Expert Statements on the Standard of Care in Critically Ill Adult Patients With Atypical Hemolytic Uremic Syndrome

Chest

Volumn 152, Issue 2, 2017, Pages 424-434

  Azoulay, Elie ^a   Knoebl, Paul ^b   Garnacho Montero, José ^c   Rusinova, Katerina ^d   Galstian, Gennadii ^e   Eggimann, Philippe ^f   Abroug, Fekri ^g   Benoit, Dominique ^h   von Bergwelt Baildon, Michael ⁱ   Wendon, Julia ^j   Scully, Marie ^k  

^a SAINT LOUIS HOSPITAL   (France)

^b MEDICAL UNIVERSITY OF VIENNA   (Austria)

^c HOSPITAL UNIVERSITARIO VIRGEN MACARENA   (Spain)

^d CHARLES UNIVERSITY   (Czech Republic)

^e NATIONAL RESEARCH CENTER FOR HEMATOLOGY   (Russian Federation)

^f LAUSANNE UNIVERSITY HOSPITAL   (Switzerland)

^g Fattouma Bourguiba Teaching Hospital Monastir   (Tunisia)

^h GHENT UNIVERSITY HOSPITAL   (Belgium)

ⁱ UNIVERSITY HOSPITAL OF COLOGNE   (Germany)

^j KING S COLLEGE HOSPITAL   (United Kingdom)

^k UNIVERSITY COLLEGE LONDON   (United Kingdom)

more..

Author keywords

atypical hemolytic uremic syndrome; eculizumab; intensive care; organ failure; plasma exchange; thrombocytopenia; thrombotic microangiopathy

Indexed keywords

ANGIOTENSIN RECEPTOR ANTAGONIST; ANTIBIOTIC AGENT; COMPLEMENT FACTOR; DIPEPTIDYL CARBOXYPEPTIDASE INHIBITOR; ECULIZUMAB; ADAMTS13 PROTEIN, HUMAN; COMPLEMENT; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ANTIBIOTIC PROPHYLAXIS; CRITICALLY ILL PATIENT; DIAGNOSTIC PROCEDURE; DIFFERENTIAL DIAGNOSIS; DRUG EFFICACY; DRUG TOLERABILITY; GENETIC SCREENING; HEMOLYTIC UREMIC SYNDROME; HUMAN; HYPERTENSION; MENINGOCOCCOSIS; PATHOGENESIS; PATIENT CARE; PHASE 2 CLINICAL TRIAL (TOPIC); PNEUMOCOCCAL HEMOLYTIC UREMIC SYNDROME; PRIORITY JOURNAL; PROTEIN BLOOD LEVEL; REVIEW; SHIGA TOXIN PRODUCING ESCHERICHIA COLI HEMOLYTIC UREMIC SYNDROME; THROMBOTIC THROMBOCYTOPENIC PURPURA; TREATMENT DURATION; ADULT; ATYPICAL HEMOLYTIC UREMIC SYNDROME; HEALTH CARE QUALITY; INTENSIVE CARE; METABOLISM; PLASMA EXCHANGE; PROCEDURES; STANDARDS;

ADAMTS13 PROTEIN; ADULT; ATYPICAL HEMOLYTIC UREMIC SYNDROME; COMPLEMENT SYSTEM PROTEINS; CRITICAL CARE; DIAGNOSIS, DIFFERENTIAL; GENETIC TESTING; HUMANS; PLASMA EXCHANGE; QUALITY OF HEALTH CARE; STANDARD OF CARE;

EID: 85027488211     PISSN: 00123692     EISSN: 19313543     Source Type: Journal    
DOI: 10.1016/j.chest.2017.03.055     Document Type: Review

References (56)

1. Campistol, J.M., Arias, M., Ariceta, G., et al. An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nefrologia 35:5 (2015), 421–447.
2. Franchini, M., Atypical hemolytic uremic syndrome: from diagnosis to treatment. Clin Chem Lab Med 53:11 (2015), 1679–1688.
3. Loirat, C., Fremeaux-Bacchi, V., Atypical hemolytic uremic syndrome. Orphanet J Rare Dis, 6, 2011, 60.
4. Fremeaux-Bacchi, V., Fakhouri, F., Garnier, A., et al. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol 8:4 (2013), 554–562.
5. Mele, C., Remuzzi, G., Noris, M., Hemolytic uremic syndrome. Semin Immunopathol 36:4 (2014), 399–420.
6. Legendre, C.M., Licht, C., Loirat, C., Eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med 369:14 (2013), 1379–1380.
7. Riedl, M., Fakhouri, F., Le Quintrec, M., et al. Spectrum of complement-mediated thrombotic microangiopathies: pathogenetic insights identifying novel treatment approaches. Semin Thromb Hemost 40:4 (2014), 444–464.
8. Noris, M., Mele, C., Remuzzi, G., Podocyte dysfunction in atypical haemolytic uraemic syndrome. Nat Rev Nephrol 11:4 (2015), 245–252.
9. Hofer, J., Rosales, A., Fischer, C., Giner, T., Extra-renal manifestations of complement-mediated thrombotic microangiopathies. Front Pediatr, 2, 2014, 97.
10. Noris, M., Caprioli, J., Bresin, E., et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol 5:10 (2010), 1844–1859.
11. Mannucci, P.M., Understanding organ dysfunction in thrombotic thrombocytopenic purpura. Intensive Care Med 41:4 (2015), 715–718.
12. Scully, M., Goodship, T., How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome. Br J Haematol 164:6 (2014), 759–766.
13. Reeves, H.M., Winters, J.L., The mechanisms of action of plasma exchange. Br J Haematol 164:3 (2014), 342–351.
14. Scully, M., Hunt, B.J., Benjamin, S., et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol 158:3 (2012), 323–335.
15. Cofiell, R., Kukreja, A., Bedard, K., et al. Eculizumab reduces complement activation, inflammation, endothelial damage, thrombosis, and renal injury markers in aHUS. Blood 125:21 (2015), 3253–3262.
16. Walle, J.V., Delmas, Y., Ardissino, G., Wang, J., Kincaid, J.F., Haller, H., Improved renal recovery in patients with atypical hemolytic uremic syndrome following rapid initiation of eculizumab treatment. J Nephrol 30:1 (2017), 127–134.
17. Zuber, J., Le, Q.M., Sberro-Soussan, R., Loirat, C., Fremeaux-Bacchi, V., Legendre, C., New insights into postrenal transplant hemolytic uremic syndrome. Nat Rev Nephrol 7:1 (2011), 23–35.
18. Sawai, T., Nangaku, M., Ashida, A., et al. Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society. Pediatr Int 56:1 (2014), 1–5.
19. Loirat, C., Fakhouri, F., Ariceta, G., et al. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol 31:1 (2016), 15–39.
20. Nester, C.M., Managing atypical hemolytic uremic syndrome: chapter 2. Kidney Int 87:5 (2015), 882–884.
21. Loirat, C., Saland, J., Bitzan, M., Management of hemolytic uremic syndrome. Presse Med 41:3 Pt 2 (2012), e115–e135.
22. Knobl, P.N., Treatment of thrombotic microangiopathy with a focus on new treatment options. Hamostaseologie 33:2 (2013), 149–159.
23. Greenbaum, L.A., Atypical hemolytic uremic syndrome. Adv Pediatr 61:1 (2014), 335–356.
24. Hunt, B.J., Bleeding and coagulopathies in critical care. N Engl J Med, 370(22), 2014, 2153.
25. Sallee, M., Ismail, K., Fakhouri, F., et al. Thrombocytopenia is not mandatory to diagnose haemolytic and uremic syndrome. BMC Nephrol, 14, 2013, 3.
26. Nester, C.M., Barbour, T., de Cordoba, S.R., et al. Atypical aHUS: state of the art. Mol Immunol 67:1 (2015), 31–42.
27. Laurence, J., Atypical hemolytic uremic syndrome (aHUS): making the diagnosis. Clin Adv Hematol Oncol 10:10 suppl 17 (2012), 1–12.
28. Shenkman, B., Einav, Y., Thrombotic thrombocytopenic purpura and other thrombotic microangiopathic hemolytic anemias: diagnosis and classification. Autoimmun Rev 13:4-5 (2014), 584–586.
29. Feng, S., Eyler, S.J., Zhang, Y., et al. Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome. Blood 122:8 (2013), 1487–1493.
30. Campistol, J.M., Arias, M., Ariceta, G., et al. An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nefrologia 33:1 (2013), 27–45.
31. Nester, C.M., Thomas, C.P., Atypical hemolytic uremic syndrome: what is it, how is it diagnosed, and how is it treated?. Hematology Am Soc Hematol Educ Program 2012 (2012), 617–625.
32. Contreras, E., de la Rubia, J., Del Rio-Garma, J., Diaz-Ricart, M., Garcia-Gala, J.M., Lozano, M., Diagnostic and therapeutic guidelines of thrombotic microangiopathies of the Spanish Apheresis Group. [in Spanish] Med Clin (Barc), 144(7), 2015, 331.
33. Tsai, H.M., Untying the knot of thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome. Am J Med 126:3 (2013), 200–209.
34. Coppo, P., Schwarzinger, M., Buffet, M., et al. Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience. PLoS One, 5(4), 2010, e10208.
35. Zafrani, L., Mariotte, E., Darmon, M., et al. Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma ADAMTS13 activity. J Thromb Haemost 13:3 (2015), 380–389.
36. Noris, M., Remuzzi, G., Cardiovascular complications in atypical haemolytic uraemic syndrome. Nat Rev Nephrol 10:3 (2014), 174–180.
37. Tsai, H.M., Thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: an update. Hematol Oncol Clin North Am 27:3 (2013), 565–584.
38. Rosove, M.H., Thrombotic microangiopathies. Semin Arthritis Rheum 43:6 (2014), 797–805.
39. Caliskan, Y., Ozluk, Y., Celik, D., et al. The clinical significance of uric acid and complement activation in the progression of IgA nephropathy. Kidney Blood Press Res 41:2 (2016), 148–157.
40. Taylor, C.M., Machin, S., Wigmore, S.J., Goodship, T.H., Clinical practice guidelines for the management of atypical haemolytic uraemic syndrome in the United Kingdom. Br J Haematol 148:1 (2010), 37–47.
41. Noris, M., Remuzzi, G., Atypical hemolytic-uremic syndrome. N Engl J Med 361:17 (2009), 1676–1687.
42. Kavanagh, D., Goodship, T.H., Richards, A., Atypical hemolytic uremic syndrome. Semin Nephrol 33:6 (2013), 508–530.
43. Studt, J.D., Binet, I., Nair, G., Schanz, U., Heparin-induced thrombocytopenia associated with thrombotic microangiopathy. Hamostaseologie 33:2 (2013), 160–163.
44. Licht, C., Ardissino, G., Ariceta, G., et al. The global aHUS registry: methodology and initial patient characteristics. BMC Nephrol, 16, 2015, 207.
45. Cataland, S.R., Wu, H.M., How I treat: the clinical differentiation and initial treatment of adult patients with atypical hemolytic uremic syndrome. Blood 123:16 (2014), 2478–2484.
46. Legendre, C.M., Licht, C., Muus, P., et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med 368:23 (2013), 2169–2181.
47. Licht, C., Greenbaum, L.A., Muus, P., et al. Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies. Kidney Int 87:5 (2015), 1061–1073.
48. Greenbaum, L.A., Fila, M., Ardissino, G., et al. Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome. Kidney Int 89:3 (2016), 701–711.
49. Fakhouri, F., Hourmant, M., Campistol, J.M., et al. Terminal complement inhibitor eculizumab in adult patients with atypical hemolytic uremic syndrome: a single-arm, open-label trial. Am J Kidney Dis 68:1 (2016), 84–93.
50. Picard, C., Burtey, S., Bornet, C., Curti, C., Montana, M., Vanelle, P., Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome. Pathol Biol (Paris) 63:3 (2015), 136–143.
51. Schmidtko, J., Peine, S., El-Housseini, Y., Pascual, M., Meier, P., Treatment of atypical hemolytic uremic syndrome and thrombotic microangiopathies: a focus on eculizumab. Am J Kidney Dis 61:2 (2013), 289–299.
52. Alexion. Soliris summary of product characteristics. 2016. https://www.medicines.org.uk/emc/medicine/19966. Accessed May 22, 2017.
53. Bouts, A., Monnens, L., Davin, J.C., Struijk, G., Spanjaard, L., Insufficient protection by Neisseria meningitidis vaccination alone during eculizumab therapy. Pediatr Nephrol 26:10 (2011), 1919–1920.
54. Caprioli, J., Noris, M., Brioschi, S., et al. Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood 108:4 (2006), 1267–1279.
55. Fremeaux-Bacchi, V., Miller, E.C., Liszewski, M.K., et al. Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome. Blood 112:13 (2008), 4948–4952.
56. Fakhouri, F., Delmas, Y., Provot, F., et al. Insights from the use in clinical practice of eculizumab in adult patients with atypical hemolytic uremic syndrome affecting the native kidneys: an analysis of 19 cases. Am J Kidney Dis 63:1 (2014), 40–48.