescent formation in more than 70% of glomeruli. The most common diseases presenting as RPGN are ANCA‑associated vasculitides, Goodpasture’s disease (anti‑renal nephritis), and the much less common immunocomplex glomerulonephritides. Treatment includes glucocorticoids, cyclophosphamide and novel therapies including targeted treatment (rituximab), sometimes in combination with plasma exchange.

Keywords:
rapidly progressive glomerulonephriti –  pulmorenal syndrome –  crescents –  renal failure –  cyclophosphamide –  plasma exchange


Sources

1. Davies DJ, Moran JE, Niall JF et al. Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology? Br Med J (Clin Res Ed) 1982; 285: 606.

2. Hall JB, Wadham BM, Wood CJ et al. Vasculitis and glomerulonephritis: a subgroup with an antineutrophil cytoplasmic antibody. Aust N Z J Med 1984; 14: 277– 278.

3. Tesař V, Shück O et al. Klinická nefrologie: Grada Avicenum: Praha 2006.

4. Little MA, Pusey CD. Rapidly progressive glomerulonephritis: current and evolving treatment strategies. J Nephrol 2004; Suppl 8: S10– S19.

5. Jennette JC. Rapidly progressive crescentic glomerulonephritis. Kidney Int 2003; 63: 1164– 1177.

6. Hellmark T, Segelmark M. Dia­gnosis and classification of Goodpasture's disease (anti‑GBM). J Autoimmun 2014. doi: 10.1016/ j.jaut.2014.01.024.

7. Alpers CE, Smith KD. Cryoglobulinemia and renal disease. Curr Opin Nephrol Hypertens 2008; 17: 243– 249. doi: 10.1097/ MNH.0b013e3282f8afe2.

8. D'Cruz D. Renal manifestations of the antiphospholipid syndrome. Curr Rheumatol Rep 2009; 11: 52– 60.

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

10. Jennette JC, Falk RJ, Bacon PA et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65: 1– 11. doi: 10.1002/ art.37715.

11. Ciferská H, Vachek J. Ledvinné manifestace u revmatických chorob. Revmatologie 2013; 5: 63– 69.

12. Little MA, Nazar L, Farrington K. Outcome in glomerulonephritis due to systemic small vessel vasculitis: effect of functional status and non‑vasculitic co‑ morbidity. Nephrol Dial Transplant 2004; 19: 356– 364.

13. Eisenberger U, Fakhouri F, Vanhille P et al. ANCA‑ negative pauci‑ immune renal vasculitis: histology and outcome. Nephrol Dial Transplant 2005; 20: 1392– 1399.

14. Levy JB, Turner AN, Rees AJ et al. Long‑term outcome of anti‑glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med 2001; 134: 1033– 1042.

15. Couser WG. Basic and translational concepts of immune‑ mediated glomerular diseases. J Am Soc Nephrol 2012; 23: 381– 399. doi: 10.1681/ ASN. 2011030304.

16. Tang W, McDonald SP, Hawley CM et al. Anti‑glomerular basement membrane antibody disease is an uncommon cause of end‑stage renal disease. Kidney Int 2013; 83: 503– 510. doi: 10.1038/ ki.2012.375.

17. Tang W, Bose B, McDonald SP et al. The outcomes of patients with ESRD and ANCA‑associated vasculitis in Australia and New Zealand. Clin J Am Soc Nephrol 2013; 8: 773– 780. doi: 10.2215/ CJN.08770812.

18. Chen YX, Chen N. Pathogenesis of rapidly progressive glomerulonephritis: what do we learn? Contrib Nephrol 2013; 181: 207– 215. doi: 10.1159/ 000348633.

19. Jones RB, Tervaert JW, Hauser T et al. Rituximab versus cyclophosphamide in ANCA‑associated renal vasculitis. N Engl J Med 2010; 363: 211– 220. doi: 10.1056/ NEJMoa0909169.

20. Stone JH, Merkel PA, Spiera R et al. Rituximab versus cyclophosphamide for ANCA‑associated vasculitis. N Engl J Med 2010; 363: 221– 232. doi: 10.1056/ NEJMoa0909905.

21. Jindal KK. Management of idiopathic crescentic and diffuse proliferative glomerulonephritis: evidence‑based recommendations. Kidney Int Suppl 1999; 70: S33– S40.

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