Pulmonary‑renal syndrome

Česká verzia

Authors: Zdenka Hrušková; Vladimír Tesař
Authors place of work: Klinika nefrologie 1. LF UK a VFN v Praze
Published in the journal: Vnitř Lék 2020; 66(5): 56-61
Category: Review Articles

Summary

Pulmonary syndrome is defined by occurrence of lung involvement (alveolar haemorrhage) in association with renal failure (with a typical crescentic necrotizing rapidly progressive glomerulonephritis). It is caused by an autoimmune disease, most frequently ANCA-associated vasculitides and anti-GBM (glomerular basement membrane) disease. Early establishment of the right diagnosis and immediate treatment are crucial for favourable prognosis of the patients. First choice therapy includes high-dose corticosteroids and cyclophosphamide, usually with plasma exchange added. Newer therapeutic possibilities include especially rituximab even though there is limited experience with its use in the settings of the most severe cases of pulmonary syndrome.

Keywords:

vasculitis – ANCA – anti‑glomerular basement membrane (anti‑GBM) antibodies – diffuse alveolar haemorrhage – rapidly progressive glomerulonephritis – rituximab

Zdroje

1. West SC, Arulkumaran N, Ind PW, et al. Pulmonary‑renal syndrome: a life threatening but treatable condition. Postgrad Med J 2013; 89: 274–283.

2. McCabe C, Jones Q, Nikolopoulou A, et al. Pulmonary‑renal syndromes: an update for respiratory physicians. Respir Med 2011; 105: 1413–1421.

3. Tesař V, Honsová E, Ryšavá R, et al. Glomerulopatie. In: Tesař V, Viklický O (eds.) Klinická nefrologie. Praha: Grada 2015; 91–164.

4. Hrušková Z, Tesař V. Rychle progredující glomerulonefritidy – novinky v léčbě. Postgraduální medicína 2015; 5: 27–32.

5. Moroni G, Ponticelli C. Rapidly progressive crescentic glomerulonephritis: Early treatment is a must. Autoimmun Rev 2014; 13: 723–729.

6. Greenhall GH, Salama AD. What is new in the management of rapidly progressive glomerulonephritis? Clin Kidney J 2015; 8: 143–150.

7. Xiao H, Hu P, Falk RJ, et al. Overview of the Pathogenesis of ANCA‑Associated Vasculitis. Kidney Dis (Basel) 2016; 1: 205–215.

8. Hruskova Z, Casian AL, Konopasek P, et al. Long‑term outcome of severe alveolar haemorrhage in ANCA‑associated vasculitis: a retrospective cohort study. Scand J Rheumatol 2013; 42: 211–214.

9. Yates M, Watts RA, Bajema IM, et al. EULAR/ERA‑EDTA recommendations for the management of ANCA‑associated vasculitis. Ann Rheum Dis 2016; 75: 1583–1594.

10. Casian A, Jayne D. Management of alveolar hemorrhage in lung vasculitides. Semin Respir Crit Care Med 2011; 32: 335–345.

11. Jennette JC, Falk RJ, Bacon PA, et al. 2012 Revised international Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis & Rheum 2013; 65: 1–11.

12. Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group. KDIGO clinical practice guideline for glomerulonephritis. Kidney Int Suppl 2012; 2: 139–274.

13. Ntatsaki E, Carruthers D, Chakravarty K, et al. BSR and BHPR guideline for the management of adults with ANCA‑associated vasculitis. Rheumatology (Oxford) 2014; 53: 2306–2309.

14. Tesar V, Hruskova Z. Conventional induction and maintenance treatment of Antineutrophil cytoplasmic antibodies‑associated vasculitis - still of value for our patients? Expert Opin Pharmacother 2015; 16: 1683–1702.

15. De Groot K, Harper L, Jayne DR, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody‑associated vasculitis: a randomized trial. Ann Intern Med 2009; 150: 670–680.

16. Pepper RJ, Chanouzas D, Tarzi R, et al. Intravenous cyclophosphamide and plasmapheresis in dialysis‑dependent ANCA‑associated vasculitis. Clin J Am Soc Nephrol 2013; 8: 219–224.

17. Harper L, Morgan MD, Walsh M, et al. Pulse versus daily oral cyclophosphamide for induction of remission in ANCA‑associated vasculitis: long‑term follow‑up. Ann Rheum Dis 2012; 71: 955–960.

18. Jayne D, Rasmussen N, Andrassy K, et al. European Vasculitis Study Group. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003; 349: 36–44.

19. Hiemstra TF, Walsh M, Mahr A, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody‑associated vasculitis: a randomized controlled trial. JAMA 2010; 304: 2381–2388.

20. Walsh M, Catapano F, Szpirt W, et al. Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta‑analysis. Am J Kidney Dis 2011; 57: 566–574.

21. Walsh M, Casian A, Flossmann O, et al. Long‑term follow‑up of patients with severe ANCA‑associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear. Kidney Int 2013; 84: 397–402.

22. Walsh M, Merkel PA, Peh CA, et al. Plasma exchange and glucocorticoid dosing in the treatment of anti‑neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial. Trials 2013; 14: 73.

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

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

25. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA‑associated vasculitis. N Engl J Med 2014; 371: 1771–1780.

26. Terrier B, Pagnoux C, Perrodeau É et al. Long‑term efficacy of remission‑maintenance regimens for ANCA‑associated vasculitides. Ann Rheum Dis 2018; 77: 1150–1156.

27. Geetha D, Hruskova Z, Segelmark M, et al. Rituximab for treatment of severe renal disease in ANCA associated vasculitis. J Nephrol 2015; 41: 296–301.

28. Pepper RJ, McAdoo SP, Moran SM, et al. A novel glucocorticoid‑free maintenance regimen for anti‑neutrophil cytoplasm antibody‑associated vasculitis. Rheumatology (Oxford) 2019; 58: 260–268.

29. McAdoo SP, Medjeral‑Thomas N, Gopaluni S, et al. Long‑term follow‑up of a combined rituximab and cyclophosphamide regimen in renal anti‑neutrophil cytoplasm antibody‑associated vasculitis. Nephrol Dial Transplant 2019; 34: 63–73.

30. Hellmark T, Segelmark M. Diagnosis and classification of Goodpasture’s disease (anti‑GBM). J Autoimmun 2014; 48–49: 108–112.

31. McAdoo SP, Tanna A, Hrušková Z, et al. Patients double‑seropositive for ANCA and anti‑GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single‑seropositive patients. Kidney Int 2017; 92: 693–702.